一名7岁男孩患伴有Xp11.2易位的肾细胞癌。
Renal cell carcinoma with Xp11.2 translocation in a 7-year-old boy.
作者信息
Jayasinghe C, Siegler N, Leuschner I, Fleischhack G, Born M, Müller A M
机构信息
Department of Paidopathology, Institute of Pathology, Medical Center, University of Bonn, Germany.
出版信息
Klin Padiatr. 2010 May;222(3):187-9. doi: 10.1055/s-0030-1252011. Epub 2010 May 31.
BACKGROUND
More than 90% of pediatric renal tumors are nephroblastomas while renal cell carcinomas (RCC) are rare in children (< 5%).
PATIENT
According to the clinical diagnoses of a nephroblastoma stage IV a 7-year-old boy with a kidney tumor and peripheral pulmonary lesion was preoperatively treated for 8 weeks with Vincristine, Actinomycin D and Adriamycin. The resected kidney displayed a RCC with Xp11.2 translocation. There was no tumor regression and the pulmonary lesion was no longer detectable. Hence chemotherapy was put to a halt.
CONCLUSION
Fine needle aspiration biopsy (FNA) would have allowed to adjust the tumor subtype. Prognosis of pediatric RCC with translocation seems more favourable than without translocation though definitive evidence will only be possible by documentation in a clinical diagnose-related register.
背景
超过90%的小儿肾肿瘤为肾母细胞瘤，而肾细胞癌（RCC）在儿童中较为罕见（<5%）。
患者
根据IV期肾母细胞瘤的临床诊断，一名患有肾肿瘤和肺部周围病变的7岁男孩术前接受了8周的长春新碱、放线菌素D和阿霉素治疗。切除的肾脏显示为伴有Xp11.2易位的肾细胞癌。肿瘤无消退，肺部病变不再可检测到。因此停止了化疗。
结论
细针穿刺活检（FNA）本可用于调整肿瘤亚型。伴有易位的小儿肾细胞癌的预后似乎比无易位的更有利，不过只有通过临床诊断相关登记处的记录才能获得确凿证据。
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