Scholz Tim, Eisenhofer Graeme, Pacak Karel, Dralle Henning, Lehnert Hendrik
Department of Endocrinology and Metabolism, Otto von Guericke University Medical School, Magdeburg, Germany, and University Hospital of Coventry, UK.
J Clin Endocrinol Metab. 2007 Apr;92(4):1217-25. doi: 10.1210/jc.2006-1544. Epub 2007 Feb 6.
Pheochromocytomas are rare tumors of predominantly adrenal origin that often produce and secrete catecholamines. Malignancy occurs in a variable percentage of cases depending on genetic background and tumor location. Definitive diagnosis relies on the detection of distant metastases. Treatments for malignant pheochromocytoma include surgical debulking, pharmacological control of hormone-mediated symptoms, targeted methods such as external irradiation, and systemic antineoplastic therapy. Different agents and protocols for this purpose are reviewed, and their therapeutic potential is discussed.
Literature on antineoplastic therapies for malignant pheochromocytoma was identified by searching the PubMed database with restriction to articles published in English during the past 30 yr.
Because of the rarity of the condition, no randomized clinical trials concerning the treatment of malignant pheochromocytoma have been performed. The strategy established best is [131I]meta-iodobenzylguanidine (MIBG) therapy, which is well tolerated. Similar to cytotoxic chemotherapy with cyclophosphamide, vincristine, and dacarbazine, MIBG can induce remission for a limited period in a significant proportion of patients. Octreotide as a single agent seems to be largely ineffective.
MIBG radiotherapy and cyclophosphamide, vincristine, and dacarbazine chemotherapy are comparable with respect to response rate and toxicity. It is unclear whether combining both can improve the outcome. Future developments may include new multimodal concepts with focus on inhibition of angiogenetic factors and heat shock protein 90. Any present or new therapeutic approach must take into account the highly variable natural course of the disease.
嗜铬细胞瘤是一种罕见肿瘤，主要起源于肾上腺，常产生和分泌儿茶酚胺。恶性肿瘤的发生率因遗传背景和肿瘤位置而异。明确诊断依赖于远处转移的检测。恶性嗜铬细胞瘤的治疗方法包括手术减瘤、激素介导症状的药物控制、外照射等靶向方法以及全身抗肿瘤治疗。本文综述了为此目的使用的不同药物和方案，并讨论了它们的治疗潜力。
通过检索PubMed数据库，筛选过去30年发表的英文文章，确定了有关恶性嗜铬细胞瘤抗肿瘤治疗的文献。
由于该疾病罕见，尚未进行关于恶性嗜铬细胞瘤治疗的随机临床试验。已确立的最佳策略是[131I]间碘苄胍（MIBG）治疗，其耐受性良好。与环磷酰胺、长春新碱和达卡巴嗪的细胞毒性化疗类似，MIBG可使相当一部分患者在有限时间内获得缓解。单独使用奥曲肽似乎大多无效。
MIBG放疗与环磷酰胺、长春新碱和达卡巴嗪化疗在缓解率和毒性方面相当。尚不清楚两者联合是否能改善治疗结果。未来的发展可能包括以抑制血管生成因子和热休克蛋白90为重点的新的多模式概念。任何当前或新的治疗方法都必须考虑到该疾病高度可变的自然病程。
