RT Journal Article SR Electronic T1 Systemic Radiopharmaceutical Therapy of Pheochromocytoma and Paraganglioma JF Journal of Nuclear Medicine JO J Nucl Med FD Society of Nuclear Medicine SP 1192 OP 1199 DO 10.2967/jnumed.120.259697 VO 62 IS 9 A1 Jorge A. Carrasquillo A1 Clara C. Chen A1 Abhishek Jha A1 Karel Pacak A1 Daniel A. Pryma A1 Frank I. Lin YR 2021 UL http://jnm.snmjournals.org/content/62/9/1192.abstract AB Whereas benign pheochromocytomas and paragangliomas are often successfully cured by surgical resection, treatment of metastatic disease can be challenging in terms of both disease control and symptom control. Fortunately, several options are available, including chemotherapy, radiation therapy, and surgical debulking. Radiolabeled metaiodobenzylguanidine (MIBG) and somatostatin receptor imaging have laid the groundwork for use of these radiopharmaceuticals as theranostic agents. 131I-MIBG therapy of neuroendocrine tumors has a long history, and the recent approval of high-specific-activity 131I-MIBG for metastatic or inoperable pheochromocytoma or paraganglioma by the U.S. Food and Drug Administration has resulted in general availability of, and renewed interest in, this treatment. Although reports of peptide receptor radionuclide therapy of pheochromocytoma and paraganglioma with 90Y- or 177Lu-DOTA conjugated somatostatin analogs have appeared in the literature, the approval of 177Lu-DOTATATE in the United States and Europe, together with National Comprehensive Cancer Network guidelines suggesting its use in patients with metastatic or inoperable pheochromocytoma and paraganglioma, has resulted in renewed interest. These agents have shown evidence of efficacy as palliative treatments in patients with metastatic or inoperable pheochromocytoma or paraganglioma. In this continuing medical education article, we discuss the therapy of pheochromocytoma and paraganglioma with 131I-MIBG and 90Y- or 177Lu-DOTA-somatostatin analogs.