RT Book, Section A1 Nguyen, Ha A1 Habra, Mouhammed Amir A2 Kantarjian, Hagop M. A2 Wolff, Robert A. A2 Rieber, Alyssa G. SR Print(0) ID 1190838676 T1 Endocrine Malignancies T2 The MD Anderson Manual of Medical Oncology, 4e YR 2022 FD 2022 PB McGraw Hill Education PP New York, NY SN 9781260467642 LK hemonc.mhmedical.com/content.aspx?aid=1190838676 RD 2024/10/08 AB KEY CONCEPTSThyroid carcinoma is the most common endocrine malignancy. Recent advances in molecular profiling identified actionable mutations, leading to the availability of multiple FDA-approved drugs. The current treatments target angiogenesis (sorafenib and lenvatinib) or can be mutation specific (larotrectinib targeting NTRK gene fusion and selpercatinib targeting RET gene fusions).Anaplastic thyroid carcinoma (ATC) has very poor prognosis, but the clinical outcomes appear to be improving with a multidisciplinary approach utilizing targeted therapy, immunotherapy, radiotherapy, and surgery when feasible.In cases with suspected parathyroid carcinoma, comprehensive surgery by an experienced team remains the mainstay of therapy, although the role of adjuvant radiation and targeted therapy remains unclear.Genetic counseling and testing is needed in all cases of pheochromocytoma/paraganglioma, even in the absence of family history of these tumorsThe management of metastatic pheochromocytoma/paraganglioma should be in the context of a multidisciplinary and experienced team. Resection of the primary tumor when feasible is associated with improved outcome. Iobenguane I 131 is FDA approved for patients with metastatic pheochromocytoma/paraganglioma that retain meta-iodobenzylguanidine avidity, whereas traditional cytotoxic chemotherapy can be offered to select patients with rapidly progressing disease, especially in the presence of excess catecholamines. The role of targeted therapy is evolving, and extreme care must be exerted when using these drugs in patients with metastatic pheochromocytoma because they could exacerbate preexisting hypertension.The combined use of mitotane with cytotoxic chemotherapy (etoposide, doxorubicin, cisplatin) is associated with a suboptimal response rate of 23% and represents the first-line systemic therapy in advanced adrenocortical carcinoma. Single-agent immune checkpoint inhibitors are associated with lower response rates based on small clinical trials.