Horm Metab Res 2012; 44(05): 390-399
DOI: 10.1055/s-0031-1299707
© Georg Thieme Verlag KG Stuttgart · New York

Metastatic Pheochromocytoma and Paraganglioma: Focus on Therapeutics

P.-F. Plouin
1   Paris-Descartes University, INSERM U-970, Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Hypertension Unit, Paris cedex 15, France
P. Fitzgerald
2   Department of Endocrinology, University of California-San Francisco, San Francisco, CA, USA
T. Rich
3   Department of Endocrine Surgery, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA
M. Ayala-Ramirez
4   Endocrine Neoplasia and Hormone Disorders, Unit 1461, The University of Texas MD Houston, Texas, USA
N. D. Perrier
3   Department of Endocrine Surgery, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA
E. Baudin
5   Département de Médecine Nucléaire et de Cancérologie Endocrinienne,Institut Gustave Roussy 39 Bis, Villejuif, France
C. Jimenez
4   Endocrine Neoplasia and Hormone Disorders, Unit 1461, The University of Texas MD Houston, Texas, USA
› Author Affiliations
Further Information

Publication History

received 01 November 2011

accepted 05 December 2011

Publication Date:
07 February 2012 (online)


Metastatic pheochromocytomas and paragangliomas are rare and challenging tumors. The tumor burden, combined with excessive catecholamine production, predispose to a broad spectrum of complications that range from spinal cord compression to any organ damage, all of which may lead to decreased quality of life and overall survival. Current therapies include surgery, systemic chemotherapy and radiopharmaceutical agents. Surgery is often a preferred therapy because it may cure or allow a long-term remission in patients with locoregional or isolated resectable distant metastases. Additionally, surgery can palliate symptoms related to tumor burden or catecholamine excess. However, in patients for whom surgery is not an option, systemic chemotherapy and radiopharmaceutical agents are preferred options. Systemic chemotherapy and radiopharmaceutical agents such as 131I-Metaiodobenzylguanidine (131I-MIBG) may cause partial responses or stabilization of disease with better blood pressure control and symptomatic and performance status improvement. However, as these therapies are only palliative, patients’ quality of life and personal preferences should always be considered. The recognition of molecular pathways involved in the pheochromocytoma and paraganglioma tumorigenesis has driven the development of new therapeutic options. Agents such as tyrosine kinase, MAPK, PI3K, or hypoxia inducible factor inhibitors, alone or in combination, may represent novel therapeutic strategies that could be evaluated in prospective clinical trials. Transcriptional profiling and the development of personalized cancer medicine will help to pave the way for more specific therapeutic approaches and combinations.

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