Pheochromocytomas and Paragangliomas: From Genetic Diversity to Targeted Therapies

Pang, Ying; Liu, Yang; Pacak, Karel; Yang, Chunzhang

doi:10.3390/cancers11040436

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Pheochromocytomas and Paragangliomas: From Genetic Diversity to Targeted Therapies

by

Ying Pang

^1,†,

Yang Liu

^2,†,

Karel Pacak

¹ and

Chunzhang Yang

^2,*

¹

Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA

²

Neuro-Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD 20892, USA

^*

Author to whom correspondence should be addressed.

^†

These authors contributed equally to the work.

Cancers 2019, 11(4), 436; https://doi.org/10.3390/cancers11040436

Submission received: 14 March 2019 / Revised: 25 March 2019 / Accepted: 26 March 2019 / Published: 28 March 2019

(This article belongs to the Special Issue Pheochromocytoma (PHEO) and Paraganglioma (PGL))

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Abstract

Pheochromocytoma and paraganglioma (PCPGs) are rare neuroendocrine tumors that arise from the chromaffin tissue of adrenal medulla and sympathetic ganglia. Although metastatic PCPGs account for only 10% of clinical cases, morbidity and mortality are high because of the uncontrollable mass effect and catecholamine level generated by these tumors. Despite our expanding knowledge of PCPG genetics, the clinical options to effectively suppress PCPG progression remain limited. Several recent translational studies revealed that PCPGs with different molecular subtypes exhibit distinctive oncogenic pathways and spectrum of therapy resistance. This suggests that therapeutics can be adjusted based on the signature molecular and metabolic pathways of PCPGs. In this review, we summarized the latest findings on PCPG genetics, novel therapeutic targets, and perspectives for future personalized medicine.

Keywords: pheochromocytoma; paraganglioma; neuroendocrine tumor; targeted therapy; therapy resistance

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MDPI and ACS Style

Pang, Y.; Liu, Y.; Pacak, K.; Yang, C. Pheochromocytomas and Paragangliomas: From Genetic Diversity to Targeted Therapies. Cancers 2019, 11, 436. https://doi.org/10.3390/cancers11040436

AMA Style

Pang Y, Liu Y, Pacak K, Yang C. Pheochromocytomas and Paragangliomas: From Genetic Diversity to Targeted Therapies. Cancers. 2019; 11(4):436. https://doi.org/10.3390/cancers11040436

Chicago/Turabian Style

Pang, Ying, Yang Liu, Karel Pacak, and Chunzhang Yang. 2019. "Pheochromocytomas and Paragangliomas: From Genetic Diversity to Targeted Therapies" Cancers 11, no. 4: 436. https://doi.org/10.3390/cancers11040436

APA Style

Pang, Y., Liu, Y., Pacak, K., & Yang, C. (2019). Pheochromocytomas and Paragangliomas: From Genetic Diversity to Targeted Therapies. Cancers, 11(4), 436. https://doi.org/10.3390/cancers11040436

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Pheochromocytomas and Paragangliomas: From Genetic Diversity to Targeted Therapies

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