IMED3004 Lecture Notes - Lecture 17: Adrenocortical Adenoma, Pancreatic Islets, Neuroendocrinology
1 Nov 2020
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Document Summary
A group of uncommon inherited diseases characterised by: Proliferative lesions (hyperplasia, adenomas and carcinomas) of multiple endocrine organs: one gland or multiple glands (can be multifocal) Tumours occur at a younger age than sporadic tumours: malignancies are usually more aggressive and recur in a higher proportion of cases than sporadic tumours, autosomal dominant - family follow-up important and early prophylactic treatment. Multiple endocrine neoplasia syndrome 1: men-1 (wermer syndrome) Primary hyperparathyroidism (adenoma or hyperplasia) in most men-1 patients by age 50. Pancreas: neuroendocrine tumours (net) of endocrine pancreas (adenomas or carcinomas, usually functional, often ppp, gastrin, insulin producing) Prolactinomas, less commonly acromegaly: also adrenocortical adenomas, carcinoid tumours of dispersed neuroendocrine system, thyroid adenomas and lipomas. Three types, all have medullary thyroid carcinoma as main manifestation ( two/thyroid : usually due to ret proto-oncogene mutations, men-2a (sipple syndrome): mtc plus adrenal phaechromocytoma and parathyroid hyperplasia, men-2b: mtc plus adrenal phaeochromocytoma.
