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01-08-2013 | Sarcomas (SR Patel, Section Editor)

Current and Future Treatments for Malignant Pheochromocytoma and Sympathetic Paraganglioma

Authors: Camilo Jimenez, Eric Rohren, Mouhammed Amir Habra, Thereasa Rich, Paola Jimenez, Montserrat Ayala-Ramirez, Eric Baudin

Published in: Current Oncology Reports | Issue 4/2013

Abstract

Pheochromocytomas (PHs) and sympathetic paragangliomas (SPGs) are rare neuroendocrine tumors. Approximately 17 % of these tumors are malignant, but because no molecular or histologic markers for malignancy exist, patients are often diagnosed with malignant PHs or SPGs after unresectable disease has formed. Patients with progressive metastatic tumors and overwhelming symptoms are currently treated with systemic chemotherapy and radiopharmaceutical agents such as metaiodobenzylguanidine. These therapies lead to partial radiographic response, disease stabilization, and symptomatic improvement in approximately 40 % of patients, and systemic chemotherapy is associated with a modest improvement in overall survival duration. However, over the past decade, substantial progress has been made in clinical, biochemical, and radiographic diagnosis of PHs and SPGs. Approximately 50 % of patients with malignant PHs and SPGs have been found to carry hereditary germline mutations in the succinate dehydrogenase subunit B gene (SDHB), and anti-angiogenic agents such as sunitinib have been found to potentially play a role in the treatment of malignant disease, especially in patients with SDHB mutations. In some patients, treatment with sunitinib has been associated with partial radiographic response, disease stabilization, decreased fluorodeoxyglucose uptake on positron emission tomography, and improved blood pressure control. These findings have led to the development of prospective clinical trials of new targeted therapies for metastatic disease. Here, we provide an updated review of the clinical and genetic predictors of malignant disease, radiographic diagnosis of malignant disease, and information from the most relevant studies of systemic therapies, as well as proposed treatment guidelines for patients with metastatic or potentially malignant PHs and SPGs.

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Title: Current and Future Treatments for Malignant Pheochromocytoma and Sympathetic Paraganglioma
Authors: Camilo Jimenez
Eric Rohren
Mouhammed Amir Habra
Thereasa Rich
Paola Jimenez
Montserrat Ayala-Ramirez
Eric Baudin
Publication date: 01-08-2013
Publisher: Springer US
Published in: Current Oncology Reports / Issue 4/2013
Print ISSN: 1523-3790
Electronic ISSN: 1534-6269
DOI: https://doi.org/10.1007/s11912-013-0320-x

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The Treatment Outcome for Adult Patients with Ewing’s Sarcoma

The Adolescent and Young Adult with Cancer: State of the Art-- Acute Leukemias

Cancer in Adolescents and Young Adults in Countries with Limited Resources

Keynote webinar | Spotlight on antibody–drug conjugates in cancer