☆ 4.2 Article

Pituitary adenomas in patients with multiple endocrine neoplasia type 1: a single-center experience in China

PITUITARY (2019)

Journal

PITUITARY

Volume 22, Issue 2, Pages 113-123

Publisher

SPRINGER

DOI: 10.1007/s11102-019-00939-x

Keywords

Multiple endocrine neoplasia type 1; Pituitary adenoma; Treatment; Chinese population

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Abstract

PurposeTo explore the clinical characteristics of pituitary adenomas in patients with MEN1 and to summarize treatment strategies for MEN1 in a Chinese population.MethodsWe retrospectively analyzed 54 MEN1 patients with pituitary adenomas diagnosed at Peking Union Medical College Hospital from March 2003 to January 2017. Clinical data, laboratory testing results, treatments of involved glands and treatment responses were collected and analyzed.ResultsThe mean age at pituitary adenoma diagnosis was 53.917.8. The patients initially consulted the Endocrinology, General Surgery and Neurosurgery departments, in descending frequency. The nonfunctioning adenoma, prolactinoma, GH-secreting adenoma, cosecreting adenoma, and ACTH-secreting adenoma subtypes accounted for 48.1%, 27.8%, 9.3%, 9.3% and 5.6% of the cases, respectively. The remission rate for prolactinomas was 46.2% (6/13) treated with bromocriptine. And the remission rates were 87.5% (7/8) and 100% (3/3) for GH-secreting adenomas and ACTH-secreting adenomas respectively achieved by transsphenoidal surgery. Nineteen (35.2%) patients with asymptomatic nonfunctioning pituitary adenomas showed no progression after a 35-month follow-up with close observation. Regarding treatment priority, patients with thymic carcinoid tumors received first-line surgery, 54% of the patients with enteropancreatic tumors had these tumors treated first, and 26% of all patients had their pituitary adenomas treated first. In acromegalic patients, pituitary lesions tended to be treated first (75%, p=0.002). PHPT and adrenocortical adenomas can be managed with elective surgery.Conclusions p id=Par4 The treatment of MEN1 requires cooperation between multidisciplinary teams. Individualized treatment according to the severity of glandular involvement is needed. GH-secreting and ACTH-secreting pituitary adenomas require active treatment, while nonfunctioning pituitary adenomas can be observed closely.

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Expressions of Cushing's syndrome in multiple endocrine neoplasia type 1

William F. Simonds

Summary: Cushing's syndrome can occur spontaneously or in the context of familial disease. Multiple endocrine neoplasia type 1 (MEN1) is unique among familial endocrine tumor syndromes as it can result in hypercortisolism due to pituitary, adrenal, or thymic neuroendocrine tumors. The clinical manifestations of MEN1 include primary hyperparathyroidism, pituitary tumors, gastroenteropancreatic neuroendocrine tumors, and bronchial carcinoid tumors. CS in MEN1 can be caused by ACTH-secreting pituitary tumors, adrenocortical neoplasms, or ectopic tumoral ACTH secretion from thymic neuroendocrine tumors.

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