Endocrine Abstracts

Context: Germline mutations of the gene encoding succinate dehydrogenase subunit B (SDHB) predispose to head and neck paraganglioma (HNPGL), sympathetic PGL, phaeochromocytoma and renal cell carcinoma for which regular surveillance is required. SDHB-associated tumors harbor germline and somatic mutations, consistent with Knudson’s two-hit hypothesis stating that the combination of an inactivating germline mutation as a first hit and somatic loss of funct...

Background: Von Hippel Lindau (VHL) disease is a rare tumour syndrome with a high penetrance. VHL mutation carriers develop numerous disease related manifestations in multiple organs during life, but precise difference in growth velocity and incidence of lesions in different organs is still unknown. We aimed to gain insight in the incidence of consecutive new disease manifestations in the organs of patients with VHL.Patients and methods: Clinical data in...

Objective: To assess the effect of somatostatin analogs (SSTA) on mortality in relation to metabolic control of acromegaly after pituitary surgery.Design: A retrospective study in two large tertiary referral centers in The Netherlands.Patients and methods: Three hundred and nineteen patients with acromegaly in whom pituitary surgery was performed as primary therapy between January 1980 and July 2017 were included (total follow-up: ...