Paya K, Horcher E, Lawrenz K, Rebhandl W, Zoubek A
Department of Pediatric Surgery, University of Vienna, Austria.
Eur J Pediatr Surg. 2001 Apr;11(2):99-104. doi: 10.1055/s-2001-13787.
Four to eight percent of all Wilms' tumors are bilateral. Achieving curative resection of such tumors by partial nephrectomy or tumor enucleation while maintaining sufficient renal function represents a surgical challenge. Effective preoperative chemotherapy facilitates this aim considerably. Seven patients who were diagnosed to have bilateral synchronous Wilms' tumors between 1990 and 1994 are being reviewed. At the time of initial diagnosis, their mean age was 24.4 months, range 7 to 45 months. In 4 cases, pre-operative imaging did not reveal the full extent of the lesions, and in one of these, involvement of the second kidney was only detected by surgical exploration. Five of the children received at least 4 weeks of neo-adjuvant chemotherapy without primary biopsy, followed by bilateral tumor resection. One child had to be operated on as an emergency without any preliminary treatment, and in one, chemotherapy was interrupted after 3 weeks because of veno-occlusive disease. After a follow-up period of 66 months on the average (range 50 to 81 months), five of the patients are free of recurrence and clinically well--one with a kidney graft. The remaining two patients have died. Discussion is focussed on different management strategies of this rather rare pathology considering SIOP und NWTS protocols.
所有肾母细胞瘤中有4%至8%为双侧性。通过部分肾切除术或肿瘤剜除术实现此类肿瘤的根治性切除,同时维持足够的肾功能,是一项外科挑战。有效的术前化疗极大地有助于实现这一目标。现对1990年至1994年间被诊断为双侧同步性肾母细胞瘤的7例患者进行回顾。初诊时,他们的平均年龄为24.4个月,范围在7至45个月。4例患者术前影像学检查未显示病变的全部范围,其中1例仅通过手术探查才发现第二侧肾脏受累。5名儿童在未进行原发灶活检的情况下接受了至少4周的新辅助化疗,随后进行双侧肿瘤切除。1名儿童未经任何初步治疗作为急诊进行了手术,1名儿童因静脉闭塞性疾病在3周后中断了化疗。平均随访66个月(范围50至81个月)后,5例患者无复发且临床状况良好——其中1例有肾移植。其余2例患者已死亡。讨论聚焦于考虑到国际小儿肿瘤学会(SIOP)和美国国立威尔姆斯瘤研究组(NWTS)方案的这种相当罕见病理情况的不同管理策略。