Hwang E H, Han S J, Lee M K, Lyu C J, Kim B S
Department of Pediatric Surgery, Yonsei University College of Medicine, Seoul, Korea.
J Pediatr Surg. 1996 Feb;31(2):219-22. doi: 10.1016/s0022-3468(96)90000-7.
Two cases of vaginal endodermal sinus tumor (EST), a rare pediatric malignancy, were managed with conservative surgery followed by adjuvant chemotherapy. The first case involved a 9-month-old girl with EST of the vagina, who was treated with a partial vaginectomy and VAC regimen (vincristine, actinomycin D, cyclophosphamide) during a 2-year period. The serum alpha-fetoprotein (AFP) level returned to normal after excision of the tumor, and it remained normal throughout the treatment period. There was no evidence of disease 30 months after diagnosis. The second case involved an 8-month-old girl with EST of the vagina, who was treated with local excision of the mass through a vaginotomy. The VAC regimen was administered, but the serum AFP level remained elevated. A follow-up abdominopelvic computed tomography scan, taken 4 months after the operation, showed local recurrence of the tumor. The VAC regimen was then changed to a BEP regimen (bleomycin, etoposide, cisplatin). The serum AFP level returned to normal after 2 courses of the new regimen, and no tumor was visible on the follow-up magnetic resonance imaging study. For vaginal EST, primary conservative surgery and adjuvant chemotherapy are attractive measures to preserve both reproductive and sexual function. The extent of conservative surgery requires at least a partial vaginectomy. Simple tumor excision may not be adequate to achieve cure or to prevent local recurrence, even with adjuvant chemotherapy. The serum AFP level is useful for diagnosing and monitoring vaginal EST in the infant.
两例阴道内胚窦瘤(EST),一种罕见的儿科恶性肿瘤,采用保守手术加辅助化疗进行治疗。第一例为一名9个月大的患有阴道EST的女孩,在两年时间里接受了部分阴道切除术和VAC方案(长春新碱、放线菌素D、环磷酰胺)治疗。肿瘤切除后血清甲胎蛋白(AFP)水平恢复正常,且在整个治疗期间一直保持正常。诊断后30个月无疾病证据。第二例为一名8个月大的患有阴道EST的女孩,通过阴道切开术对肿块进行了局部切除。给予了VAC方案,但血清AFP水平仍升高。术后4个月进行的腹部盆腔计算机断层扫描显示肿瘤局部复发。然后将VAC方案改为BEP方案(博来霉素、依托泊苷、顺铂)。新方案两个疗程后血清AFP水平恢复正常,随访磁共振成像检查未见肿瘤。对于阴道EST,原发性保守手术和辅助化疗是保留生殖和性功能的有吸引力的措施。保守手术的范围至少需要部分阴道切除术。即使进行辅助化疗,单纯肿瘤切除可能不足以实现治愈或预防局部复发。血清AFP水平对诊断和监测婴儿阴道EST很有用。