Open AccessUpdate on Pheochromocytoma and Paraganglioma from the SSO Endocrine/Head and Neck Disease-Site Work Group. Part 1 of 2: Advances in Pathogenesis and Diagnosis of Pheochromocytoma and Paraganglioma
Author(s) -
Dhaval Patel,
John E. Phay,
Tina W.F. Yen,
Paxton V. Dickson,
Tracy Wang,
Roberto Garcia,
Anthony D. Yang,
Carmen C. Solórzano,
Lawrence T. Kim
Publication year - 2020
Publication title -
annals of surgical oncology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.764
H-Index - 173
eISSN - 1534-4681
pISSN - 1068-9265
DOI - 10.1245/s10434-020-08220-3
Subject(s) - paraganglioma , pheochromocytoma , medicine , presentation (obstetrics) , endocrine disease , surgical oncology , neuroendocrine tumors , etiology , disease , multiple endocrine neoplasia type 2 , work up , endocrine system , pathology , radiology , germline mutation , hormone , gene , mutation , biochemistry , chemistry
This first part of a two-part review of pheochromocytoma and paragangliomas (PPGLs) addresses clinical presentation, diagnosis, management, treatment, and outcomes. In this first part, the epidemiology, prevalence, genetic etiology, clinical presentation, and biochemical and radiologic workup are discussed. In particular, recent advances in the genetics underlying PPGLs and the recommendation for genetic testing of all patients with PPGL are emphasized. Finally, the newer imaging methods for evaluating of PPGLs are discussed and highlighted.