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Therapies targeting the signal pathways of pheochromocytoma and paraganglioma

Authors Liu Y, Liu L, Zhu F

Received 11 June 2019

Accepted for publication 14 August 2019

Published 4 September 2019 Volume 2019:12 Pages 7227—7241


Checked for plagiarism Yes

Review by Single-blind

Peer reviewer comments 3

Editor who approved publication: Dr XuYu Yang

Yalin Liu1, Longfei Liu2, Feizhou Zhu1

1Department of Biochemistry and Molecular Biology, Xiangya School of Medicine, Central South University, Changsha, People’s Republic of China; 2Department of Urology, Xiangya Hospital, Central South University, Changsha, People’s Republic of China

Correspondence: Feizhou Zhu
Department of Biochemistry and Molecular Biology, Xiangya School of Medicine, Central South University (CSU), Tongzipo Road 172, Changsha, Hunan 410013, People’s Republic of China
Tel +86 01 370 749 6211
Fax +86 8 265 0230

Abstract: Pheochromocytoma and paraganglioma (PCC/PGL) are rare tumors that originate from adrenal or extra-adrenal chromaffin cells. A significant clinical manifestation of PCC/PGL is that the tumors release a large number of catecholamines continuously or intermittently, causing persistent or paroxysmal hypertension and multiple organ functions and metabolic disorders. Though majority of the tumors are non-metastatic, about 10% are metastatic tumors. Others even have estimated that the rate of metastasis may be as high as 26%. The disease is most common in individuals ranging from 20 to 50 years old and the age of onset strongly depends on the genetic background: patients with germline mutations in susceptible genes have an earlier presentation. Besides, there are no significant differences in the incidence between men and women. At present, traditional treatments, such as surgical treatment, radionuclide therapy, and chemotherapy are still prior choices. However, they all have several deficiencies so that the effects are not extremely significant. Contemporary studies have shown that hypoxia-associated signal pathway, associated with the cluster 1 genes of PCC/PGL, and increased kinase signal pathways, associated with the cluster 2 genes of PCC/PGL, are the two major pathways involving the molecular pathogenesis of PCC/PGL, indicating that PCC/PGL can be treated with targeted therapies in emerging trends. This article reviews the progress of molecular-targeted therapies for PCC/PGL.

Keywords: pheochromocytoma, paraganglioma, targeted therapies, signal pathways

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