Department of Endocrine Neoplasia and Hormonal Disorders, Unit 1461, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX, 77030, USA.
Hemato-Oncologos Asociados, Bogota, Colombia.
Curr Oncol Rep. 2017 Oct 28;19(12):83. doi: 10.1007/s11912-017-0643-0.
The purpose of this manuscript is to review the progress in the field of therapeutics for malignant pheochromocytomas and sympathetic paraganglioma (MPPG) over the past 5 years.
The manuscript will describe the clinical predictors of survivorship and their influence on the first TNM staging classification for pheochromocytomas and sympathetic paragangliomas, the treatment of hormonal complications, and the rationale that supports the resection of the primary tumor and metastases in patients with otherwise incurable disease. Therapeutic options for patients with bone metastasis to the spine will be presented. The manuscript will also review chemotherapy and propose a maintenance regimen with dacarbazine for patients initially treated with cyclophosphamide, vincristine, and dacarbazine. Finally, the manuscript will review preliminary results of several phase 2 clinical trials of novel radiopharmaceutical agents and tyrosine kinase inhibitors. MPPGs are very rare neuroendocrine tumors. MPPGs are usually characterized by a large tumor burden, excessive secretion of catecholamines, and decreased overall survival. Recent discoveries have enhanced our knowledge of the pathogenesis and phenotypes of MPPG. This knowledge is leading to a better understanding of the indications and limitations of the currently available localized and systemic therapies as well as the development of phase 2 clinical trials for novel medications.
目的综述：本文旨在回顾过去 5 年中恶性嗜铬细胞瘤和副神经节瘤（MPPG）治疗领域的进展。
最新发现：本文将描述生存预测因子及其对嗜铬细胞瘤和副神经节瘤的首次 TNM 分期分类的影响、激素并发症的治疗，以及支持切除原发性肿瘤和转移灶的基本原理，这些转移灶存在于其他情况下无法治愈的疾病患者中。还将介绍治疗脊柱骨转移患者的方法。本文还将回顾化疗，并为最初接受环磷酰胺、长春新碱和达卡巴嗪治疗的患者提出用达卡巴嗪维持治疗的方案。最后，本文将回顾几种新型放射性药物和酪氨酸激酶抑制剂的 2 期临床试验的初步结果。MPPG 是非常罕见的神经内分泌肿瘤。MPPG 通常表现为肿瘤负荷大、儿茶酚胺过度分泌和总生存期缩短。最近的发现增强了我们对 MPPG 发病机制和表型的认识。这种认识正在导致更好地理解当前可用的局部和全身治疗的适应证和局限性，以及开发新型药物的 2 期临床试验。
