Saarinen-Pihkala U M, Wikström S, Vettenranta K
Hospital for Children and Adolescents, University of Helsinki, Finland.
Bone Marrow Transplant. 1998 Jul;22(1):53-9. doi: 10.1038/sj.bmt.1701282.
In children with bilateral Wilms' tumor, the therapy should aim at maximal preservation of renal parenchyma and function. Local radiotherapy may give rise to second malignant neoplasms and may impair renal function. We present a therapeutic strategy without any irradiation. Three children were diagnosed with bilateral Wilms' tumor at ages from 6 months to 5 years. Each patient had a massive tumor with local stage III on one side; one had pulmonary metastases. The therapeutic strategy was first to obtain tissue for histology by percutaneous needle biopsy, to administer pre-operative chemotherapy until desired tumor shrinkage, and then to perform kidney-sparing resective surgery. After a period of conventional chemotherapy, the patients were consolidated with high-dose (HD) melphalan and ABMT. Renal parenchyma spared post-surgery (right/left) was 0%/70%, 60%/40% and 40%/60% of the original kidney volumes. The toxicity of the ABMT procedure was mild, the patients engrafted promptly, and were discharged on days +14 to +27. All patients survive disease-free, 3 years 4 months to 4 years 5 months post-transplant. Our program resulted in good preservation of renal parenchyma and normal function, and we consider the risk of this ABMT program smaller than the late consequences of local radiotherapy for children with bilateral Wilms' tumor. The therapeutic strategy described merits further evaluation.
对于双侧肾母细胞瘤患儿,治疗应旨在最大程度地保留肾实质和肾功能。局部放疗可能会引发第二原发性恶性肿瘤,并可能损害肾功能。我们提出一种不进行任何放疗的治疗策略。三名年龄在6个月至5岁之间的儿童被诊断为双侧肾母细胞瘤。每位患者一侧都有巨大肿瘤且局部分期为III期;其中一名有肺转移。治疗策略首先是通过经皮针吸活检获取组织进行组织学检查,给予术前化疗直至肿瘤缩小至理想程度,然后进行保留肾单位的切除手术。经过一段时间的常规化疗后,患者接受大剂量(HD)美法仑和自体骨髓移植(ABMT)巩固治疗。术后保留的肾实质(右/左)分别为原肾体积的0%/70%、60%/40%和40%/60%。ABMT程序的毒性轻微,患者迅速植入,并在第14天至第27天出院。所有患者均无病存活,移植后3年4个月至4年5个月。我们的方案使肾实质得到了良好保留且功能正常,并且我们认为对于双侧肾母细胞瘤患儿,这种ABMT方案的风险小于局部放疗的远期后果。所描述的治疗策略值得进一步评估。