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Current management of pheochromocytoma/paraganglioma: A guide for the practicing clinician in the era of precision medicine
Nölting, S.; Ullrich, M.ORC; Pietzsch, J.ORC; Ziegler, C. G.; Eisenhofer, G.; Grossman, A.; Pacak, K.
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. Besides tumor size, location (adrenal vs. extra-adrenal), 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 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.
Keywords: pheochromocytoma, paraganglioma, guideline, genetics, diagnosis, imaging, follow-up, therapy, precision medicine


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Publ.-Id: 29705