Molecular and therapeutic advances in the diagnosis and management of malignant pheochromocytomas and paragangliomas.

ABSTRACT: Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare catecholamine-secreting tumors derived from chromaffin cells originating in the neural crest. These tumors represent a significant diagnostic and therapeutic challenge because the diagnosis of malignancy is frequently made in retrospect by the development of metastatic or recurrent disease. Complete surgical resection offers the only potential for cure; however, recurrence can occur even after apparently successful resection of the primary tumor. The prognosis for malignant disease is poor because traditional treatment modalities have been limited. The last decade has witnessed exciting discoveries in the study of PCCs and PGLs; advances in molecular genetics have uncovered hereditary and germline mutations of at least 10 genes th

SUBMITTER: Lowery AJ 

PROVIDER: S-EPMC3639526 | biostudies-literature | 2013

REPOSITORIES: biostudies-literature