【摘要】 双侧肾母细胞瘤(bilateral Wilms’tumor,BWT)发病率极低,约占肾母细胞瘤(Wilms’tumor,WT)的5%。BWT的治疗原则是尽可能保留正常的肾功能,以降低终末期肾病(ESRD)及其他并发症发生的风险。BWT化疗以长春新碱/放线菌素D/阿霉素为主,不良组织型加用环磷酰胺、依托泊苷、卡铂等治疗。手术治疗主要采用保留肾单位手术。手术切缘阳性、有肿瘤溢出、淋巴结阳性以及病理类型为间变型者,应予以放疗。BWT复发后生存率低,可采用大剂量化疗并行自体造血干细胞移植以期提高患儿生存率,然其疗效仍需进一步探讨。更多还原

【Abstract】 Bilateral Wilms’ tumor(BWT) affects approximately 5% of children with WT. The goal of treatment in BWT is to preserve normal renal function as much as possible, in order to postpone and reduce the risk of end-stage renal disease and other long-term complications. For neoadjuvant chemotherapy, vincristine/dactinomycin/doxorubicin is used in cases with favorable histology. Cases with diffuse anaplasia or other unfavorable histology receive an intensified regimen, including additional agents such as ifosfamide, cyclophosphamide, carboplatin, and others. Nephron-sparing surgery is performed to preserve long-term renal function. Radiotherapy is given according to local staging, when margins or lymph nodes are positive, or tumor spillage has occurred, or pathological type is anaplasia. The survival rate of relapsed BWT is low. Some experts suggest that patients with recurrent BWT are most likely to benefit from high-dose therapy including stem-cell transplantation, but this remains controversial.更多还原