Babin E A, Davis J R, Hatch K D, Hallum A V
Department of Obstetrics and Gynecology, University of Arizona Health Sciences Center, Tucson, Arizona.
Gynecol Oncol. 2000 Jan;76(1):107-11. doi: 10.1006/gyno.1999.5625.
Extrarenal Wilms' tumors are rare, with only 55 well-documented cases in the literature and only 4 confined to the uterus. A fifth case along with review of the current literature is presented.
A 13-year-old girl presented with a 2-month history of irregular vaginal spotting. A 6.5 x 4.5 x 2.5-cm polypoid mass meeting criteria for an extrarenal Wilms tumor was removed from the superior aspect of the cervix, but recurred 8 months later on the posterior lip. The patient underwent a Schauta procedure, lymph node dissection, and bilateral oophoropexy. The surgery was followed by typical renal Wilms' tumor chemotherapy. The patient is without evidence of disease 5 years after surgery.
This case uniquely suggests that limited excision of the original tumor has inherent risks for relapse. Oophoropexy appears to be reasonable when fertility preservation is desired and reconfirms that treatment mimicking renal nephroblastomas offers good long-term survival.
肾外威尔姆斯瘤罕见，文献中仅有55例记录详实的病例，其中仅4例局限于子宫。本文报告第5例病例并对当前文献进行综述。
一名13岁女孩，有2个月不规则阴道点滴出血史。从宫颈上方切除一个6.5×4.5×2.5厘米的息肉样肿物，符合肾外威尔姆斯瘤标准，但8个月后在后唇复发。患者接受了绍塔手术、淋巴结清扫和双侧卵巢固定术。术后进行了典型的肾威尔姆斯瘤化疗。术后5年，患者无疾病迹象。
该病例独特地表明，对原发肿瘤进行有限切除存在复发的固有风险。当希望保留生育功能时，卵巢固定术似乎是合理的，并且再次证实模仿肾母细胞瘤的治疗可带来良好的长期生存。
