TY - JOUR
T1 - Multiple Endocrine Neoplasia Syndromes
AU - Callender, Glenda G.
AU - Rich, Thereasa A.
AU - Perrier, Nancy D.
N1 - Copyright:
Copyright 2008 Elsevier B.V., All rights reserved.
PY - 2008/8
Y1 - 2008/8
N2 - The multiple endocrine neoplasia (MEN) syndromes are rare autosomal-dominant conditions that predispose affected individuals to benign and malignant tumors of the pituitary, thyroid, parathyroids, adrenals, endocrine pancreas, paraganglia, or nonendocrine organs. The classic MEN syndromes include MEN type 1 and MEN type 2. However, several other hereditary conditions should also be considered in the category of MEN: von Hippel-Lindau syndrome, the familial paraganglioma syndromes, Cowden syndrome, Carney complex, and hyperparathyroidism jaw-tumor syndrome. In addition, researchers are becoming aware of other familial endocrine neoplasia syndromes with an unknown genetic basis that might also fall into the category of MEN. This article reviews the clinical features, diagnosis, and surgical management of the various MEN syndromes and genetic risk assessment for patients presenting with one or more endocrine neoplasms.
AB - The multiple endocrine neoplasia (MEN) syndromes are rare autosomal-dominant conditions that predispose affected individuals to benign and malignant tumors of the pituitary, thyroid, parathyroids, adrenals, endocrine pancreas, paraganglia, or nonendocrine organs. The classic MEN syndromes include MEN type 1 and MEN type 2. However, several other hereditary conditions should also be considered in the category of MEN: von Hippel-Lindau syndrome, the familial paraganglioma syndromes, Cowden syndrome, Carney complex, and hyperparathyroidism jaw-tumor syndrome. In addition, researchers are becoming aware of other familial endocrine neoplasia syndromes with an unknown genetic basis that might also fall into the category of MEN. This article reviews the clinical features, diagnosis, and surgical management of the various MEN syndromes and genetic risk assessment for patients presenting with one or more endocrine neoplasms.
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U2 - 10.1016/j.suc.2008.05.001
DO - 10.1016/j.suc.2008.05.001
M3 - Review article
C2 - 18672144
AN - SCOPUS:47849089705
SN - 0039-6109
VL - 88
SP - 863
EP - 895
JO - Surgical Clinics of North America
JF - Surgical Clinics of North America
IS - 4
ER -