Update on the management of unusual neuroendocrine tumors: pheochromocytoma and paraganglioma, medullary thyroid cancer and adrenocortical carcinoma.

Abstract

Pheochromocytomas, paragangliomas, and medullary thyroid carcinomas (MTCs) originate in cells that share a common neuroectodermal origin. Like other neuroendocrine neoplasms, they are characterized by a propensity to secrete amines (epinephrine and norepinephrine) and peptide hormones (calcitonin). Improved understanding of underlying molecular pathways, such as mutations of the RET (rearranged during transfection) proto-oncogene, has led to new rational targeted therapies. Adrenocortical carcinomas (ACCs) originate in the steroid hormone-producing adrenal cortex. While tumors of the adrenal cortex are not, strictly speaking, part the "diffuse neuroendocrine system," they are often included in neuroendocrine tumor guidelines due to their orphan status. In this update on management of unusual neuroendocrine tumors, we review the biology and treatment of these rare neoplasms.

• Authors: Jonathan R Strosberg
• Journal: Seminars in oncology (Semin Oncol) Vol. 40 Issue 1 Pg. 120-33 (Feb 2013) ISSN: 1532-8708 [Electronic] United States
• PMID: 23391119 (Publication Type: Journal Article, Review)
• Copyright: Copyright © 2013 Elsevier Inc. All rights reserved.

Topics

• Adrenal Cortex Neoplasms (genetics, pathology, therapy)
• Adrenal Gland Neoplasms (genetics, pathology, therapy)
• Adrenocortical Carcinoma (genetics, pathology, therapy)
• Carcinoma, Neuroendocrine
• Genetic Predisposition to Disease
• Humans
• Paraganglioma (genetics, pathology, therapy)
• Pheochromocytoma (genetics, pathology, therapy)
• Proto-Oncogene Mas
• Thyroid Neoplasms (genetics, pathology, therapy)
• Treatment Outcome