Andrews P E, Kelalis P P, Haase G M
Department of Urology, Mayo Clinic, Rochester, MN 55905.
J Pediatr Surg. 1992 Sep;27(9):1181-4. doi: 10.1016/0022-3468(92)90782-3.
Extrarenal Wilms' tumor is extremely rare and occurs predominantly in children. Eight cases of extrarenal Wilms' tumor were reported to the National Wilms' Tumor Study from 1980 to 1986. Patients were followed in the study and not randomized to a particular treatment protocol. Seven patients had a favorable histology. One tumor located in the sacrococcygeal region showed immature teratoma with nephroblastic tissue. The embryogenesis of extrarenal Wilms' tumor is controversial; however, tumor containing teratomatous elements most likely represents a different embryologic origin and, therefore, should be classified separately. All eight patients were treated with operative excision and chemotherapy. Seven of the eight patients were disease-free with a mean follow-up of 34.3 months. It can be inferred from this small group of patients that the prognosis is comparable to intrarenal Wilms' tumor in the National Wilms' Tumor Study.
肾外威尔姆斯瘤极为罕见，主要发生于儿童。1980年至1986年期间，有8例肾外威尔姆斯瘤病例报告给了国家威尔姆斯瘤研究项目。研究对患者进行了随访，并未将其随机分配至特定治疗方案。7例患者组织学表现良好。1例位于骶尾区域的肿瘤显示为含有肾母细胞组织的未成熟畸胎瘤。肾外威尔姆斯瘤的胚胎发生存在争议；然而，含有畸胎瘤成分的肿瘤很可能代表不同的胚胎学起源，因此应单独分类。所有8例患者均接受了手术切除及化疗。8例患者中有7例无疾病复发，平均随访时间为34.3个月。从这一小群患者中可以推断，其预后与国家威尔姆斯瘤研究中的肾内威尔姆斯瘤相当。
