儿童内胚窦瘤临床治疗研究

Treatment of endodermal sinus tumor in children

目的探讨儿童内胚窦瘤的治疗与转归。方法回顾性分析2000年4月至2013年7月收治的12例内胚窦瘤患儿的临床资料。其中男7例，女5例；11例年龄在1～3．3岁之间，1例11岁。Ⅰ期2例，Ⅱ期4例，Ⅲ期2例，Ⅳ期4例。1例采用单纯手术，1例采用手术联合VAC（长春新碱＋放线菌素D＋环磷酰胺）方案化疗，10例采用手术联合以PEB（顺铂＋依托？自苷＋博来霉素）方案为主的化疗。结果成功随访11例，目前存活10例，生存期为4．5—66个月。10例接受了PEB方案化疗的患儿，8例达到完全缓解，l例达到部分缓解，1例失访。PEB方案治疗并发症主要包括骨髓抑制及胃肠道反应，未发现远期并发症。结论手术联合PEB化疗方案是有效、安全的治疗儿童内胚窦瘤的方法，但需积累更多病例进一步证实。

Objective To study the treatment and outcome of childhood endodermal sinus tumor. Methods The clinical data of twelve children with endodermal sinus tumor between April 2000 and July 2013 were reviewed. The basic demographics, stages of the lesion and the treatment outcome were analyzed. Of the twelve patients, seven were boys and five were girls. The age of the disease onset was between 1 and 3.3 years, except one in 11 years. Two patients were in Brodeur Stage I, four in Stage II, two in Stage III, and four in Stage IV. One patient underwent surgery alone, one underwent surgery plus a combination therapy with vincristine, actinomycin and cyclophosphamide （VAC）, and the other ten were treated by surgery with the use of cisplatin, etoposide and bleomycin （PEB） before or after the operation. Results Eleven patients were successfully followed up and ten were alive. The length of survival was 4.5 to 66 months in the 10 patients. In the 10 patients treated with PEB before or after surgery, 8 achieved complete remission, one achieved partial remission and one was not followed up. The major complications associated with the PEB regimen included myelosuppression and gastrointestinal upset symptoms and no late toxicity was observed. Conclusions Preoperative or postoperative administration of PEB may be an effective and safe management modality for childhood endodermal sinus tumor. Nevertheless, further validation is warranted in prospective studies involving a larger sample size.