Autoimmune Disorders Associated With Surgical Remission of Cushing's Disease : A Cohort Study.

Background: Glucocorticoids suppress inflammation. Autoimmune disease may occur after remission of Cushing's disease (CD). However, the development of autoimmune disease in this context is not well described.

Health-related quality of life analyses in nonfunctioning pituitary macroadenoma patients identifies at-risk populations.

Purpose: The quality of life (QoL) impact of multidisciplinary treatment for patients with nonfunctioning pituitary macroadenomas (NFPMA) is unclear. We sought to investigate associations between patient factors, clinical data, and patient-reported QoL in patients with NFPMA.

Methods: Patients with treated NFPMA and > 1 year of follow up after transsphenoidal surgery (TSS) and with no evidence of progressive disease were evaluated utilizing the following patient-reported outcome measures: RAND-36-Item Health Survey, Multidimensional Fatigue Inventory, Cognitive Failures Questionnaire.

Pituitary surgery in Cushing's disease: first line treatment and role of reoperation.

Cushing's disease is the most common cause of endogenous hypercortisolemia, and transsphenoidal surgery remains the first line therapy for removal of the ACTH-secreting adenoma. While post-operative remission rates are high in experienced hands, there remains a 2% risk of recurrence per year. Patients with the highest chance for cure are those with small, non-invasive tumors that are visible on pre-operative MRI and identified during surgery and are performed by high-volume pituitary neurosurgeons.

High Histone Deacetylase 2/3 Expression in Non-Functioning Pituitary Tumors.

Epigenetic modification of chromatin is involved in non-malignant pituitary neoplasia by causing abnormal expression of tumor suppressors and oncogenes. These changes are potentially reversible, suggesting the possibility of targeting tumor cells by restoring the expression of epigenetically silenced tumor suppressors. The role of the histone deacetylase (HDAC) family in pituitary tumorigenesis is not known.

Sprouting Angiogenesis in Human Pituitary Adenomas.

Introduction: Angiogenesis in pituitary tumors is not fully understood, and a better understanding could help inform new pharmacologic therapies, particularly for aggressive pituitary tumors.

Materials And Methods: 219 human pituitary tumors and 12 normal pituitary glands were studied. Angiogenic genes were quantified by an angiogenesis qPCR array and a TaqMan probe-based absolute qPCR.

Quality of life after long-term biochemical control of acromegaly.

Purpose: To assess long-term quality of life (QoL) in patients with sustained biochemical control of acromegaly, comparing those receiving vs not receiving pharmacotherapy (primary analysis); to assess change in QoL over time (secondary analysis).

Methods: Cross-sectional study, with a secondary longitudinal component, of 58 patients with biochemically controlled acromegaly. All had participated in studies assessing QoL years previously, after having undergone surgery ± radiotherapy.

Recurrent Acromegaly in a Patient With a Mutation.

Background/objective: is a cell-cycle checkpoint kinase and is part of the ATM-CHEK2-p53 cascade, which is protective against carcinogenesis. We describe a germline CHEK2 mutation in a patient with acromegaly and other tumors.

Case Report: We present a woman with a germline 110delC mutation previously diagnosed with fibroadenoma of the breast and papillary thyroid carcinoma.

The Prolactin per Unit Tumor Volume Ratio Accurately Distinguishes Prolactinomas From Secondary Hyperprolactinemia due to Stalk Effect.

Objective: The prolactin levels alone are insufficient to distinguish between some cases of prolactinomas and stalk effect. We aimed to formally characterize the relationship between serum prolactin and prolactinoma volume, determine a cutoff for prolactin/mm that accurately distinguishes prolactinomas from stalk effect, and validate this cutoff in a cohort selected to include ambiguous prolactin values ranging from 50 to 150 ng/mL.

Methods: We used the Research Patient Data Registry and transsphenoidal surgery database in our institution to retrospectively identify adult patients with clinically nonfunctioning (NF) tumors (primary analysis, n = 279; validation cohort, n = 10) and prolactinomas (primary analysis, n = 94; validation cohort, n = 18).

Consensus on diagnosis and management of Cushing's disease: a guideline update.

Cushing's disease requires accurate diagnosis, careful treatment selection, and long-term management to optimise patient outcomes. The Pituitary Society convened a consensus workshop comprising more than 50 academic researchers and clinical experts to discuss the application of recent evidence to clinical practice. In advance of the virtual meeting, data from 2015 to present about screening and diagnosis; surgery, medical, and radiation therapy; and disease-related and treatment-related complications of Cushing's disease summarised in recorded lectures were reviewed by all participants.

Multidisciplinary management of acromegaly: A consensus.

The 13th Acromegaly Consensus Conference was held in November 2019 in Fort Lauderdale, Florida, and comprised acromegaly experts including endocrinologists and neurosurgeons who considered optimal approaches for multidisciplinary acromegaly management. Focused discussions reviewed techniques, results, and side effects of surgery, radiotherapy, and medical therapy, and how advances in technology and novel techniques have changed the way these modalities are used alone or in combination. Effects of treatment on patient outcomes were considered, along with strategies for optimizing and personalizing therapeutic approaches.

Effects of growth hormone receptor antagonism and somatostatin analog administration on quality of life in acromegaly.

Objective: Acromegaly is associated with impaired quality of life (QoL). We investigated the effects of biochemical control of acromegaly by growth hormone receptor antagonism vs somatostatin analog therapy on QoL.

Design: Cross-sectional.

Clinical MEN-1 Among a Large Cohort of Patients With Acromegaly.

Context: Clinical multiple endocrine neoplasia type 1 (MEN-1) is diagnosed by the presence of at least 2 MEN-1-associated tumors. Many patients with acromegaly and clinical MEN-1 yield negative testing for MEN1 mutations. While cases of acromegaly and primary hyperparathyroidism (PHP) with negative genetic testing have been reported, its prevalence among patients with acromegaly is undetermined, and the clinical presentation has not been well characterized.

Stereotactic Radiation as Salvage Therapy for Recurrent Rathke Cleft Cysts.

Background: Rathke cleft cysts (RCCs) are sellar-based cystic lesions that are often found incidentally but occasionally become symptomatic with significant visual and/or endocrine deficits. The standard of treatment is surgery, but rare cases of multiply recurrent RCCs can be refractory to surgical drainage, leading to significant morbidity.

Objective: To demonstrate the safety and feasibility of fractionated stereotactic radiotherapy (SRT) as salvage therapy in multiply recurrent RCCs refractory to surgical drainage.

MRI texture analysis in acromegaly and its role in predicting response to somatostatin receptor ligands.

Purpose: Given the paucity of reliable predictors of tumor recurrence, progression, or response to somatostatin receptor ligand (SRL) therapy in acromegaly, we attempted to determine whether preoperative MR image texture was predictive of these clinical outcomes. We also determined whether image texture could differentiate somatotroph adenomas from non-functioning pituitary adenomas (NFPAs).

Methods: We performed a retrospective study of patients with acromegaly due to a macroadenoma who underwent transsphenoidal surgery at our institution between 2007 and 2015.

Biochemical Control in Acromegaly With Multimodality Therapies: Outcomes From a Pituitary Center and Changes Over Time.

Purpose: To determine the prevalence of insulin-like growth factor-1 (IGF-1) normalization with long-term multimodality therapy in a pituitary center and to assess changes over time.

Methods: Patients with acromegaly (N = 409), with ≥1 year of data after surgery and at least 2 subsequent clinic visits were included in long-term analysis (N = 266). Biochemical data, clinical characteristics, and therapeutic interventions were reviewed retrospectively.

Intraoperative MRI for Pituitary Adenomas.

Since the 1990s, MRI scanners have been incorporated into the operating room environment. Studies of the use of intraoperative MRI (iMRI) for pituitary adenomas have shown that images are highly sensitive and specific for residual tumor detection, especially at higher Tesla magnet strengths. Given this accuracy, iMRI also increases the rates of achieving gross total resection (GTR).

The Utility of Intraoperative Cytological Smear and Frozen Section in the Surgical Management of Patients with Cushing's Disease due to Pituitary Microadenomas.

Cushing's disease (CD) is most commonly caused by a microadenoma, which at surgical exploration may not provide adequate tissue for pathologic diagnosis using standard techniques. We wished to determine the accuracy of intraoperative pathologic examination and whether the addition of intraoperative cytology increased the diagnostic yield. We reviewed the pathology reports from 403 operations on 341 patients with CD microadenomas from a single institution.

MRI texture analysis as a predictor of tumor recurrence or progression in patients with clinically non-functioning pituitary adenomas.

Background: There are limited predictors of prognosis in patients with clinically non-functioning pituitary adenomas (NFPAs). We hypothesized that MRI texture analysis may predict tumor recurrence or progression in patients with NFPAs undergoing transsphenoidal pituitary surgery (TSS).

Objective: To characterize texture parameters on preoperative MRI examinations in patients with NFPAs in relation to prognosis.

Cushing's disease in older patients: Presentation and outcome.

Background: To define the symptoms, signs and treatment outcomes in a population of older patients with Cushing's disease (CD).

Methods: We analysed the clinical presentation and treatment outcomes in 45 CD patients older than 60 years, in comparison with 90 CD patients younger than 60, and a control group of 45 older patients with nonfunctioning pituitary adenomas. We reviewed preoperative clinical characteristics, medical comorbidities, imaging findings and endocrine testing as well as surgical and endocrine outcomes.

Characterization of cyclic Cushing's disease using late night salivary cortisol testing.

Objective: To characterize a cohort of patients with cyclic Cushing's disease (CD) in comparison with noncyclic CD using late night salivary cortisol (LNSC) and examine the diagnostic sensitivity of LNSC in comparison with that of 24-hour urine-free cortisol (UFC) in this population.

Design: Retrospective study of patients with CD seen in our institution between 2008 and 2017.

Patients: A total of 205 patients, including 17 (8%) with cyclic CD (based on a minimum of 3 peaks and 2 troughs in cortisol levels).

Body Composition and Ectopic Lipid Changes With Biochemical Control of Acromegaly.

Context: Acromegaly is characterized by growth hormone (GH) and insulinlike growth factor-1 (IGF-1) hypersecretion, and GH and IGF-1 play important roles in regulating body composition and glucose homeostasis.

Objective: The purpose of our study was to investigate body composition including ectopic lipids, measures of glucose homeostasis, and gonadal steroids in patients with active acromegaly compared with age-, body mass index (BMI)-, and sex-matched controls and to determine changes in these parameters after biochemical control of acromegaly.

Design: Cross-sectional study of 20 patients with active acromegaly and 20 healthy matched controls.

Surgical debulking of pituitary adenomas improves responsiveness to octreotide lar in the treatment of acromegaly.

Background: Studies comparing primary medical treatment of acromegaly with surgery are often non-randomized, and not stratified by illness severity. We prospectively compared primary medical therapy with pituitary surgery in patients with acromegaly. All patients had macroadenomas, at least one random human growth hormone (GH) level ≥12.

Dopamine Agonists Can Reduce Cystic Prolactinomas.

Context: Cystic prolactinomas are considered resistant to volume reduction by dopamine agonists (DAs). Although several individual case reports and small case series have suggested that DAs may reduce these lesions, larger series using standardized imaging metrics are lacking.

Objective: The objectives of the study were to assess the efficacy of DAs on cyst size in patients with predominantly cystic prolactinomas and to characterize the clinical course and treatment outcomes in these patients.