Medicine Endocrinology, Diabetes and Metabolism

Pituitary Gland Disorders and Treatments

Description

This cluster of papers focuses on the diagnosis and management of various pituitary disorders, including pituitary adenomas, Cushing's syndrome, acromegaly, and craniopharyngiomas. It covers topics such as hormonal replacement, endoscopic surgery, somatostatin analogues, and the impact of these disorders on growth hormone and prolactin levels.

Keywords

Pituitary Adenomas; Cushing's Syndrome; Acromegaly; Craniopharyngiomas; Hormonal Replacement; Endoscopic Surgery; Somatostatin Analogues; Prolactinomas; Growth Hormone; Pituitary Tumors

Pituitary tumors account for about 15% of primary intracranial neoplasms. Proliferation of pituitary cells, which secrete hormones, may result in a spectrum of endocrine symptoms. Tumors that originate from pituitary … Pituitary tumors account for about 15% of primary intracranial neoplasms. Proliferation of pituitary cells, which secrete hormones, may result in a spectrum of endocrine symptoms. Tumors that originate from pituitary somatotroph cells lead to aberrant secretion of growth hormone and the distinctive features of acromegaly. This review discusses advances in the understanding and treatment of acromegaly.
This dynamic CD-ROM provides one-click access to the full text and illustrations of Principles and Practice of Endocrinology and Metabolism, Third Edition, plus over 3,400 multiple-choice self-assessment questions and answers--in … This dynamic CD-ROM provides one-click access to the full text and illustrations of Principles and Practice of Endocrinology and Metabolism, Third Edition, plus over 3,400 multiple-choice self-assessment questions and answers--in an interactive format perfect for consultation, study, or review. Coverage includes new chapters on molecular biology, new therapies for pituitary tumors, pancreatic transplantation, appetite regulation, growth factors and cytokines, endocrine effects on lipids, endocrine blood cells, the endocrine adipocyte, adrenal incidentaloma, endocrine disorders of HIV infection, and more. Also included are quick-access references to aid in clinical decisions--a complete endocrine drug formulary, an extraordinary compendium of normal laboratory values, and a detailed listing of dynamic diagnostic testing. The program allows users to select the most helpful mode of review. Study Mode presents questions with immediate feedback through the chapter list. Quiz Mode displays randomized questions based on settings the user chooses. Exam Mode presents a set number of questions in random order and displays results at the end of the exam. Windows Compatible Compatibility: BlackBerry(r) OS 4.1 or Higher / iPhone/iPod Touch 2.0 or Higher /Palm OS 3.5 or higher / Palm Pre Classic / Symbian S60, 3rd edition (Nokia) / Windows Mobile(t) Pocket PC (all versions) / Windows Mobile Smartphone / Windows 98SE/2000/ME/XP/Vista/Tablet PC
The clinical features, perioperative course, and postoperative outcomes of 144 patients who underwent microsurgical resection of craniopharyngioma were reviewed. Overall, 90% of the tumors were completely resected and 7% recurred. … The clinical features, perioperative course, and postoperative outcomes of 144 patients who underwent microsurgical resection of craniopharyngioma were reviewed. Overall, 90% of the tumors were completely resected and 7% recurred. Evaluation of those patients who underwent primary resection revealed much better results. The operative techniques and approaches are reviewed in detail. The results of this series suggest that primary total removal of craniopharyngiomas yields the best long-term outcome for the patient. Experience has shown that the larger the tumor the greater will be the damage, both preoperatively and intraoperatively, to vital intracranial structures. Consequently, early diagnosis, at a stage when the tumor is still small, improves the chances of accomplishing complete removal and of achieving good operative results. The early diagnosis of craniopharyngioma, before it can produce devastating neurological defects, continues to be the principal goal of our medical and pediatric colleagues.
Abstract BACKGROUND Pituitary adenomas display an array of hormonal and proliferative activity. Once primarily classified according to size (microadenomas, < 1 cm; macroadenomas, ≥ 1 cm), these tumors are now … Abstract BACKGROUND Pituitary adenomas display an array of hormonal and proliferative activity. Once primarily classified according to size (microadenomas, < 1 cm; macroadenomas, ≥ 1 cm), these tumors are now further classified according to immunohistochemistry and functional status. With these additional classifications in mind, the goals of the current study were to determine the prevalence of pituitary adenomas and to explore the clinical relevance of the findings. METHODS The authors conducted a metaanalysis of all existing English‐language articles in MEDLINE. They used the search string (pituitary adenoma or pituitary tumor) and prevalence and selected relevant autopsy and imaging evaluation studies for inclusion. RESULTS The authors found an overall estimated prevalence of pituitary adenomas of 16.7% (14.4% in autopsy studies and 22.5% in radiologic studies). CONCLUSIONS Given the high frequency of pituitary adenomas and their potential for causing clinical pathologies, the findings of the current study suggest that early diagnosis and treatment of pituitary adenomas should have far‐reaching benefits. Cancer 2004. © 2004 American Cancer Society.
To determine the prevalence of focal lesions of the pituitary gland that suggest the presence of a pituitary adenoma in asymptomatic persons.100 normal volunteers (70 women, 30 men; age range, … To determine the prevalence of focal lesions of the pituitary gland that suggest the presence of a pituitary adenoma in asymptomatic persons.100 normal volunteers (70 women, 30 men; age range, 18 to 60 years old) were studied by high-resolution magnetic resonance imaging (MRI) of the pituitary gland before and after administration of gadolinium-diethylenetriaminepentaacetic acid (Gd-DTPA).Occult pituitary adenomas are identified at autopsy in 3% to 27% of unselected asymptomatic patients. The frequency of incidental pituitary adenomas detected by MRI in normal persons is unknown.The MRI scans from volunteers were randomly mixed with scans of 57 patients with Cushing disease and interpreted independently by three blinded reviewers.Seven women (10%) and three men (10%) had focal areas of decreased signal intensity in the pituitary gland after administration of Gd-DTPA. The lesions ranged from 3 to 6 mm in greatest diameter and were diagnosed as pituitary adenomas by at least two of the three reviewers. When similar lesions were detected on MRI scans in patients with Cushing disease, the positive predictive value for identification of an adenoma at that site was 86%.About 10% of the normal adult population have pituitary abnormalities on MRI scans that are compatible with the diagnosis of asymptomatic pituitary adenomas. Most pituitary adenomas remain asymptomatic and do not require treatment.
The primary objectives of this report were, first, to determine the number and incidence of complications of transsphenoidal surgery performed by a cross-section of neurosurgeons in the United States and, … The primary objectives of this report were, first, to determine the number and incidence of complications of transsphenoidal surgery performed by a cross-section of neurosurgeons in the United States and, second, to ascertain the influence of the surgeon's experience with the procedure on the occurrence of these complications. The secondary objective was to review complications of transsphenoidal surgery from the standpoint of their causation, treatment, and prevention.Questionnaires regarding 14 specific complications of transsphenoidal surgery were mailed to 3172 neurosurgeons. The data reported were analyzed from the 958 respondents (82%) who reported performing the operation. The neurosurgeons surveyed were asked to estimate the number of transsphenoidal operations performed, to identify any complications observed, and to estimate the percentage of operations that had resulted in any of the 14 specific complications. The 958 respondents were placed into three experience groups, based on the number of transsphenoidal operations performed. The data were analyzed by using chi 2 tests and Spearman correlation coefficients. The secondary objectives were met through a detailed review of the literature, in light of our experience.Of the respondents, 87.3% reported having performed < 200 operations and 9.7% reported 200 to 500 previous operations. The remaining 3% reported more than 500 previous operations. More extensive previous experience with transsphenoidal surgery was associated with a greater likelihood of having witnessed each specific complication. The mean operative mortality rate for all three groups was 0.9%. Anterior pituitary insufficiency (19.4%) and diabetes insipidus (17.8%) were complications with the highest incidence of occurrence. The overall incidence of cerebrospinal fluid fistulas was 3.9%. Other significant complications, such as carotid artery injuries, hypothalamic injuries, loss of vision, and meningitis, occurred with incidence rates between 1 and 2%. An inverse relationship was found between the experience group and the likelihood of complications, as indicated by significant negative Spearman correlation coefficients for all but 2 of the 14 complications listed in the survey (P < 0.05). Thus, increased experience with transsphenoidal surgery seems to be associated with a decreased percentage of operations resulting in complications. Some caution should be exercised in interpreting these data, because they are based on the respondents' estimates.Transsphenoidal surgery seems to be a reasonably safe procedure, with a mortality rate of less than 1%. However, a significant number of complications do occur. The incidence of these complications seems to be higher, with statistical significance, in the hands of less experienced surgeons. The learning curve seems to be relatively shallow, because a statistically significantly decreased incidence of morbidity and death could be documented after 200 and even 500 transsphenoidal operations. Better understanding of the indications for transsphenoidal surgery and improved familiarity with the regional anatomy should further lower the incidence of death and morbidity resulting from this procedure in the hands of all neurosurgeons.
I. Introduction II. Definitions and Etiology III. Diagnostic Overview IV. Clinical Features V. Biochemical Diagnosis of Cushing’s Syndrome A. Cardinal features B. Urinary free cortisol (UFC) C. Low-dose dexamethasone testing … I. Introduction II. Definitions and Etiology III. Diagnostic Overview IV. Clinical Features V. Biochemical Diagnosis of Cushing’s Syndrome A. Cardinal features B. Urinary free cortisol (UFC) C. Low-dose dexamethasone testing D. Circadian rhythm assessment E. Cyclical Cushing’s syndrome VI. ACTH-Dependent vs. ACTH-Independent Cushing’s Syndrome VII. Differential Diagnosis of ACTH-Dependent Cushing’s Syndrome A. Overview B. Basal testing C. Dynamic noninvasive testing D. Invasive testing VIII. Other Causes of Cushing’s Syndrome IX. Imaging A. Pituitary B. Adrenal C. Ectopic secretion X. Differentiation from Pseudo-Cushing’s States XI. Conclusions
Studies of acromegaly have shown a doubling of mortality compared with the general population. With the development of new modalities of treatment, it has become important to identify prognostic factors … Studies of acromegaly have shown a doubling of mortality compared with the general population. With the development of new modalities of treatment, it has become important to identify prognostic factors relating to mortality. Between 1964 and 2000, 208 acromegalic patients were followed for a mean of 13 yr at Auckland Hospital. Treatment was by surgery or radionuclide pituitary implantation, and all except 27 patients received pituitary radiation. Over the duration of the study, 72 patients died at a mean age of 61 ± 12.8 yr. Those dying were significantly older at diagnosis, had a higher prevalence of hypertension and diabetes, and were more likely to have hypopituitarism. The observed to expected mortality ratio (O/E ratio) fell from 2.6 (95% confidence interval, 1.9–3.6) in those with last follow-up GH greater than 5 μg/liter to 2.5 (1.6–3.8), 1.6 (0.9–3), and 1.1 (0.5–2.1) for those with GH less than 5, less than 2, and less than 1 μg/liter, respectively (P < 0.001). Serum IGF-I, expressed as an sd score, was significantly associated with mortality, with O/E mortality ratios of 3.5 (95% confidence interval, 2.8–4.2) for those with an sd score greater than 2, 1.6 (0.6–2.6) for those with an sd score less than 2 (normal or low levels), and 1.0 (0.1–3) for those with an sd score less than zero. When assessed by multivariate analysis, last serum GH (P < 0.001), age, duration of symptoms before diagnosis (P < 0.03), and hypertension (P < 0.04) were independent predictors of survival. If IGF-I was substituted for GH, then survival was independently related to last IGF-I sd score (P < 0.02), indicating that GH and IGF-I act equivalently as predictors of mortality. These findings indicate that reduction of GH to less than 1 μg/liter or normalization of serum IGF-I reduces mortality to expected levels.
Prolactinomas account for approximately 40% of all pituitary adenomas and are an important cause of hypogonadism and infertility. The ultimate goal of therapy for prolactinomas is restoration or achievement of … Prolactinomas account for approximately 40% of all pituitary adenomas and are an important cause of hypogonadism and infertility. The ultimate goal of therapy for prolactinomas is restoration or achievement of eugonadism through the normalization of hyperprolactinemia and control of tumor mass. Medical therapy with dopamine agonists is highly effective in the majority of cases and represents the mainstay of therapy. Recent data indicating successful withdrawal of these agents in a subset of patients challenge the previously held concept that medical therapy is a lifelong requirement. Complicated situations, such as those encountered in resistance to dopamine agonists, pregnancy, and giant or malignant prolactinomas, may require multimodal therapy involving surgery, radiotherapy, or both. Progress in elucidating the mechanisms underlying the pathogenesis of prolactinomas may enable future development of novel molecular therapies for treatment-resistant cases. This review provides a critical analysis of the efficacy and safety of the various modes of therapy available for the treatment of patients with prolactinomas with an emphasis on challenging situations, a discussion of the data regarding withdrawal of medical therapy, and a foreshadowing of novel approaches to therapy that may become available in the future.
Objective:The aim was to formulate practice guidelines for the diagnosis and treatment of hyperprolactinemia. Objective:The aim was to formulate practice guidelines for the diagnosis and treatment of hyperprolactinemia.
Summary OBJECTIVE Although Cushing's disease is a well documented clinical entity, there is no epidemiological information about it. The present study tries to obtain this information. DESIGN AND PATIENTS Forty‐nine … Summary OBJECTIVE Although Cushing's disease is a well documented clinical entity, there is no epidemiological information about it. The present study tries to obtain this information. DESIGN AND PATIENTS Forty‐nine patients affected by Cushing's disease living in Vizcaya (Spain) between 1975 and 1992 were considered for an epidemiological study. RESULTS The prevalence of known cases at the end of 1992 was 39.1 per million inhabitants. The average incidence of newly diagnosed cases was 2 4 cases per million people per year. Cushing's disease was more frequent in women ( n = 46) than in men ( n = 3), with a ratio of 15:1. Diabetes mellitus and hypertension were observed in 38.7 and 55.1% of patients, respectively. Remission of Cushing's disease was achieved in 36 out of 41 patients (87.5%). In general, the mortality was higher than that expected for the control population (standardized mortality ratio, SMR 3.8, 95% confidence interval, CI 2.5–17.9, P &lt;0.03). Concerning the cause of death, the SMR of vascular disease was 5 (95% CI 3.4–48.6, P &lt; 0.05). Higher age, persistence of hypertension and abnormalities of glucose metabolism after treatment, were independent predictors of mortality (multivariate analyses, P &lt;0.01). CONCLUSIONS Prevalence of Cushing's disease was 39.1 cases/million inhabitants and average incidence was 2.4 cases/million per year. Mortality was elevated, due to vascular disease, associated with higher age, persistence of hypertension and impaired glucose metabolism.
Cabergoline is a long-acting dopamine-agonist drug that suppresses prolactin secretion and restores gonadal function in women with hyperprolactinemic amenorrhea. We designed a study to compare its safety and efficacy with … Cabergoline is a long-acting dopamine-agonist drug that suppresses prolactin secretion and restores gonadal function in women with hyperprolactinemic amenorrhea. We designed a study to compare its safety and efficacy with those of bromocriptine, which has been the standard therapy.A total of 459 women with hyperprolactinemic amenorrhea were treated with either cabergoline (0.5 to 1.0 mg twice weekly) or bromocriptine (2.5 to 5.0 mg twice daily), administered in a double-blind fashion for 8 weeks and subsequently in an open fashion for 16 weeks, during which adjustments in the dose were made according to the response. Of the 459 women, 279 had microprolactinomas, 3 had macroprolactinomas, 1 had a craniopharyngioma, 167 had idiopathic hyperprolactinemia, and the remainder had an empty sella. Clinical and biochemical status was assessed at 2-week intervals for 8 weeks and monthly thereafter for a total of 6 months, with an additional assessment at 14 weeks.Stable normoprolactinemia was achieved in 186 of the 223 women treated with cabergoline (83 percent) and 138 of the 236 women treated with bromocriptine (59 percent, P < 0.001). Seventy-two percent of the women treated with cabergoline and 52 percent of those treated with bromocriptine had ovulatory cycles or became pregnant during treatment (P < 0.001). Amenorrhea persisted in 7 percent of the cabergoline-treated women and 16 percent of the bromocriptine-treated women. Adverse effects were recorded in 68 percent of the women taking cabergoline and 78 percent of those taking bromocriptine (P = 0.03); 3 percent discontinued taking cabergoline, and 12 percent stopped taking bromocriptine (P < 0.001) because of drug intolerance. Gastrointestinal symptoms were significantly less frequent, less severe, and shorter-lived in the women treated with cabergoline.Cabergoline is more effective and better tolerated than bromocriptine in women with hyperprolactinemic amenorrhea.
Pituitary adenomas (PAs) are associated with increased morbidity and mortality. The optimal delivery of services and the provision of care for patients with PAs require distribution of the resources proportionate … Pituitary adenomas (PAs) are associated with increased morbidity and mortality. The optimal delivery of services and the provision of care for patients with PAs require distribution of the resources proportionate to the impact of these conditions on the community. Currently, the resource allocation for PAs in the health care system is lacking a reliable and an up-to-date epidemiological background that would reflect the recent advances in the diagnostic technologies, leading to the earlier recognition of these tumours.To determine the prevalence, the diagnostic delay and the characteristics of patients with PA in a well-defined geographical area of the UK (Banbury, Oxfordshire).Sixteen general practitioner (GP) surgeries covering the area of Banbury and a total population of 89 334 inhabitants were asked to participate in the study (data confirmed on 31 July 2006). Fourteen surgeries with a total of 81,449 inhabitants (91% of the study population) agreed to take part. All cases of PAs were found following an exhaustive computer database search of agreed terms by the staff of each Practice and data on age, gender, presenting manifestations and their duration, imaging features at diagnosis, history of multiple endocrine neoplasia type 1 and family history of PA were collected.A total of 63 patients with PA were identified amongst the study population of 81,149, with a prevalence of 77.6 PA cases/100,000 inhabitants (prolactinomas; PRLoma: 44.4, nonfunctioning PAs: 22.2, acromegaly; ACRO: 8.6, corticotroph adenoma: 1.2 and unknown functional status; UFS: 1.2/100,000 inhabitants). The distribution of each PA subtype was for PRLoma 57%, nonfunctioning PAs 28%, ACRO 11%, corticotroph adenoma 2% and UFS 2%. The median age at diagnosis and the duration of symptoms until diagnosis (in years) were for PRLoma 32.0 and 1.5, nonfunctioning PAs 51.5 and 0.8, ACRO 47 and 4.5 and corticotroph adenoma 57 and 7, respectively. PRLoma was the most frequent PA diagnosed up to the age of 60 years (0-20 years: 75% and 20-60 years: 61% of PAs) and nonfunctioning PA after the age of 60 years (60% of PAs). Nonfunctioning PAs dominated in men (57% of all men with PA) and PRLoma in women (76% of all women with PA). Five patients (7.9%) presented with classical pituitary apoplexy, with a prevalence of 6.2 cases/100,000 inhabitants.Based on a well-defined population in Banbury (Oxfordshire, UK), we have shown that PAs have a fourfold increased prevalence than previously thought; our data confirm that PAs have a higher burden on the Health Care System and optimal resource distribution for both clinical care and research activities aiming to improve the outcome of these patients are needed.
Past attempts to test suppressibility of pituitary-adrenal function by the use of exogenous corticoids have yielded inconsistent findings in patients with Cushing's syndrome. The present study indicates that, with proper … Past attempts to test suppressibility of pituitary-adrenal function by the use of exogenous corticoids have yielded inconsistent findings in patients with Cushing's syndrome. The present study indicates that, with proper selection of suppressive agent and adjustment of dosage, valuable diagnostic information can be obtained from such tests. Δ1-9α-Fluorocortisol (ΔFF) and its 16α:-methylated analog (dexamethasone) were employed as suppressive agents. At dosage levels of 0.5 mg. every six hours for 8 doses these agents induced almost complete suppression of 17-hydroxycorticoid excretion in all 54 endocrinologically normal subjects tested. In contrast, all 35 of the patients with true Cushing's syndrome who were studied maintained relatively high urinary 17-hydroxycorticoid levels during such treatment. At these low dosages, therefore, the suppressive agents were useful in separating patients with Cushing's syndrome from those with normal adrenal function. Administration of ΔFF or dexamethasone in larger dosages of 2.0 mg. every six hours for 8 doses was used to separate patients with ACTH-dependent hyperadrenocorticism from those with autonomously functioning adrenal tissue. In response to large doses of suppressive agents, all patients with bilateral adrenocortical hypersecretion exhibited a definite decrease in 17-hydroxycorticoid excretion, whereas patients with adrenocortical tumors did not. The results support the view that the primary disorder in Cushing's syndrome with bilateral adrenocortical hypersecretion involves alteration of pituitary function in such a way that ACTH secretion is not suppressed by normal levels of cortisol.
In October 2002, a workshop was held in Ancona, Italy, to reach a Consensus on the management of Cushing's syndrome. The workshop was organized by the University of Ancona and … In October 2002, a workshop was held in Ancona, Italy, to reach a Consensus on the management of Cushing's syndrome. The workshop was organized by the University of Ancona and sponsored by the Pituitary Society, the European Neuroendocrine Association, and the Italian Society of Endocrinology. Invited international participants included almost 50 leading endocrinologists with specific expertise in the management of Cushing's syndrome. The consensus statement on diagnostic criteria and the diagnosis and treatment of complications of this syndrome reached at the workshop is hereby summarized.
The Acromegaly Consensus Group met in April 2009 to revisit the guidelines on criteria for cure as defined in 2000.Participants included 74 neurosurgeons and endocrinologists with extensive experience of treating … The Acromegaly Consensus Group met in April 2009 to revisit the guidelines on criteria for cure as defined in 2000.Participants included 74 neurosurgeons and endocrinologists with extensive experience of treating acromegaly. EVIDENCE/CONSENSUS PROCESS: Relevant assays, biochemical measures, clinical outcomes, and definition of disease control were discussed, based on the available published evidence, and the strength of consensus statements was rated.Criteria to define active acromegaly and disease control were agreed, and several significant changes were made to the 2000 guidelines. Appropriate methods of measuring and achieving disease control were summarized.
We present 25 pituitary adenomas that were confirmed surgically to have invaded the cavernous sinus space. The surgical results are compared with the preoperative magnetic resonance imaging findings. For comparable … We present 25 pituitary adenomas that were confirmed surgically to have invaded the cavernous sinus space. The surgical results are compared with the preoperative magnetic resonance imaging findings. For comparable radiological criteria, we classified parasellar growth into five grades. This proposed classification is based on coronal sections of unenhanced and gadolinium diethylene-triamine-pentaacetic acid enhanced magnetic resonance imaging scans, with the readily detectable internal carotid artery serving as the radiological landmark. The anatomical, radiological, and surgical conditions of each grade are considered. Grades 0, 1, 2, and 3 are distinguished from each other by a medial tangent, the intercarotid line--through the cross-sectional centers--and a lateral tangent on the intra- and supracavernous internal carotid arteries. Grade 0 represents the normal condition, and Grade 4 corresponds to the total encasement of the intracavernous carotid artery. According to this classification, surgically proven invasion of the cavernous sinus space was present in all Grade 4 and Grade 3 cases and in all but one of the Grade 2 cases; no invasion was present in Grade 0 and Grade 1 cases. Therefore, the critical area where invasion of the cavernous sinus space becomes very likely and can be proven surgically is located between the intercarotid line and the lateral tangent, which is represented by our Grade 2. We also measured tumor growth rates, using the monoclonal antibody KI-67, which shows a statistically higher proliferation rate (P < 0.001) in adenomas with surgically observed invasion into the cavernous sinus space, as compared with noninvasive adenomas.
✓ An endoscope was used in transsphenoidal surgery and eventually replaced the operating microscope as the tool for visualization. This study focuses on 50 patients (28 females and 22 males) … ✓ An endoscope was used in transsphenoidal surgery and eventually replaced the operating microscope as the tool for visualization. This study focuses on 50 patients (28 females and 22 males) with a median age of 38 years (range 14–88 years). Initially, four patients underwent operation via a sublabial—transseptal approach using a rigid endoscope in conjunction with an operating microscope. The 48 subsequent operations were performed through a nostril using only rigid endoscopes. Forty-four patients had pituitary adenomas and six had various other lesions. Thirteen patients had microadenomas, 16 had intrasellar macroadenomas, nine had macroadenomas with suprasellar extension, and six had invasive macroadenomas involving the cavernous sinus. Seven patients had recurrent pituitary adenomas and 25 had hormonesecreting adenomas (eight patients with Cushing's disease and 17 patients with prolactinomas). Among the eight patients with Cushing's disease, seven had resolution of hypercortisolism clinically and chemically. Of the 17 patients with prolactinomas, 10 improved clinically with normal serum prolactin levels, four improved clinically with elevated serum prolactin levels, and three had residual tumors in the cavernous sinus. Among the 19 patients with nonsecreting adenomas, 16 underwent total resection and three subtotal resection leaving residual tumor in the cavernous sinus. Postoperatively, all patients who had undergone endonasal endoscopic surgery had unobstructed nasal airways with minimal discomfort. More than half of the patients required only an overnight hospitalization.
Summary In June 2005, an ad hoc Expert Committee formed by the Pituitary Society convened during the 9th International Pituitary Congress in San Diego, California. Members of this committee consisted … Summary In June 2005, an ad hoc Expert Committee formed by the Pituitary Society convened during the 9th International Pituitary Congress in San Diego, California. Members of this committee consisted of invited international experts in the field, and included endocrinologists and neurosurgeons with recognized expertise in the management of prolactinomas. Discussions were held that included all interested participants to the Congress and resulted in formulation of these guidelines, which represent the current recommendations on the diagnosis and management of prolactinomas based upon comprehensive analysis and synthesis of all available data.
The main purpose was to assess the incidence and late outcome of Cushing's syndrome, particularly in Cushing's disease. Information for all patients diagnosed with Cushing's syndrome during an 11-yr period … The main purpose was to assess the incidence and late outcome of Cushing's syndrome, particularly in Cushing's disease. Information for all patients diagnosed with Cushing's syndrome during an 11-yr period in Denmark was retrieved. The incidence was 1.2–1.7/million·yr (Cushing's disease), 0.6/million·yr (adrenal adenoma) and 0.2/million·yr (adrenal carcinoma). Other types of Cushing's syndrome were rare. In 139 patients with nonmalignant disease, 11.1% had died during follow-up (median, 8.1 yr; range, 3.1–14.0), yielding a standard mortality ratio (SMR) of 3.68 [95% confidence interval (CI), 2.34–5.33]. The SMR was partly attributable to an increased mortality within the first year after diagnosis. Eight patients died before treatment could be undertaken. The prognosis in patients with malignant disease was very poor. Patients in whom more than 5 yr had elapsed since initial surgery were studied separately, including a questionnaire on their perceived quality of health. In 45 patients with Cushing's disease who had been cured through transsphenoidal neurosurgery, only 1 had died (SMR, 0.31; CI, 0.01–1.72) compared with 6 of 20 patients with persistent hypercortisolism after initial neurosurgery (SMR, 5.06; CI, 1.86–11.0). In patients with adrenal adenoma, SMR was 3.95 (CI, 0.81–11.5). The perceived quality of health was significantly impaired only in patients with Cushing's disease and appeared independent of disease control or presence of hypopituitarism. It is concluded that 1) Cushing's syndrome is rare and is associated with increased mortality, in patients with no concurrent malignancy also; 2) the excess mortality was mainly observed during the first year of disease; and 3) the impaired quality of health in long-term survivors of Cushing's disease is not fully explained.
This review focuses on the systemic complications of acromegaly. Mortality in this disease is increased mostly because of cardiovascular and respiratory diseases, although currently neoplastic complications have been questioned as … This review focuses on the systemic complications of acromegaly. Mortality in this disease is increased mostly because of cardiovascular and respiratory diseases, although currently neoplastic complications have been questioned as a relevant cause of increased risk of death. Biventricular hypertrophy, occurring independently of hypertension and metabolic complications, is the most frequent cardiac complication. Diastolic and systolic dysfunction develops along with disease duration; and other cardiac disorders, such as arrhythmias, valve disease, hypertension, atherosclerosis, and endothelial dysfunction, are also common in acromegaly. Control of acromegaly by surgery or pharmacotherapy, especially somatostatin analogs, improves cardiovascular morbidity. Respiratory disorders, sleep apnea, and ventilatory dysfunction are also important contributors in increasing mortality and are beneficially advantaged by controlling GH and IGF-I hypersecretion. An increased risk of colonic polyps, which more frequently recur in patients not controlled after treatment, has been reported by several independent investigations, although malignancies in other organs have also been described, but less convincingly than at the gastrointestinal level. Finally, the most important cause of morbidity and functional disability of the disease is arthropathy, which can be reversed at an initial stage, but not if the disease is left untreated for several years.
We have developed a sensitive homologous radioimmunoassay for human prolactin (hPRL). Prolactin isolated from human pituitary glands was used for generating antibodies in rabbits, for iodination, and for standards. The … We have developed a sensitive homologous radioimmunoassay for human prolactin (hPRL). Prolactin isolated from human pituitary glands was used for generating antibodies in rabbits, for iodination, and for standards. The assay is highly specific for hPRL and measures concentrations of hPRL as low as 1 ng/ml of human plasma. In this assay, the plasma concentrations of hPRL in a group of randomly selected male and female individuals averaged 13 ± 4 (SD) and 14 ± 4 ng/ml, respectively. In contrast, hPRL in the pooled plasma of pregnant women assayed 80 ng/ml; a post-partum plasma measured 215 ng/ml. Of 6 patients with acromegaly, 5 possessed hPRL levels above 34 ng/ml, the average being 40 ± 9 ng/ml. Administration of TSH releasing hormone increased plasma hPRL concentrations by 4-fold in both a male and a female subject, while LH releasing hormone depressed them slightly. The concentration of hPRL in pituitary glands removed at autopsy was 1.5 μg/mg for a male and 1.3 μg/mg for a female. These values approximate those found in other mammals.
In February 1999, a workshop was held in Cortina, Italy to develop a consensus defining the criteria for cure of acromegaly. The workshop was sponsored by the University of Brescia … In February 1999, a workshop was held in Cortina, Italy to develop a consensus defining the criteria for cure of acromegaly. The workshop was sponsored by the University of Brescia and hosted by the Italian Society of Endocrinology. Invited international participants included endocrinologists, neurosurgeons, and radiotherapists skilled in the management of acromegaly. This statement summarizes the consensus achieved in these discussions.
The objective is to formulate clinical practice guidelines for treating Cushing's syndrome. The objective is to formulate clinical practice guidelines for treating Cushing's syndrome.
Prevalence data are important for assessing the burden of disease on the health care system; data on pituitary adenoma prevalence are very scarce.The objective of the study was to measure … Prevalence data are important for assessing the burden of disease on the health care system; data on pituitary adenoma prevalence are very scarce.The objective of the study was to measure the prevalence of clinically relevant pituitary adenomas in a well-defined population.This was a cross-sectional, intensive, case-finding study performed in three regions of the province of Liège, Belgium, to measure pituitary adenoma prevalence as of September 30, 2005.The study was conducted in specialist and general medical practitioner patient populations, referral hospitals, and investigational centers.Three demographically and geographically distinct districts of the province of Liège were delineated precisely using postal codes. Medical practitioners in these districts were recruited, and patients with pituitary adenomas under their care were identified. Diagnoses were confirmed after retrieval of clinical, hormonal, radiological, and pathological data; full demographic and therapeutic follow-up data were collected in all cases.Sixty-eight patients with clinically relevant pituitary adenomas were identified in a population of 71,972 individuals; the mean (+/- sd) prevalence was 94 +/- 19.3 cases per 100,000 population (95% confidence interval, 72.2 to 115.8). The group was 67.6% female and had a mean age at diagnosis of 40.3 yr; 42.6% had macroadenomas and 55.9% underwent surgery. Prolactinomas comprised 66% of the group, with the rest having nonsecreting tumors (14.7%), somatotropinomas (13.2%), or Cushing's disease (5.9%); 20.6% had hypopituitarism.The prevalence of pituitary adenomas in the study population (one case in 1064 individuals) was more than 3.5-5 times that previously reported. This increased prevalence may have important implications when prioritizing funding for research and treatment of pituitary adenomas.
The Acromegaly Consensus Group reconvened in November 2007 to update guidelines for acromegaly management.The meeting participants comprised 68 pituitary specialists, including neurosurgeons and endocrinologists with extensive experience treating patients with … The Acromegaly Consensus Group reconvened in November 2007 to update guidelines for acromegaly management.The meeting participants comprised 68 pituitary specialists, including neurosurgeons and endocrinologists with extensive experience treating patients with acromegaly. EVIDENCE/CONSENSUS PROCESS: Goals of treatment and the appropriate imaging and biochemical and clinical monitoring of patients with acromegaly were enunciated, based on the available published evidence.The group developed a consensus on the approach to managing acromegaly including appropriate roles for neurosurgery, medical therapy, and radiation therapy in the management of these patients.
Our objective was to evaluate the published literature and reach a consensus on the treatment of patients with ACTH-dependent Cushing's syndrome, because there is no recent consensus on the management … Our objective was to evaluate the published literature and reach a consensus on the treatment of patients with ACTH-dependent Cushing's syndrome, because there is no recent consensus on the management of this rare disorder.Thirty-two leading endocrinologists, clinicians, and neurosurgeons with specific expertise in the management of ACTH-dependent Cushing's syndrome representing nine countries were chosen to address 1) criteria for cure and remission of this disorder, 2) surgical treatment of Cushing's disease, 3) therapeutic options in the event of persistent disease after transsphenoidal surgery, 4) medical therapy of Cushing's disease, and 5) management of ectopic ACTH syndrome, Nelson's syndrome, and special patient populations.Participants presented published scientific data, which formed the basis of the recommendations. Opinion shared by a majority of experts was used where strong evidence was lacking.Participants met for 2 d, during which there were four chaired sessions of presentations, followed by general discussion where a consensus was reached. The consensus statement was prepared by a steering committee and was then reviewed by all authors, with suggestions incorporated if agreed upon by the majority.ACTH-dependent Cushing's syndrome is a heterogeneous disorder requiring a multidisciplinary and individualized approach to patient management. Generally, the treatment of choice for ACTH-dependent Cushing's syndrome is curative surgery with selective pituitary or ectopic corticotroph tumor resection. Second-line treatments include more radical surgery, radiation therapy (for Cushing's disease), medical therapy, and bilateral adrenalectomy. Because of the significant morbidity of Cushing's syndrome, early diagnosis and prompt therapy are warranted.
Patients with acromegaly are treated with surgery, radiation therapy, and drugs to reduce hypersecretion of growth hormone, but the treatments may be ineffective and have adverse effects. Pegvisomant is a … Patients with acromegaly are treated with surgery, radiation therapy, and drugs to reduce hypersecretion of growth hormone, but the treatments may be ineffective and have adverse effects. Pegvisomant is a genetically engineered growth hormone–receptor antagonist that blocks the action of growth hormone.
The aim was to formulate clinical practice guidelines for acromegaly.The Task Force included a chair selected by the Endocrine Society Clinical Guidelines Subcommittee (CGS), five experts in the field, and … The aim was to formulate clinical practice guidelines for acromegaly.The Task Force included a chair selected by the Endocrine Society Clinical Guidelines Subcommittee (CGS), five experts in the field, and a methodologist. The authors received no corporate funding or remuneration. This guideline is cosponsored by the European Society of Endocrinology.This evidence-based guideline was developed using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system to describe both the strength of recommendations and the quality of evidence. The Task Force reviewed primary evidence and commissioned two additional systematic reviews.One group meeting, several conference calls, and e-mail communications enabled consensus. Committees and members of the Endocrine Society and the European Society of Endocrinology reviewed drafts of the guidelines.Using an evidence-based approach, this acromegaly guideline addresses important clinical issues regarding the evaluation and management of acromegaly, including the appropriate biochemical assessment, a therapeutic algorithm, including use of medical monotherapy or combination therapy, and management during pregnancy.
Ectopic ACTH secretion (EAS) is difficult to diagnose and treat. We present our experience with EAS from 1983 to 2004.The study was performed at a tertiary care clinical research center.Ninety … Ectopic ACTH secretion (EAS) is difficult to diagnose and treat. We present our experience with EAS from 1983 to 2004.The study was performed at a tertiary care clinical research center.Ninety patients, aged 8-72 yr, including 48 females were included in the study.Tests included 8 mg dexamethasone suppression, CRH stimulation, inferior petrosal sinus sampling (IPSS), computed tomography, octreotide scan, magnetic resonance imaging, and/or venous sampling. Therapies, pathological examinations, and survival were noted.Eighty-six to 94% of patients did not respond to CRH or dexamethasone suppression, whereas 66 of 67 had negative IPSS. To control hypercortisolism, 62 patients received medical treatment, and 33 had bilateral adrenalectomy. Imaging localized tumors in 67 of 90 patients. Surgery confirmed an ACTH-secreting tumor in 59 of 66 patients and cured 65%. Nonthymic carcinoids took longest to localize. Deaths included three of 35 with pulmonary carcinoid, two of five with thymic carcinoid, four of six with gastrinoma, two of 13 with neuroendocrine tumor, two of two with medullary thyroid cancer, one of five with pheochromocytoma, three of three with small-cell lung cancer, and two of 17 with occult tumor. Patients with other carcinoids and ethesioneuroblastoma are alive.IPSS best identifies EAS. Initial failed localization is common and suggests pulmonary carcinoid. Although only 47% achieved cure, survival is good except in patients with small-cell lung cancer, medullary thyroid cancer, and gastrinoma.
Dysregulated growth hormone (GH) hypersecretion is usually caused by a GH-secreting pituitary adenoma and leads to acromegaly — a disorder of disproportionate skeletal, tissue, and organ growth. High GH and … Dysregulated growth hormone (GH) hypersecretion is usually caused by a GH-secreting pituitary adenoma and leads to acromegaly — a disorder of disproportionate skeletal, tissue, and organ growth. High GH and IGF1 levels lead to comorbidities including arthritis, facial changes, prognathism, and glucose intolerance. If the condition is untreated, enhanced mortality due to cardiovascular, cerebrovascular, and pulmonary dysfunction is associated with a 30% decrease in life span. This Review discusses acromegaly pathogenesis and management options. The latter include surgery, radiation, and use of novel medications. Somatostatin receptor (SSTR) ligands inhibit GH release, control tumor growth, and attenuate peripheral GH action, while GH receptor antagonists block GH action and effectively lower IGF1 levels. Novel peptides, including SSTR ligands, exhibiting polyreceptor subtype affinities and chimeric dopaminergic-somatostatinergic properties are currently in clinical trials. Effective control of GH and IGF1 hypersecretion and ablation or stabilization of the pituitary tumor mass lead to improved comorbidities and lowering of mortality rates for this hormonal disorder.
Patients with acromegaly have a reduced life expectancy, with the accepted causes for premature death being vascular and respiratory disease. Increased mortality from malignant disease has also been reported. We, … Patients with acromegaly have a reduced life expectancy, with the accepted causes for premature death being vascular and respiratory disease. Increased mortality from malignant disease has also been reported. We, therefore, performed a multicenter retrospective cohort study of 1362 patients with acromegaly and investigated the relationships of mortality and cancer incidence with GH levels, duration of disease, and age at diagnosis. The overall cancer incidence rate [standardized incidence ratio, 0.76; 95% confidence interval (CI), 0.60–0.95] was lower than that in the general population of the United Kingdom, and there was no significant increase in site-specific cancer incidence rates. The overall cancer mortality rate was not increased, but the colon cancer mortality rate (standardized mortality ratio, 2.47; 95% CI, 1.31–4.22) was higher than expected. Mortality rates due to colon cancer, all malignant disease, cardiovascular disease and overall mortality were increased with higher posttreatment GH levels (P for trends,<0.02, <0.05, <0.02, and <0.0001). The overall mortality rate in patients with acromegaly with posttreatment GH levels less than 2.5 ng/mL (5 mU/L) was comparable to that in the general population of the United Kingdom (standardized mortality ratio, 1.10; 95% CI, 0.89–1.35). We conclude that high posttreatment GH levels are associated with an increased overall mortality rate and increased mortality rates due to colon cancer, cardiovascular disease, and all malignant disease. Posttreatment GH levels less than 2.5 ng/mL (5 mU/L) result in an overall mortality rate similar to that in the general population.
Transsphenoidal approaches have been used for a century for the resection of pituitary and other sellar tumors. More recently, the standard endonasal approach has been expanded to provide access to … Transsphenoidal approaches have been used for a century for the resection of pituitary and other sellar tumors. More recently, the standard endonasal approach has been expanded to provide access to other, parasellar lesions. With the addition of the endoscope, this expansion carries significant potential for the resection of skull base lesions.The anatomical landmarks and surgical techniques used in expanded (extended) endoscopic approaches to the rostral, anterior skull base are reviewed and presented, accompanied by case illustrations of each segment (or module) of approach. The rostral half of the anterior skull base is divided into modules of approach: sellar/parasellar, transplanum/transtuberculum, and transcribriform. Case illustrations of successful resections of lesions with each module are presented and discussed.Endoscopic, expanded endonasal approaches to rostral anterior skull base lesions are feasible and hold great potential for decreased morbidity. The effectiveness and appropriate use of these techniques must be evaluated by close examination of outcomes as case series expand.
The goal of this study was to assess the outcome of surgical management in 168 consecutive patients harboring craniopharyngiomas treated between January 1983 and April 1997.In 148 patients undergoing initial … The goal of this study was to assess the outcome of surgical management in 168 consecutive patients harboring craniopharyngiomas treated between January 1983 and April 1997.In 148 patients undergoing initial (primary) surgery, the pterional approach was most frequently used (39.2%), followed by the transsphenoidal approach (23.6%). For large retrochiasmatic craniopharyngiomas, the bifrontal interhemispheric approach was used increasingly over the pterional approach and led to improved surgical results. Total tumor removal was accomplished in 45.7% of transcranial and 85.7% of transsphenoidal procedures. The main reasons for incomplete removal were attachment to and/or infiltration of the hypothalamus, major calcifications, and attachment to vascular structures. The success rate in total tumor removal was inferior in the cases of tumor recurrence. The operative mortality rate in transcranial surgery was 1.1% in primary cases and 10.5% in cases of tumor recurrence. No patient died in the group that underwent transsphenoidal surgery. The rate of recurrence-free survival after total removal was 86.9% at 5 years and 81.3% at 10 years. In contrast, the 5-year recurrence-free survival rate was only 48.8% after subtotal removal and 41.5% after partial removal. Following primary surgery, the actuarial survival rate was 92.7% at 10 years, with the best results after complete tumor removal. At last follow up, 117 (79%) of 148 patients who underwent primary surgery were independent and without impairment.Total tumor removal while avoiding hazardous intraoperative manipulation provides favorable early results and a high rate of long-term control in craniopharyngiomas.
Patients with brain tumors who are treated with radiation frequently have growth hormone deficiency, but other neuroendocrine abnormalities are presumed to be uncommon. Patients with brain tumors who are treated with radiation frequently have growth hormone deficiency, but other neuroendocrine abnormalities are presumed to be uncommon.
Measurement of adrenocorticotropin levels in plasma from the inferior petrosal sinuses of patients with Cushing's syndrome can distinguish adrenocorticotropin-secreting pituitary tumors (Cushing's disease) from other causes of the syndrome, principally … Measurement of adrenocorticotropin levels in plasma from the inferior petrosal sinuses of patients with Cushing's syndrome can distinguish adrenocorticotropin-secreting pituitary tumors (Cushing's disease) from other causes of the syndrome, principally ectopic adrenocorticotropin secretion from an occult tumor. However, it is unknown whether such measurement consistently identifies patients with Cushing's disease and whether testing with corticotropin-releasing hormone (CRH) enhances the value of the procedure.
Pituitary adenomas may hypersecrete hormones or cause mass effects. Therefore, early diagnosis and treatment are important.Prevalence of pituitary adenomas ranges from 1 in 865 adults to 1 in 2688 adults. … Pituitary adenomas may hypersecrete hormones or cause mass effects. Therefore, early diagnosis and treatment are important.Prevalence of pituitary adenomas ranges from 1 in 865 adults to 1 in 2688 adults. Approximately 50% are microadenomas (<10 mm); the remainder are macroadenomas (≥10 mm). Mass effects cause headache, hypopituitarism, and visual field defects. Treatments include transsphenoidal surgery, medical therapies, and radiotherapy. Prolactinomas account for 32% to 66% of adenomas and present with amenorrhea, loss of libido, galactorrhea, and infertility in women and loss of libido, erectile dysfunction, and infertility in men; they are generally treated with the dopamine agonists cabergoline and bromocriptine. Growth hormone-secreting tumors account for 8% to 16% of tumors and usually present with enlargement of the lips, tongue, nose, hands, and feet and are diagnosed by elevated insulin-like growth factor 1 levels and growth hormone levels; initial treatment is surgical. Medical therapy with somatostatin analogues, cabergoline, and pegvisomant is often also needed. Adrenocorticotropic hormone (ACTH)-secreting tumors account for 2% to 6% of adenomas and are associated with obesity, hypertension, diabetes, and other morbidity. Measurement of a late-night salivary cortisol level is the best screening test but petrosal sinus sampling for ACTH may be necessary to distinguish a pituitary from an ectopic source. The primary treatment of Cushing disease (hypercortisolism due to ACTH-producing adenomas, which is the cause in approximately 65% of the cases of hypercortisolism) is adenoma resection and medical therapies including ketoconazole, mifepristone, and pasireotide. Hyperthyroidism due to thyroid-stimulating hormone-secreting tumors accounts for 1% of tumors and is treated with surgery and somatostatin analogues if not surgically cured. Clinically nonfunctioning adenomas account for 15% to 54% of adenomas and present with mass effects; surgery is generally required, although incidentally found tumors can be followed if they are asymptomatic.Patients with pituitary adenomas should be identified at an early stage so that effective treatment can be implemented. For prolactinomas, initial therapy is generally dopamine agonists. For all other pituitary adenomas, initial therapy is generally transsphenoidal surgery with medical therapy being reserved for those not cured by surgery.
In this report the authors describe the epidemiology of craniopharyngioma.The incidence of craniopharyngioma in the United States was estimated from two population-based cancer registries that include brain tumors of benign … In this report the authors describe the epidemiology of craniopharyngioma.The incidence of craniopharyngioma in the United States was estimated from two population-based cancer registries that include brain tumors of benign and borderline malignancy: the Central Brain Tumor Registry of the United States (CBTRUS) and the Los Angeles county Cancer Surveillance Program. Information on additional pediatric tumors was available from the Greater Delaware Valley Pediatric Tumor Registry (GDVPTR). The overall incidence of craniopharyngioma was 0.13 per 100,000 person years and did not vary by gender or race. A bimodal distribution by age was noted with peak incidence rates in children (aged 5-14 years) and among older adults (aged 65-74 years in CBTRUS and 50-74 years in Los Angeles county). Survival information was available from GDVPTR and the National Cancer Data Base (NCDB), a hospital-based reporting system. In the NCDB, the 5-year survival rate was 80% and decreased with older age at diagnosis. Survival is higher among children and has improved in recent years.Craniopharyngioma is a rare brain tumor of uncertain behavior that occurs at a rate of 1.3 per million person years. Approximately 338 cases of this disease are expected to occur annually in the United States, with 96 occurring in children from 0 to 14 years of age.
Journal Article THE JOURNAL OF CLINICAL ENDOCRINOLOGY AND METABOLISM Get access The Journal of Clinical Endocrinology & Metabolism, Volume 48, Issue 2, 1 February 1979, Pages 363–364, https://doi.org/10.1210/jcem-48-2-363 Published: 01 … Journal Article THE JOURNAL OF CLINICAL ENDOCRINOLOGY AND METABOLISM Get access The Journal of Clinical Endocrinology & Metabolism, Volume 48, Issue 2, 1 February 1979, Pages 363–364, https://doi.org/10.1210/jcem-48-2-363 Published: 01 February 1979
Journal Article THE JOURNAL OF CLINICAL ENDOCRINOLOGY AND METABOLISM Get access The Journal of Clinical Endocrinology & Metabolism, Volume 46, Issue 2, 1 February 1978, Pages 356–357, https://doi.org/10.1210/jcem-46-2-356 Published: 01 … Journal Article THE JOURNAL OF CLINICAL ENDOCRINOLOGY AND METABOLISM Get access The Journal of Clinical Endocrinology & Metabolism, Volume 46, Issue 2, 1 February 1978, Pages 356–357, https://doi.org/10.1210/jcem-46-2-356 Published: 01 February 1978
BACKGROUND AND OBJECTIVES: So far, little is known about early macular microcirculation changes in nonfunctioning pituitary adenomas (NFPAs), particularly in patients before visual field defects (VFD) occur. The aim of … BACKGROUND AND OBJECTIVES: So far, little is known about early macular microcirculation changes in nonfunctioning pituitary adenomas (NFPAs), particularly in patients before visual field defects (VFD) occur. The aim of our study was to evaluate the early retinal microcirculation alteration in NFPA before VFD by wide-field swept source optical coherence tomography (WF SS-OCTA). METHODS: A total of 64 NFPA patients without VFD and 64 healthy controls were included. The thickness of the retinal nerve fiber layer (RNFL), ganglion cell complex (GCC), flow area (FA) and vessel density (VD) of radial capillary plexus (RCP), superficial vascular complex (SVC), and deep vascular complex (DVC) of their right eyes were measured on 12 mm × 12 mm macular area by WF SS-OCTA. The differences between the 2 groups were analyzed, and receiver operating characteristic curves were conducted to evaluate the diagnostic performance of retinal microcirculation. RESULTS: Compared with the controls, RCP VD, SVC FA, SVC VD, DVC FA, and DVC VD in NFPA were significantly lower ( P &lt; .05). RCP FA, RNFL and GCC thicknesses were not significantly different between the 2 groups ( P &gt; .05). Multiple regressions showed that RCP FA, RCP VD, SVC FA, and SVC VD were positively correlated with RNFL and GCC thicknesses ( P &lt; .01), whereas DVC FA and DVC VD were associated with neither RNFL nor GCC thicknesses ( P &gt; .05). The area under the receiver operating characteristic curve showed RCP VD, SVC VD, deep capillary plexus FA, and deep capillary plexus VD (area under the curve &gt;0.6, P &lt; .05) had diagnostic significances for NFPA. CONCLUSION: NFPA patients before VFD produce a characteristic pattern of early retinal microcirculation loss that WF SS-OCTA can recognize. WF SS-OCTA is a useful tool for early predicting visual impairment in NFPA.
H. F. Atkinson | Radiopaedia.org
Pituitary neuroendocrine tumors (PitNETs) are common intracranial neoplasms with complex biology underpinned by unresolved cellular origins, molecular heterogeneity, and microenvironment interactions. Here, we employ single-nuclei RNA-sequencing (snRNA-seq) of 419,874 cells … Pituitary neuroendocrine tumors (PitNETs) are common intracranial neoplasms with complex biology underpinned by unresolved cellular origins, molecular heterogeneity, and microenvironment interactions. Here, we employ single-nuclei RNA-sequencing (snRNA-seq) of 419,874 cells from human normal pituitaries and PitNETs with spatial transcriptomics to resolve these challenges. We identify multi-hormonal neuroendocrine cells in both normal and tumor tissues, originating as early pseudotime intermediates from pituitary stem cells, revealing an inherent plasticity that blurs traditional lineage boundaries. PitNETs exhibit a transcriptional continuum across subtypes, challenging their classification into discrete categories. Trajectory analysis uncovers divergent cellular origins: silent gonadotroph adenomas (SGAs), prolactinomas, silent corticotroph adenomas (SCAs), and Cushing's adenomas are closely linked to differentiated neuroendocrine cells, while somatotroph and null cell adenomas (NCAs) appear to derive more directly from adult stem cells. Tumor cells co-opt robust cell-cell communication networks found in normal adult neuroendocrine cells. Spatial profiling further demonstrates that perivascular niches enhance tumorigenicity through angiogenic and epithelial-mesenchymal transition programs. Our work redefines PitNETs as ecosystems shaped by developmental plasticity and microenvironmental crosstalk, offering a roadmap for future therapies targeting lineage fluidity and stromal dependencies.
(1) Background: Acromegaly is a rare disease associated with multiple complications. Consequently, it has a high clinical burden, which leads to a lower quality of life (QoL). The Acromegaly Quality … (1) Background: Acromegaly is a rare disease associated with multiple complications. Consequently, it has a high clinical burden, which leads to a lower quality of life (QoL). The Acromegaly Quality of Life Questionnaire (AcroQoL) is a specific tool developed to assess the impact of the disease on a patient’s physical and emotional well-being. Current research on anxiety has shown that higher levels of psychosocial factors are linked to a poorer quality of life. (2) Methods: Our study included 40 patients (26 women and 14 men) with a mean disease duration of 85.9 ± 97.7 months. Information about disease status, associated comorbidities, and clinical and paraclinical data was obtained. All patients completed the AcroQoL questionnaire. (3) Results: The lowest score was observed on the physical scale, while the least affected scale was personal relations. Biochemical parameters, biochemical control, and adenoma size were not associated with a lower QoL. Gender, age at diagnosis, and comorbidities, such as hypertension and arthropathy, were associated with a decrease in QoL. Additionally, the presence of anxiety and depression, which were mostly reported by women (30.7%), had a negative impact on the global QoL. (4) Conclusions: Early diagnosis of acromegaly can increase the QoL by preventing comorbidities, but there are also non-modifiable factors that have been associated with a decreased QoL. Preventing depression and anxiety could serve as important targets for future interventions.
E. Melikov , A. Drobyshev , Ekaterina Pigarova +5 more | International Journal of Oral and Maxillofacial Surgery
Az elmúlt évtizedekben a klinikai endokrinológia jelentős fejlődésen ment át hazánkban. E rövid áttekintésben e fejlődés néhány fő elemét mutatom be, saját szakterületemre, a mellékvese betegségeire és a neuroendokrin daganatokra, … Az elmúlt évtizedekben a klinikai endokrinológia jelentős fejlődésen ment át hazánkban. E rövid áttekintésben e fejlődés néhány fő elemét mutatom be, saját szakterületemre, a mellékvese betegségeire és a neuroendokrin daganatokra, valamint az öröklődő daganatszindrómákra összpontosítva. A diagnosztika és kezelés fejlődésének fő elemeit, a várható kihívásokat és további fejlődési irányokat is tárgyaljuk.
MRI fails to localize a tumor in up to 40% of cases of ACTH dependent Cushing Disease (CD), impacting patient outcomes and creating uncertainty in the diagnosis. Photon-counting detector CT … MRI fails to localize a tumor in up to 40% of cases of ACTH dependent Cushing Disease (CD), impacting patient outcomes and creating uncertainty in the diagnosis. Photon-counting detector CT (PCD-CT) is a new CT technology with better imaging performance than conventional CT. PCD-CT is a novel imaging technique for CD, especially for MR-negative cases, however its application to CD remains unknown. We present a cohort of consecutive patients with a pre-operative PCD-CT undergoing transsphenoidal surgery for CD at a single institution from September 2023 to January 2025. All patients underwent gadolinium-enhanced pituitary MRI for baseline work-up and PCD-CT as part of their preoperative planning imaging. Both imaging modalities were used to localize the lesion and guide surgical planning. The accuracies of MRI and PCD-CT were based on confirmation of the lesion location by a combination of intraoperative visualization, pathologic confirmation, and postoperative biochemical remission, and were compared with each other via Fisher's exact test. Twenty-five patients with CD were included in this analysis. The cohort's median age was 50 years old, and 88% were female. At diagnosis, 88% had central obesity, 76% had hypertension, 48% had type II diabetes mellitus, and 20% had osteoporosis. Contrast-enhanced MRI accurately identified 56% of lesions, while it was negative in 24% and misleading in 20%. Dynamic contrast-enhanced PCD-CT was superior in localizing CD (p=0.01), accurately identifying 92% of lesions, while it was negative in 4% and misleading in 4% of cases. Early postoperative biochemical remission was achieved in 88% of cases, while 84% remained in remission at follow-up. PCD-CT had a high rate of adenoma localization confirmed intraoperatively, even among MR-negative cases, potentially leading to high remission and gland preservation rates. Its role in the clinical workflow of preoperative evaluation for CD requires further investigation, but current evidence supports its value in lesion localization, especially in MR-negative disease. CD= cushing disease; PCD= photon counting detector.
Pituitary adenomas (PAs) are generally benign tumors with varying clinical presentations. While functioning PAs (FPAs) secrete hormones leading to distinct endocrine disorders, non-functioning PAs (NFPAs) often manifest with mass effect … Pituitary adenomas (PAs) are generally benign tumors with varying clinical presentations. While functioning PAs (FPAs) secrete hormones leading to distinct endocrine disorders, non-functioning PAs (NFPAs) often manifest with mass effect symptoms. Current medical therapies for PAs have limited efficacy, particularly for NFPAs. This highlights the need for new diagnostic and therapeutic targets. This study aimed to investigate the expression of Neuro-Oncological Ventral Antigen 2 (NOVA2), a factor involved in alternative splicing, in normal pituitary glands and PAs, to evaluate its potential as a novel therapeutic target. Tissue samples from 12 PA patients (9 NFPAs and 3 FPAs) and 8 normal pituitary glands were collected. Immunohistochemical analysis was performed to assess NOVA2 expression. Semi-quantitative evaluation of NOVA2 staining was conducted using Histoscore. NOVA2 was expressed in the endothelial cells of both PAs and normal pituitary glands, with higher expression in PAs. Notably, NOVA2 was present in PA cells regardless of adenoma type, while normal pituitary epithelial cells were negative for NOVA2 expression. There was no statistically significant difference in NOVA2 expression between FPAs and NFPAs. This is the first study to demonstrate that NOVA2 is expressed in PA cells but not in normal pituitary epithelial cells. These findings suggest that NOVA2 could serve as a potential target for diagnostic and therapeutic strategies in PAs, especially considering the lack of specific biomarkers and effective medical therapies for these tumors. Further research with a larger sample size is warranted to validate these preliminary findings.
Functional gonadotroph adenoma (FGA) is a rare condition associated with secretion of biologically active gonadotropins which affect reproductive organs. In women of reproductive age, it has been reported as a … Functional gonadotroph adenoma (FGA) is a rare condition associated with secretion of biologically active gonadotropins which affect reproductive organs. In women of reproductive age, it has been reported as a cause of spontaneous ovarian hyperstimulation syndrome (OHSS) occurring outside the context of assisted reproductive technology (ART). In rare instances, FGA may present as suspicious ovarian masses, leading to an overlooked pituitary disorder. We report the case of a 34-year-old woman initially suspected of having a bilateral ovarian tumor with a borderline component due to thick-walled cystic masses. She underwent pelvic surgery, resulting in an oophorectomy. However, a few weeks postoperatively, the sudden onset of galactorrhea prompted further investigation, revealing hyperprolactinemia, FSH hypersecretion, and low LH levels. Ultimately, the diagnosis of FGA was established. A literature review was conducted to analyze similar cases where patients underwent ovarian surgery without prior hormonal assessment or suspicion of pituitary pathology, only to be diagnosed with FGA later. Thirteen additional cases were identified, including ovarian cysts and two cases of suspicious ovarian masses, with diagnostic delays ranging from 1.5 to 10 years. This case highlights the importance of considering FGA in the differential diagnosis of bilateral ovarian masses to avoid unnecessary surgical procedures.
Syeda Maria Ahmad Zaidi , Haissan Iftikhar , Shahzada Ahmed | The Egyptian Journal of Neurosurgery : the official publication of the Egyptian Society of Neurological Surgeons/Egyptian journal of neurosurgery
Abstract Background Pituitary metastasis is a rare entity, accounting for less than 1% of all intracranial neoplasms. Common primary sites include the breast and lungs. Due to the indolent clinical … Abstract Background Pituitary metastasis is a rare entity, accounting for less than 1% of all intracranial neoplasms. Common primary sites include the breast and lungs. Due to the indolent clinical nature of the disease and non-specific radiological findings, they are not easily discernible from more common pituitary lesions. Diagnosis is further complicated in the absence of an established underlying malignancy. Case presentation A 67-year old male patient presented with severe acute onset headache and vomiting, with associated left third cranial nerve palsy and a rapid decline in visual acuity. Imaging revealed a large 35.8 × 13.6 mm (CC x AP) haemorrhagic sellar lesion with suprasellar extension and compression of the optic chiasma. A primary non-functioning pituitary macro-adenoma was suspected and endoscopic trans-nasal trans-sphenoidal surgery under neuroimaging was undertaken. Histopathology of the necrotic tissue confirmed an apoplectic gonadotropinoma, with areas of malignant epithelial cells and a high mitotic rate (Ki-67 index of up to 30%). Primary disease, squamous cell carcinoma and neoplasms of bronchial and germ cell origins were excluded via immunohistochemistry. Whole body PET CT revealed no underlying malignancy. In view of the unknown origin of the primary tumour, the patient received radiotherapy to the pituitary fossa, and was kept on surveillance for five years. His visual function improved, and he remained stable post treatment. His diagnosis of primary lesion was still uncertain despite extensive histological and radiological investigations. Conclusion This case report underscores the challenges in diagnosis and treatment of metastatic pituitary lesions. Due to the unpredictable course of the disease, improvisation in management enabled favourable outcome.
Abstract Purpose Biochemical remission is the primary treatment goal in acromegaly. However, some patients experience biochemical discordance between growth hormone (GH) and insulin-like growth factor-I (IGF-I) levels following multimodal therapy, … Abstract Purpose Biochemical remission is the primary treatment goal in acromegaly. However, some patients experience biochemical discordance between growth hormone (GH) and insulin-like growth factor-I (IGF-I) levels following multimodal therapy, complicating disease assessment and management. This study aims to identify predictive factors associated with post-therapeutic biochemical discrepancy. Methods We conducted a retrospective monocentric analysis of 156 patients with GH-producing pituitary adenomas (PAs) who underwent transsphenoidal surgery between 1984 and 2017. Biochemical outcomes were classified into four groups: group 1 (biochemical remission), group 2 (isolated GH normalization), group 3 (isolated IGF-I normalization), and group 4 (persistent acromegaly). Predictive factors for biochemical discrepancy were assessed, including demographic data, tumor characteristics, medication, irradiation, follow up duration, and disease recurrence. Results The median age of the cohort was 48.2 years, with a female predominance (61.5%). Most PAs were macroadenomas (79.6%) and invasive (53.9%). Biochemical remission was achieved in 69.9%, while 19.2% exhibited biochemical discrepancy. Univariate analysis identified overall medication (pre- and/or postoperative), irradiation, and invasive PAs as significant factors associated with biochemical discordance. Logistic regression confirmed medication as the most influential predictor, with irradiation as a potential contributing factor. Disease recurrence was the only distinguishing factor between persistent acromegaly and biochemical discrepancy. Conclusion Overall medication use is the strongest predictor of biochemical discrepancy, with irradiation potentially contributing. No clear distinguishing factors between biochemical discordance and persistent acromegaly were identified, except from disease recurrence. Managing patients with biochemical discrepancy similarly to those with persistent acromegaly may be advisable. Further research is needed to refine treatment strategies.
Abstract Purpose Double or multiple pituitary adenomas account for only 1.6–3.3% of all corticotroph tumors. We sought to better understand the underlying molecular pathogenesis of two distinct corticotroph adenomas in … Abstract Purpose Double or multiple pituitary adenomas account for only 1.6–3.3% of all corticotroph tumors. We sought to better understand the underlying molecular pathogenesis of two distinct corticotroph adenomas in a 43-year-old female. Methods Two histopathologically confirmed corticotroph adenomas were submitted for whole-exome sequencing along with a matched blood sample. The functional effects of identified variants of uncertain significance on corticotroph tumor pro-opiomelanocortin transcription and proliferation were characterized. Results WES demonstrated a loss-of-function variant in the G-protein coupled receptor 162 [ GPR162 (R218*)] in the right corticotroph tumor, and a novel missense variant in ubiquitin specific peptidase 8 [ USP8 (P681Q)] in the left tumor. Compared to wild-type GPR162 which potently suppressed POMC transcription, the stop-gain variant (R218*) exhibited reduced inhibitory effect. The novel USP8 variant (P681Q) found in the contra-lateral tumor led to increased POMC transcription although weaker than the well characterized hotspot variant S718P, and did not affect EGFR ubiquitin. Interestingly, the patient also had a germline variant in the 21-alpha-hydroxylase gene ( CYP21A2 p.A392T) though without clinical features of congenital adrenal hyperplasia. Conclusion We report, for the first time, the genetic profiles of a patient with dual pituitary corticotroph tumors, identifying a stop-gain variant in GPR162 in one tumor and a novel USP8 variant (S718P) in the other. While both somatic variants increased POMC expression, only GPR162 R218* affected proliferation. We hypothesize that alterations in adrenal steroidogenesis due to the CYP21A1 mutation may have reduced negative feedback on corticotroph cells and acted in a permissive way to facilitate corticotroph tumorigenesis.

Midazolam

2025-06-21
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Pituitary surgical intervention remains the preferred treatment for Cushing's disease (CD) while postoperative venous thromboembolism (VTE) is a significant risk. Whether to prescribe pharmacological thromboprophylaxis presents a clinical dilemma, balancing … Pituitary surgical intervention remains the preferred treatment for Cushing's disease (CD) while postoperative venous thromboembolism (VTE) is a significant risk. Whether to prescribe pharmacological thromboprophylaxis presents a clinical dilemma, balancing the benefit of reducing VTE risk with the potential for increasing hemorrhagic events in these patients. Currently, strong evidence and established protocols for routine pharmacological thromboprophylaxis in this population are lacking. Therefore, a randomized, controlled trial is warranted to determine the efficacy and safety of combined pharmacological and mechanical thromboprophylaxis in reducing postoperative VTE risk in patients with CD. This investigator-initiated, multi-center, prospective, randomized, open-label trial with blinded outcome assessment aims to evaluate the efficacy and safety of combined pharmacological and mechanical thromboprophylaxis compared to mechanical thromboprophylaxis alone in postoperative patients with CD. A total of 206 patients diagnosed with CD who will be undergoing transsphenoidal surgery will be randomized in a 1:1 ratio to receive either combined pharmacological and mechanical thromboprophylaxis (intervention) or mechanical thromboprophylaxis only (control). The primary outcome is the risk of VTE within 12 weeks following surgery. This trial represents a significant milestone in evaluating the efficacy of combined pharmacological and mechanical prophylaxis in reducing VTE events in postoperative CD patients. ClinicalTrials.gov Identifier: NCT04486859, first registered on 22 July 2020.
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Cushing syndrome (CS) is a rare endocrine disorder characterized by excessive secretion of glucocorticoids, leading to a variety of clinical manifestations, comorbidities, and increased mortality despite treatment. Despite advances in … Cushing syndrome (CS) is a rare endocrine disorder characterized by excessive secretion of glucocorticoids, leading to a variety of clinical manifestations, comorbidities, and increased mortality despite treatment. Despite advances in imaging modalities and biochemical testing, the diagnosis and management of CS remains challenging. Several tests are used to confirm the diagnosis of CS, including urinary free cortisol measurements, dexamethasone suppression tests (1 mg, 2 mg, and 8 mg), and nocturnal salivary cortisol measurements. However, each of these tests has some limitations, making the diagnosis of CS. In this paper, we explore the potential of state-of-the-art machine learning algorithms as a clinical decision support system for analyzing and classifying CS. Our aim is to use advanced machine learning methods to analyze the accuracy rates of diagnostic tests and identify the most sensitive tests for diagnosing CS. In this study, we performed binary classification based on data from 278 patients with CS (CS+) and 220 healthy patients (CS-). We developed a linear mathematical model with high predictive ability, achieving a classification accuracy of 97.03% and a Kappa value of 94.05%. The correlation graph shows that CS has strong positive relationships with 2 mg (78.8%), 1 mg (76.9%), and mc (72.1%), and moderate positive correlations with 8 mg (45%) and saliva (45.4%). In contrast, gender has almost no correlation with CS, so it was removed from the dataset. As a result, the model achieves an overall classification accuracy of 97.03%. Finally, we converted the linear model into a mobile application for use by specialist doctors in the field of endocrinology. Traditional diagnostic methods can be time-consuming and require specialized medical expertise. Recently, advances in machine learning and mobile technology have opened new avenues for improving diagnostic accuracy and accessibility. This study explores the integration of machine learning algorithms into a mobile application designed to assist healthcare professionals and patients in the diagnosis of CS.
Isolated adrenocorticotropic hormone deficiency (IAD) secondary to chronic alcohol abuse is an extremely rare condition, often underdiagnosed due to nonspecific clinical features overlapping with complications of alcoholism. A 42-year-old Brazilian … Isolated adrenocorticotropic hormone deficiency (IAD) secondary to chronic alcohol abuse is an extremely rare condition, often underdiagnosed due to nonspecific clinical features overlapping with complications of alcoholism. A 42-year-old Brazilian male with chronic alcohol abuse was admitted for hematemesis and severe hyponatremia refractory to conventional therapy. Biochemical evaluation revealed profound hypocortisolism with inappropriately low adrenocorticotropic hormone (ACTH) levels and normal pituitary function and imaging. A comprehensive workup ruled out other potential causes of hyponatremia. Diagnosis of IAD was hypothesized. Hydrocortisone replacement therapy led to rapid clinical improvement and normalization of sodium levels. This case highlights the importance of considering IAD in chronic alcoholics with unexplained hyponatremia. Early diagnosis and corticosteroid replacement therapy are essential to prevent life-threatening complications.

Galactorrhée

2025-06-19
Juliette Mosseri , Geoffroy Canlorbe , Cathérine Uzan | EMC - Traité de médecine AKOS
Clinically nonfunctioning pituitary tumors (CNFPTs) typically do not cause hormonal excess, progress insidiously, and are often large and invasive at presentation. Complete resection is frequently not attainable; radiotherapy (RT) may … Clinically nonfunctioning pituitary tumors (CNFPTs) typically do not cause hormonal excess, progress insidiously, and are often large and invasive at presentation. Complete resection is frequently not attainable; radiotherapy (RT) may effectively limit growth but carries a significant risk of hypopituitarism. Medical therapy with dopamine D2 receptor agonists and/or somatostatin analogs has been explored in CNFPTs but have yielded inconsistent results, and there is an unmet need for novel efficacious and safe medical therapies. The authors used the PubMed database to identify and review articles published from January 1982 to July 2024, that discussed the medical treatment of CNFPTs. The most commonly studied medical therapies were somatostatin receptor ligands (SRLs) and dopamine D2 receptor agonists. Of 111 patients with CNFPTs treated with SRLs, 31 (28%) exhibited tumor shrinkage. Following dopamine agonist treatment in 355 patients, tumor shrinkage occurred in 113 (32%), tumor stabilization in 182 (51%), and tumor growth in 60 (17%). The efficacy of other less commonly employed therapies such as GnRH analogs, PRRT, and temozolomide was also reviewed. Efficacious and safe medical therapies evaluated in robust randomized placebo-controlled clinical trials are needed to improve the management of CNFPTs.
The parasellar ligaments have been previously described in cadaver specimens and intraoperatively, but identification on MR imaging has eluded radiologists. Using high-resolution T2-weighted MR imaging, we identified the parasellar ligaments … The parasellar ligaments have been previously described in cadaver specimens and intraoperatively, but identification on MR imaging has eluded radiologists. Using high-resolution T2-weighted MR imaging, we identified the parasellar ligaments as T2-hypointense, bandlike structures that emanate from the medial wall of the cavernous sinus. Subsequent dissection of the same specimen provided matching anatomic images of the parasellar ligaments identified on MRI. This imaging finding is important because resection of the medial wall of the cavernous sinus has been tied to improved outcomes for gross total resection and endocrinologic remission of functioning pituitary adenomas.
<title>Abstract</title> Background Non-adenomatous pituitary lesions are rare and often misdiagnosed as pituitary adenomas. Accurate diagnosis is crucial to avoid unnecessary surgery and to tailor treatment approaches appropriately Methods A retrospective … <title>Abstract</title> Background Non-adenomatous pituitary lesions are rare and often misdiagnosed as pituitary adenomas. Accurate diagnosis is crucial to avoid unnecessary surgery and to tailor treatment approaches appropriately Methods A retrospective cohort study was conducted at Nizam's Institute of Medical Sciences, Hyderabad, from 2019 to 2024, including 18 patients with histologically confirmed non-adenomatous pituitary lesions. Clinical features, hormonal profiles, radiological characteristics, interventions, and outcomes were analyzed. Results Of the 18 patients, 77.7% were female. Mean age was 42.1 years and mean follow-up was 19.17 months. Presenting symptoms included neurological deficits in 83.4% and endocrine disturbances in 88.8%. Histopathological classifications included inflammatory/granulomatous (55.5%), Rathke’s cleft cysts (22.2%), neurohypophyseal tumors (11.1%), and arachnoid cysts (11.1%). Biopsy was performed in 33.3%, transsphenoidal surgery in 61.1%, and craniotomy in 5.5%. Hormone replacement was needed in 77.7% post-intervention. Mortality was 11.1%, and recurrence was 5.5%. Conclusion Non-adenomatous pituitary lesions remain an elusive set of entities to neurosurgeons, radiologists and endocrinologists alike due to their varied presentations and subtle imaging findings that may be overlooked and mistook as the more common pituitary adenomas. An attention to demographic clues and knowledge of clinico-radiology of these lesions maybe helpful to identify these “zebras” from the usual herd of “horses” that are pituitary adenomas.
Background Pituitary metastases are rare, accounting for only 1% of all pituitary tumor resections. Prostate adenocarcinoma, a common malignancy in men, seldom metastasizes to the pituitary gland, with only a … Background Pituitary metastases are rare, accounting for only 1% of all pituitary tumor resections. Prostate adenocarcinoma, a common malignancy in men, seldom metastasizes to the pituitary gland, with only a few reported cases. Given their rarity and non-specific clinical presentation, pituitary metastases are often mistaken for primary sellar lesions. Advanced imaging techniques, including Prostate-Specific Membrane Antigen (PSMA) positron emission tomography (PET)/Computed Tomography (CT) and molecular diagnostics, such as DNA methylation profiling, can aid in accurate diagnosis and differentiation from pituitary adenomas. Case presentation We report the case of a 71-year-old male with a history of prostate adenocarcinoma who presented with biochemical recurrence and underwent PSMA PET/CT imaging, revealing intense tracer uptake in the pituitary gland. Magnetic Resonance Imaging (MRI) findings were suggestive of a pituitary macroadenoma, and the patient developed bitemporal hemianopia, necessitating transsphenoidal surgical resection. Histopathological and immunohistochemical analyses were not compatible with a primary pituitary lesion, prompting further investigation via DNA methylation profiling. The analysis revealed a DNA-methylation signature consistent with prostate carcinoma, confirming pituitary metastasis. The patient subsequently received systemic treatment with androgen deprivation therapy, abiraterone, and docetaxel, achieving an excellent biochemical and imaging response. Conclusion This case highlights the importance of considering metastatic prostate cancer in the differential diagnosis of pituitary lesions, particularly when PSMA PET/CT shows focal uptake in atypical locations. Integration of histopathological, immunohistochemical, and molecular techniques, such as DNA-methylation profiling, was essential for confirming the diagnosis. Clinicians should remain vigilant for atypical metastatic presentations and leverage advanced diagnostic tools to ensure accurate diagnosis and optimal patient management.
ABSTRACT A 12‐year‐old, male Labrador Retriever was presented because of polyuria, polydipsia, polyphagia, joint pain, and physical features consistent with acromegaly. Circulating insulin‐like growth factor‐1 (IGF‐1) concentration was increased (&gt; … ABSTRACT A 12‐year‐old, male Labrador Retriever was presented because of polyuria, polydipsia, polyphagia, joint pain, and physical features consistent with acromegaly. Circulating insulin‐like growth factor‐1 (IGF‐1) concentration was increased (&gt; 1000 ng/mL; reference interval [RI], 42–449), suggestive of hypersomatotropism. An abnormal low‐dose dexamethasone suppression test and increased circulating adrenocorticotropic (ACTH) concentration indicated pituitary‐dependent hypercortisolism. Computed tomography identified an enlarged pituitary gland. Treatment with cabergoline initially decreased circulating IGF‐1 and ACTH concentrations and urinary cortisol‐to‐creatinine ratio (UCCR), with a notable reduction in acromegalic physical features. However, 7 months after the start of cabergoline treatment, IGF‐1, ACTH, and UCCR had increased again, although pituitary gland size remained stable. Because of worsening joint pain, euthanasia was performed. On necropsy, double immunohistochemistry identified pituitary tumor cells with cytoplasmic co‐expression of both growth hormone (GH) and ACTH, consistent with a monomorphic plurihormonal macroadenoma. This case shows that concurrent hypersomatotropism and hypercortisolism can occur in dogs caused by a plurihormonal pituitary adenoma.
Background: The main mechanism of optic nerve damage in patients with pituitary adenoma (PA) is the pressure of optic chiasm. The retinal nerve fiber layer (RNFL), the ganglion cell layer … Background: The main mechanism of optic nerve damage in patients with pituitary adenoma (PA) is the pressure of optic chiasm. The retinal nerve fiber layer (RNFL), the ganglion cell layer (GCL)+, and GCL++ thickness measurement by optical coherence tomography (OCT), visual function evaluation, and magnetic resonance imaging (MRI) can be used to predict visual function recovery. In our study, we investigated the associations between visual acuity (VA), visual field (VF), RNFL, GCL changes, and the findings of MRI in patients with PA. Methods: This study was conducted in the Departments of Ophthalmology and Neurosurgery of the Lithuanian University of Health Sciences Hospital. A total of 25 patients diagnosed with PA were included in the study group, and 27 healthy subjects were included in the control group. The thickness of the RNFL and ganglion cell layer (GCL+, GCL++) and optic nerve disc diameter was analysed with OCT. Moreover, an MRI was performed for patients with PA. Results: The RNFL thickness around the optic disk measured preoperatively was reduced significantly in the temporal quadrant in PA patients compared with the control group (median (min; max); mean rank: 73.5 (52; 109); 58.39 vs. 69.5 (16; 168); 46.14; p = 0.038). We found that it was reduced significantly only in the inferior quadrant of the macro-PA group compared to the micro-PA group (median (min; max); mean rank: 99.5 (61; 115); 21.07 vs. 106.5 (90; 121); 32.15), p = 0.008, respectively). The RNFL thickness was reduced significantly only in the inferior quadrant of the non-active PA group compared to the active PA group (median (min; max); mean rank: 118.5 (49; 144); 17.42 vs. 130.5 (77; 156); 28.05), p = 0.028, respectively). RNFL thickness was reduced significantly only in the temporal quadrant in the PA with suprasellar extension group compared to the PA without suprasellar extension group (median (min; max); mean rank: 67.5 (16; 99); 21.66 vs. 72 (58; 168); 30.39), p = 0.036, respectively). Furthermore, GCL++ thickness was reduced significantly in total and in superior and inferior sectors of the PA with suprasellar extension group compared to the PA without suprasellar extension group (median (min; max); mean rank: 98.5 (57; 113); 21.8; 101 (61; 121); 21.48 and 102.5 (59; 116); 21.71 vs. 103.5 (95; 115); 30.2; 106.5 (90; 115); 30.61 and 104.5 (95; 113); 30.32), p = 0.043; p = 0.028 and p = 0.038, respectively). In the control group, significant positive correlations were found between optic disc area and the total RNFL thickness (r = 0.440, p < 0.001). In the PA group, significant correlations were observed between optic rim area and total RNFL thickness (r = 0.493, p < 0.001) and all quadrants, with the strongest in the nasal quadrant (r = 0.503, p < 0.001). A moderate to strong negative correlation was found between visual field (VF) defects and RNFL thickness, with the strongest correlation observed in the superior quadrant. Conclusions: OCT offers a detailed insight into the microscopic structural and functional changes throughout the entire visual pathway in patients with PA. Our findings demonstrate a significant negative correlation between RNFL thickness and visual field defects, highlighting the clinical relevance of OCT measurements in visual function assessment. Moreover, the results suggest that optic rim area may be a more reliable indicator of RNFL thickness variations than optic disc area in patients with PA.
Francis Deng | Radiopaedia.org
BACKGROUND Carcinosarcomatous transformation is a rare complication following radiotherapy for pituitary adenomas (PAs). OBSERVATIONS The authors report what they believe to be the first case of a benign nonfunctioning PA … BACKGROUND Carcinosarcomatous transformation is a rare complication following radiotherapy for pituitary adenomas (PAs). OBSERVATIONS The authors report what they believe to be the first case of a benign nonfunctioning PA developing into a carcinosarcoma after surgery and radiotherapy. LESSONS Radiotherapy for PAs should account for the risk of secondary intracranial tumors, with long-term follow-up recommended for treated patients. Combined therapeutic strategies may be considered for radiation-induced sarcomas, and comprehensive molecular pathological studies of radiation-induced tumors are imperative for advancing understanding and management. https://thejns.org/doi/10.3171/CASE24765
Abstract: Craniopharyngiomas (CPMs) are uncommon embryonic anomalies located in the sellar and parasellar regions, characterized by their exceedingly low histological grade (WHO I). The incidence of 1-3 new cases per … Abstract: Craniopharyngiomas (CPMs) are uncommon embryonic anomalies located in the sellar and parasellar regions, characterized by their exceedingly low histological grade (WHO I). The incidence of 1-3 new cases per million each year globally. The aim of this systematic review is to conduct a comparative analysis of EEN and TC therapies, irrespective of the total number of patients who received alternative interventions. Two primary subtypes of CPMs have been recognized: papillary, predominantly observed in adults, and adamantinomatous, primarily found in children and resembling neoplasms that generate enamel-like structures in the oropharynx. Adults with the adamantinomatous variant are associated with mutations in the β-catenin gene, a key mediator of the Wnt CTNNB1 pathway, while adults with the BRAF V600E mutation are frequently associated with the papillary subtype. Materials and Methods: Following that, the scientific literature is examined using the Preferred Reporting Items for Systematic Reviews (PRISMA) Direct and PubMed/MEDLINE through January-August 2024. The IBM SPSS Statistics for Windows, Version 26.0 (released 2020; IBM Corp., Armonk, New York, United States) and EXCEL criteria were used for the statistical study. Results: This comprehensive analysis includes 3076 patients with craniopharyngiomas. Among these, 1339 patients had endoscopic endonasal resections, whereas 591 patients underwent open transcranial resections. For this form of pathology, endoscopic resection was the more favorable alternative. Conclusion: We discovered that craniopharyngiomas Because total eradication is not achieved, recurrences are common, therefore elimination by new intervention, plus adjuvant radiation or chemotherapy, plus surgery.
Introduction: Cushing’s disease in young-onset cases is a rare and diagnostically challenging endocrinopathy, most commonly caused by an adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma. Early recognition and management are crucial to … Introduction: Cushing’s disease in young-onset cases is a rare and diagnostically challenging endocrinopathy, most commonly caused by an adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma. Early recognition and management are crucial to preventing long-term complications associated with hypercortisolism. This report highlights the case of a pediatric patient with Cushing’s disease, emphasizing diagnostic challenges and treatment considerations. Case Presentation: We present the case of a 14-year-old male from Kashmore with a four-year history of progressive truncal obesity, growth failure, and episodic headaches. Clinical evaluation revealed hallmark cushingoid features, including moon facies, dorsocervical and supraclavicular fat accumulation, acanthosis nigricans, and hypertension. A hormonal assessment confirmed hypercortisolism, and magnetic resonance imaging (MRI) revealed a 0.9 cm pituitary microadenoma. Clinical Discussion: Cushing’s disease in pediatric patients presents unique diagnostic and therapeutic challenges due to its rarity and insidious onset. First-line treatment for pituitary-dependent Cushing’s disease is transsphenoidal surgery; however, in cases where surgery is declined or contraindicated, alternative therapies such as medical management and radiosurgery become viable options. In this case, Gamma Knife Radiosurgery (GKRS) led to significant clinical improvements, including weight loss and biochemical remission. However, the patient later developed adrenal insufficiency, necessitating immediate steroid therapy, a known risk associated with successful treatment of hypercortisolism. Conclusion: GKRS has proven to be an effective treatment for Cushing’s disease in younger individuals, both as a standalone therapy and as an adjunct to other interventions. This case underscores the importance of individualized treatment approaches and close post-treatment monitoring for potential complications such as adrenal insufficiency.
Background: Secondary adrenal insufficiency is a rare condition in which the adrenal glands fail to produce sufficient cortisol and aldosterone, commonly referred to as Addison’s disease. We report a case … Background: Secondary adrenal insufficiency is a rare condition in which the adrenal glands fail to produce sufficient cortisol and aldosterone, commonly referred to as Addison’s disease. We report a case of Addison’s disease coexisting with chronic plaque psoriasis, both requiring medical management. Case Presentation: A 40-year-old male patient was admitted to the ICU with a known history of chronic plaque psoriasis. He had been undergoing long-term corticosteroid therapy (dexamethasone and betamethasone) for approximately four months, which he abruptly discontinued one week prior to admission. He presented with symptoms of easy fatigability, reduced appetite, and persistent vomiting. Laboratory tests revealed abnormal cortisol and ACTH levels. Contrast-enhanced CT (CECT) of the abdomen and pelvis showed bilaterally atrophic adrenal glands, likely secondary to a chronic autoimmune disorder. Based on these findings, a diagnosis of secondary adrenal insufficiency was confirmed. The patient responded well to steroid replacement therapy with hydrocortisone. Conclusion: This case report highlights steroid-induced secondary adrenal insufficiency presenting as Addison’s disease in a patient with chronic plaque psoriasis. Prolonged unsupervised use of oral dexamethasone and betamethasone led to HPA axis suppression, confirmed by low ACTH levels and bilateral adrenal atrophy. Delayed diagnosis due to limited rural healthcare access emphasizes the importance of awareness and monitoring in long-term steroid users. Lifelong follow-up, medication adherence, and supervised tapering are essential to prevent recurrence.
BACKGROUND AND OBJECTIVES: Enhanced recovery after surgery protocols have resulted in a paradigm change in perioperative care with applications in cranial neurosurgery. Protocols have been implemented to increase rates of … BACKGROUND AND OBJECTIVES: Enhanced recovery after surgery protocols have resulted in a paradigm change in perioperative care with applications in cranial neurosurgery. Protocols have been implemented to increase rates of early discharge with length of stay being an important metric after pituitary adenoma (PA) resection. The aim of this study was to assess characteristics associated with early discharge on postoperative day 1 or 2 (POD 1-2) after endoscopic endonasal surgery (EES) for PA. METHODS: An analysis was performed of 524 patients undergoing EES for PA resection from December 2012 to December 2022. Patients discharged POD 1 were compared with POD &gt;2 patients to determine associations with tumor and patient characteristics, preoperative symptoms, and postoperative complications. RESULTS: Among 524 patients (mean age 51.7 years, 55.3% female), 267 (51.0%) were discharged POD 1-2. These patients were less likely to present with headaches (odds ratio [OR] = 0.61, 95% CI: 0.43-0.86, P = .007), vision loss (OR = 0.46, 95% CI: 0.32-0.65, P &lt; .001), and cranial nerve palsy (OR = 0.42, 95% CI: 0.21-0.83, P = .02). Early discharge patients had smaller (22.7 vs 27.7 mm diameter, P &lt; .001) and less invasive tumors (Knosp 3-4: OR = 0.66, 95% CI: 0.45-0.97, P = .04). Late discharge patients were more likely to have intraoperative cerebrospinal fluid leaks (OR = 2.40, 95% CI: 1.65-3.50, P &lt; .001), transient diabetes insipidus (OR = 4.94, 95% CI: 2.23-10.88, P &lt; .001), and more often received additional treatment such as hydrocortisone (OR = 3.23, 95% CI: 2.14-4.86, P &lt; .001) or reoperation (OR = 11.18, 95% CI: 2.59-48.34, P &lt; .001). In our multivariable analysis, smaller tumors were positively associated with early discharge (OR = 0.96, 95% CI: 0.94-0.98 per mm, P &lt; .001), whereas transient diabetes insipidus was inversely associated (OR = 0.27, 95% CI: 0.10-0.53, P &lt; .001). There was no significant association between readmission and early vs late discharge (8.3% vs 13.3%, P = .09). CONCLUSION: Tumor characteristics and presenting symptoms may assist in determining patients who are appropriate for early discharge after EES for PA resection. POD 1-2 discharge seems to be a practical option for many patients after EES, without additional risk of readmission or further complications.