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Objective: To investigate the impact of musculoskeletal (MSK)‐related symptoms on the quality of life of patients with multiple endocrine neoplasia Type 2b (MEN2B). Design: An online survey was distributed by the Association for Multiple Endocrine Neoplasia Disorders (AMEND) to their members and worldwide via a social media group for MEN2B patients. Methods: The survey consisted of a detailed questionnaire analysing the MSK‐related symptoms and structural deformities of MEN2B patients and their impact on patient's lives. Participants: Forty‐eight participants completed the survey. Results: Participants reported several musculoskeletal complaints; the most prevalent being musculoskeletal weakness at 73% (n = 35) and pain 58% (n = 28). The median pain score was 7 (interquartile range [IQR]: 5–8) and the frequency of pain was daily in 44% (n = 15) and constant in 21% (n = 7). Structural complaints were common with 63% (n = 30) stating their physique was 'different' and 40% (n = 19) describing marfanoid body features. Spinal curvature and foot deformities were the commonest structural abnormalities with scoliosis 70% (n = 16) and pes cavus 63% (n = 22) prevailing. Dental problems were mentioned by 69% (n = 33) with interdental spacing being the most common complaint at 61% (n = 20). The musculoskeletal symptoms of MEN2B had a median impact of 6 (IQR: 3–9) on quality of life (QOL) with structural deformities 53% (n = 18) and pain 26% (n = 9) listed as having the highest impact. Poor MSK health affected exercise, work and mobility. Conclusions: We report a high prevalence of musculoskeletal‐related complaints in MEN2B which significantly affects QOL. This suggests a need to provide better holistic care including a multidisciplinary team with physiotherapist, orthopaedic and dental specialist input. [ABSTRACT FROM AUTHOR]

Objective: Acromegaly is a condition characterized by an overproduction of growth hormone which infers high morbidity and mortality if left untreated. The objective of this review is to analyse and appraise the current evidence for the generalized use of preoperative medications and the various surgical approaches as described in the literature. Design: A thorough search from MEDLINE via PubMed, EMBASE, and Cochrane Library has been performed which identified a total of 37 papers. Conclusion: The preoperative use of somatostatin receptor agonists (SAs) in acromegaly is a controversial topic with current guidelines suggesting against their generalized routine use. Most authors noticed an insignificant long‐term remission of acromegaly when given SAs compared with nil preoperative therapy, except for invasive macroadenomas as SAs have been found to reduce the tumour volume and aid towards the total resection of the adenoma. Furthermore, according to the evidence available, endoscopic transsphenoidal surgery is the optimum method for hypophysectomy in terms of its remission and safety profile. [ABSTRACT FROM AUTHOR]

Objective: Hypertension cure following adrenalectomy in unilateral primary aldosteronism is not guaranteed. Its likelihood is associated with pre‐operative parameters, which have been variably combined in six different predictive scoring systems. The relative performance of these systems is currently unknown. The objective of this work was to identify the best performing scoring system for predicting hypertension cure following adrenalectomy for primary aldosteronism. Design: Retrospective analysis in a single tertiary referral centre. Patients: Eighty‐seven adult patients with unilateral primary aldosteronism who had undergone adrenalectomy between 2004 and 2018 for whom complete data sets were available to calculate all scoring systems. Measurements: Prediction of hypertension cure by each of the six scoring systems. Results: Hypertension cure was achieved in 36/87 (41.4%) patients within the first post‐operative year, which fell to 18/71 (25.4%) patients at final follow‐up (median 53 months, P =.002). Analysis of receiver operating characteristic area under the curves for the different scoring systems identified a difference in performance at early, but not late, follow‐up. For all systems, the area under the curve was lower at early compared with late follow‐up and compared to performance in the cohorts in which they were originally defined. Conclusions: No single scoring system performed significantly better than all others when applied in our cohort, although two did display particular advantages. It remains to be determined how best such scoring systems can be incorporated into the routine clinical care of patients with PA. [ABSTRACT FROM AUTHOR]

Context: Besides medullary thyroid carcinoma and other endocrinopathies, people with Multiple Endocrine Neoplasia Type 2 (MEN2) are at risk of gastrointestinal (GI) symptoms. Objective: To investigate the impact of GI symptoms on the daily lives of patients with MEN2. Design: An online survey was conducted among patients with MEN2 via the Association for Multiple Endocrine Neoplasia Disorders (AMEND). Methods: The survey incorporated two validated questionnaires for the assessment of GI symptoms (SAGIS, PAC‐QoL). Participants: There were 91 respondents, MEN2A (n = 57), MEN2B (n = 34). Results: People in the MEN2A group reported a high level of GI symptoms, the most prevalent being abdominal pain 85% (n = 49), diarrhoea 85% (n = 49) and constipation 75% (n = 43) with one patient having a SAGIS score > 10/12 in the constipation domain. People in the MEN2B group reported constipation in 79% (n = 27) with one quarter of these scoring > 10/12 in the constipation domain. Other GI symptoms included diarrhoea 62% (n = 21), excessive gas and flatulence (79%), epigastric pain (59%) abdominal cramps (76%) and dysphagia (41%). The effect of constipation on quality of life was severe in all MEN2 patients as measured by PAC‐QOL and all patients reported dissatisfaction of with their current treatment for constipation. There was a trend towards higher severity of GI symptoms in MEN2B. Conclusions: We report unmet needs of patients with MEN2 syndromes. The GI symptoms, especially constipation, had a severe impact on quality of life in people with MEN2. This suggests that there is room for improvement in the quality of care offered for these patients. [ABSTRACT FROM AUTHOR]

Background: The optimal management of craniopharyngiomas remains controversial. Objectives: To examine temporal trends in the management of craniopharyngioma with a focus on endocrine outcomes. Methods: This was a cross‐sectional, multicentre study. Patients treated between 1951 and 2015 were identified and divided into four quartiles. Demographics, presentation, treatment and outcomes were collected. Results: In total, 142 patients with childhood‐onset craniopharyngioma (48/142; 34%) and adult‐onset disease (94/142; 66%) were included. The median follow‐up was 15 years (IQR 5‐23 years). Across quartiles, there was a significant trend towards using transsphenoidal surgery (P <.0001). The overall use of radiotherapy was not different among the four quartiles (P =.33). At the latest clinical review, the incidence of GH, ACTH, gonadotrophin deficiencies and anterior panhypopituitarism fell significantly across the duration of the study. Anterior panhypopituitarism was not affected by treatment modality (surgery vs surgery and radiotherapy) (P =.23). There was no difference in the incidence of high BMI (≥25 kg/m2) among the four quartiles (P =.14). BMI was higher in patients who treated with surgery and radiotherapy than those treated with surgery only (P =.006). Tumour regrowth occurred in 51 patients (51/142; 36%) with no difference in regrowth among quartiles over the time course of the study (P =.15). Conclusion: We demonstrate a significant reduction in panhypopituitarism in craniopharyngioma patients over time, most likely because of a trend towards more transsphenoidal surgery. However, long‐term endocrine sequelae remain common and lifelong follow‐up is required. [ABSTRACT FROM AUTHOR]