Emerging Treatments for Advanced/Metastatic Pheochromocytoma and Paraganglioma
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Neuroendocrine Cancers (JR Strosberg, Section Editor)
Emerging Treatments for Advanced/Metastatic Pheochromocytoma and Paraganglioma Maran Ilanchezhian, BS1 Abhishek Jha, MD2 Karel Pacak, MD, PhD, DSc2 Jaydira Del Rivero, MD3,4,* Address 1 Pediatric Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD, USA 2 Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA *,3 Developmental Therapeutics Branch, Rare Tumor Initiative, Center for Cancer Research, National Cancer Institute, Bethesda, MD, USA Email: [email protected] 4 Medical Oncology & Clinical Endocrinology, Center for Cancer Research, National Cancer Institute/National Institutes of Health, 10 Center Drive, MSC 1906, Building 10, CRC 13C-434, Bethesda, MD, 20892, USA
* The Author(s) 2020
This article is part of the Topical Collection on Neuroendocrine Cancers Keywords Pheochromocytoma I Paraganglioma I Genetics I Imaging I Oncogenic signaling I Therapy
Opinion statement The incidence of metastatic pheochromocytoma (PHEO) and paraganglioma (PGL) may occur in as many as 35% of patients particularly with PGL and even more frequently in those with specific mutations. Biochemical, morphological, and molecular markers have been investigated for use in the distinction of benign from malignant PHEO/PGL. PHEO/PGL metastasizes via hematogenous or lymphatic routes and shows differences based on mutational status. The most common sites of involvement in patients that have an SDHB mutation are the bone (78%), lungs (45%), lymph nodes (36%), and liver (35%). In patients with sporadic PHEO/PGL, the most common sites of metastasis are the bones (64%), lungs (47%), lymph nodes (36%), and liver (32%). Metastases may be present at presentation or may occur later. Metastases to the liver and lungs are associated with a shorter survival. Overall, the estimated 5-year survival rates are between 34 and 74%. Currently, treatments for metastatic PHEO/PGL are essentially palliative. Surgery is potentially curative; however, tumor dissemination limits the chance for a curative resection.
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Curr. Treat. Options in Oncol.
(2020) 21:85
When surgical intervention is not amenable, the therapeutic options include radiolabeled MIBG (Azedra®—iobenguane 131 was recently FDA-approved for patients 9 12 years and older with iobenguane scan positive) or systemic chemotherapy with cyclophosphamide, vincristine, and dacarbazine (CVD) with an overall objective response rate (ORR) of less than 40%; however, it is not clear if the administration of CVD impacts overall survival, as nearly all patients develop progressive and ultimately fatal disease. Other treatment modalities under investigation include cytoreductive techniques, novel radiopharmaceuticals, chemotherapy, radiotherapy, immunotherapy, and experimental therapies. Here we are discussing emerging treatment for advanced/metastatic PHEO/PGL.
Introduction Pheochromocytomas (PHEOs) and paragangliomas (PGLs) a
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