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Review
. 2024 Aug:51:101116.
doi: 10.1016/j.currproblcancer.2024.101116. Epub 2024 Jul 17.

Management of metastatic pheochromocytomas and paragangliomas: when and what

Affiliations
Free article
Review

Management of metastatic pheochromocytomas and paragangliomas: when and what

Vineeth Sukrithan et al. Curr Probl Cancer. 2024 Aug.
Free article

Abstract

Recently, the treatment landscape for metastatic pheochromocytomas and paragangliomas (MPPGL) has seen both progress and setbacks. We provide an up-to-date review of the multimodality management of MPPGL and discuss novel opportunities and current challenges in the treatment landscape. Given the unique clinical presentation of MPPGL, we discuss the management of hormone-related clinical sequelae and traditional modalities of therapy. Advances in the understanding of the molecular biology of these diverse tumors have enabled novel strategies such as augmenting DNA damage by targeted delivery of radionuclides such as 131I and 177Lu, abrogating tumor angiogenesis, hypoxia resistance, and DNA damage repair. Despite progress, we address the significant challenges still faced by patients and researchers engaged in efforts to improve outcomes in these rare cancers.

Keywords: Azedra; Belzutifan; CVD; Cabozantinib; Capecitabine/Temozolomide; I-131 Iobenguane; Lutathera; Olaparib; PRRT; Paraganglioma; Pheochromocytoma.

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Conflict of interest statement

Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: V.S. reports research funding to the institution from Eli Lilly and Company and Crinetics Pharmaceuticals. He has served as a consultant to GE Oncology and has received speaking fees from Lantheus. He serves on the advisory board for Exelixis. N.P-T. has served as a consultant or advisory board member for and/or received honoraria from Actinium Pharma, Progenics, Medimmune/Astrazeneca, Illumina, and ImaginAb and conducts research institutionally supported by Y-mAbs, ImaginAb, BMS, Bayer, Clarity Pharma, Janssen, and Regeneron. C.J. reports receiving research funding to the institution from Exelixis, Lantheus Pharmaceuticals, Progenics Pharmaceuticals, and Merck Sharp and Dohme and is a scientific advisory board member for Lantheus Pharmaceuticals, Progenics Pharmaceuticals, and Merck Sharp and Dohme. K.P. was a one-time advisory board member of Exelixis, Lantheus, Ipsen, and Novartis.

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