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Endocrine Tumors as Part of Inherited Tumor Syndromes

Zhang, Yaxia MD, PhD; Nosé, Vânia MD, PhD

Advances in Anatomic Pathology: May 2011 - Volume 18 - Issue 3 - p 206–218
doi: 10.1097/PAP.0b013e3182169916
Review Articles

The endocrine system is an embryological diverse system consisted of a variety of glands, such as pituitary, parathyroid, thyroid, adrenal, endocrine pancreas, and the diffuse neuroendocrine system. Endocrine tumor can be presented as a sporadic event, or as part of an inherited tumor syndrome. During the past century, inherited tumor syndromes have emerged as an important group of diseases. With the growing knowledge of each inherited tumor syndrome in the molecular genetic level, many changes in clinical management have occurred. This review focuses on the endocrine tumors involving the pituitary, parathyroid, thyroid, adrenal, and endocrine pancreas, in the setting of inherited tumor syndromes. We discuss the specific clinical, molecular genetic, and pathologic features of endocrine tumor in each gland and their associated inherited tumor syndromes. By understanding the pathogenesis of inherited endocrine tumor syndromes and recognizing the unique features of pathologic findings, pathologists bear the responsibility to provide the clinicians the guidelines in terms of screening and treatment of inherited tumor syndromes.

Department of Pathology, University of Miami Miller School of Medicine, Miami, FL

Reprints: Vânia Nosé, MD, PhD, Anatomic Pathology, University of Miami Miller School of Medicine, 1200 NW 14th Ave, Room 1411, Miami, FL 33136 (e-mail:

All figures can be viewed online in color at

© 2011 Lippincott Williams & Wilkins, Inc.