Ein S H, Weitzman S, Thorner P, Seagram C G, Filler R M
Division of General Surgery, Hospital for Sick Children, Toronto, Ontario, Canada.
J Pediatr Surg. 1994 Sep;29(9):1197-201. doi: 10.1016/0022-3468(94)90799-4.
Pediatric malignant pheochromocytomas are very rare tumors, and no institution has more than one or two of these problem cases. The authors report on two children with such tumors, over a 9-year period, from two hospitals. In 1984, D.B. (14 years of age) presented with symptoms and signs of extradural metastasis from a right adrenal primary; he also had lung and bone metastases. After spinal decompression almost 4 years. He has remained well and is in remission 6 years later. In 1987, G.R. (13 years of age) presented with a larger right adrenal malignant pheochromocytoma invading surrounding structures; he also had liver metastasis. Preoperative chemotherapy did not shrink the tumor much; it was grossly resected, and there were many postoperative problems. In 1990, bone metastases developed, for which radiotherapy and chemotherapy were used. Three years later, the metastases have not disappeared; he remains on chemotherapy, and his liver function is borderline. From our small experience as well as a literature review, it appears that surgical excision remains the treatment of choice for the pediatric malignant phoechromocytoma. Unresectable tumors may be rendered resectable by intensive chemotherapy (similar to that used for neuroblastoma); adjuvant chemotherapy should be used for residual disease after surgery and for metastatic disease.
小儿恶性嗜铬细胞瘤是非常罕见的肿瘤，没有哪家机构遇到过超过一两个这样的疑难病例。作者报告了来自两家医院的两名患有此类肿瘤的儿童，时间跨度为9年。1984年，14岁的D.B.出现了右肾上腺原发性肿瘤硬膜外转移的症状和体征；他还伴有肺和骨转移。在进行脊髓减压近4年后，他一直状况良好，6年后病情缓解。1987年，13岁的G.R.出现了一个较大的右肾上腺恶性嗜铬细胞瘤，侵犯周围组织；他也有肝转移。术前化疗并没有使肿瘤明显缩小；肿瘤被大体切除，但术后出现了许多问题。1990年发生了骨转移，为此采用了放疗和化疗。三年后，转移灶并未消失；他仍在接受化疗，肝功能处于临界状态。根据我们的少量经验以及文献回顾，手术切除似乎仍是小儿恶性嗜铬细胞瘤的首选治疗方法。无法切除的肿瘤可通过强化化疗（类似于用于神经母细胞瘤的化疗）变得可切除；辅助化疗应用于术后残留疾病和转移性疾病。
