A narrative review of multiple endocrine neoplasia syndromes: genetics, clinical features, imaging findings, and diagnosis
نویسندگان
چکیده
منابع مشابه
Epidemiology, clinical features, and genetics of multiple endocrine neoplasia type 2B in a complete population.
Introduction Multiple endocrine neoplasia type 2 (MEN 2) is a hereditary autosomal dominant endocrine syndrome comprising MEN 2A,MEN2B,and familialmedullary thyroid cancer (MTC) [1, 2]. MEN 2B is characterized by early development of aggressive MTC, and prophylactic total thyroidectomy is advocated by 6 months of age [3–5]. Multiple and bilateral pheochromocytoma occur in 50% of patients, and s...
متن کاملDiagnosis and surgical treatment of multiple endocrine neoplasia type 2A
BACKGROUND This study aims to introduce the diagnosis and surgical treatment of the rare disease multiple endocrine neoplasia type 2A (MEN 2A). METHODS Thirteen cases of MEN 2A were diagnosed as medullary thyroid carcinoma (MTC) and pheochromocytoma by biochemical tests and imaging examination. They were treated by bilateral adrenal tumor excision or laparoscopic surgery. RESULTS Nine patie...
متن کاملDiagnosis and Management of Multiple Endocrine Neoplasia Type 1 (MEN1)
Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominantly inherited disorder, characterised by the occurrence of tumours of the parathyroid glands, the pancreatic islets, the pituitary gland, the adrenal glands and neuroendocrine carcinoid tumours. Carcinoid tumours of the thymus and pancreatic-duodenal gastrinomas are the most harmful tumour types, since these tumours have malignan...
متن کاملGenetic and Clinical Features of Multiple Endocrine Neoplasia Types 1 and 2
Multiple endocrine neoplasia (MEN) are clinical inherited syndromes affecting different endocrine glands. Three different patterns of MEN syndromes can occur (MEN 1, MEN 2A, and MEN 2B). MEN syndromes are very rare, affect all ages and both sexes are equally affected. MEN 1 is characterized by the neoplastic transformation of the parathyroid glands, pancreatic islets, anterior pituitary, and ga...
متن کاملHyperparathyroidism in multiple endocrine neoplasia type II A Mini - review
Primary hyperparathyroidism (PHPT) in multiple endocrine neoplasia (MEN) 2A occurs in only 15-30% of patients. It is rarely the first feature recognized in the syndrome, is generally mild and is sometimes expressed only as parathyroid tumors discovered during surgery for medullary thyroid carcinoma. A predisposition to MEN 2 is caused by germline mutations of the RET proto-oncogene on chromosom...
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ژورنال
عنوان ژورنال: Annals of Translational Medicine
سال: 2021
ISSN: 2305-5839,2305-5847
DOI: 10.21037/atm-21-1165