Current management of pheochromocytoma/paraganglioma: a guide for the practicing clinician in the era of precision medicine

Journal article

Pheochromocytomas and paragangliomas (PCC/PGLs) are rare, mostly catecholamine-producing neuroendocrine tumors of the adrenal gland (PCCs) or the extra-adrenal paraganglia (PGL). They can be separated into three different molecular clusters depending on their underlying gene mutations in any of the at least 20 known susceptibility genes: The pseudohypoxia-associated cluster 1, the kinase signaling-associated cluster 2, and the Wnt signaling-associated cluster 3. In addition to tumor size, location (adrenal vs. extra-adrenal), multiplicity, age of first diagnosis, and presence of metastatic disease (including tumor burden), other decisive factors for best clinical management of PCC/PGL include the underlying germline mutation. The above factors can impact the choice of different biomarkers and imaging modalities for PCC/PGL diagnosis, as well as screening for other neoplasms, staging, follow-up, and therapy options. This review provides a guide for practicing clinicians summarizing current management of PCC/PGL according to tumor size, location, age of first diagnosis, presence of metastases, and especially underlying mutations in the era of precision medicine.

Authors: Nölting, S; Ullrich, M; Pietzsch, J; Ziegler, C; Eisenhofer, G

• Publication status: Published
• Peer review status: Peer reviewed
• Publisher: MDPI
• Journal: Cancers
• Volume: 11
• Issue: 10
• Article number: 1505
• Publication date: 2019-10-08
• Acceptance date: 2019-09-24
• DOI: 10.3390/cancers11101505
• EISSN: 2072-6694
• ISSN: 2072-6694
• Pmid: 31597347
• Language: English
• Keywords: FFR