Pheochromocytomas and paragangliomas represent less than 1% of all endocrine tumors. Approximately 15–20% of these tumors are malignant. The definition of malignancy relies on the presence of metastasis. Metastatic pheochromocytomas and paragangliomas are usually advanced, incurable tumors with limited therapeutic options. About 50–60% of these tumors express the noradrenaline transporter in their cell membranes. Recently, the United States Food and Drug Administration approved high-specific-activity iodine 131 metaiodobenzylguanidine (HSA-I-131-MIBG) for the treatment of metastatic pheochromocytomas and paragangliomas that express the noradrenaline transporter. This review reports the benefits and toxicity of HSA-I-131-MIBG, its physical and dosimetric aspects, and radiation safety precautions, as well as its potential therapeutic value for other malignancies (neuroblastoma, gastroenteropancreatic neuroendocrine tumors, and medullary thyroid carcinoma).

中文翻译：

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高特异性活性碘131碘碘苄胍用于治疗转移性嗜铬细胞瘤或副神经节瘤：一种孤儿疾病的新疗法。

嗜铬细胞瘤和副神经节瘤占所有内分泌肿瘤的不到1％。这些肿瘤中约有15–20％是恶性的。恶性肿瘤的定义取决于转移的存在。转移性嗜铬细胞瘤和神经节旁瘤通常是晚期，无法治愈的肿瘤，治疗选择有限。这些肿瘤中约有50-60％在其细胞膜中表达去甲肾上腺素转运蛋白。最近，美国食品药品监督管理局批准了高特异性活性碘131碘碘苄基胍（HSA-I-131-MIBG）用于治疗表达去甲肾上腺素转运蛋白的转移性嗜铬细胞瘤和副神经节瘤。这篇综述报告了HSA-I-131-MIBG的益处和毒性，其物理和剂量方面以及放射安全预防措施，以及其对其他恶性肿瘤（神经母细胞瘤，胃肠胰腺神经内分泌肿瘤和甲状腺髓样癌）的潜在治疗价值。