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Review
. 2019 Oct 8;11(10):1505.
doi: 10.3390/cancers11101505.

Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

Svenja Nölting  1 , Martin Ullrich  2 , Jens Pietzsch  3   4 , Christian G Ziegler  5 , Graeme Eisenhofer  6   7 , Ashley Grossman  8   9 , Karel Pacak  10
Affiliations
Review

Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

Svenja Nölting et al. Cancers (Basel). .

Abstract

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.

Keywords: biomarkers; diagnosis; follow-up; genetics; guideline; imaging; paraganglioma; pheochromocytoma; precision medicine; therapy.

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Conflict of interest statement

The authors declare no conflict of interest. The funders had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript, or in the decision to publish the results.

Figures

Figure 1
Figure 1
(Modified from [34]): Therapy options in metastatic PCCs/PGLs.
See this image and copyright information in PMC

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