Ewing sarcoma/primitive neuroectodermal tumor of the kidney in a child
Ewing sarcoma/primitive neuroectodermal tumor of the kidney in a child
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DOI：
10.1002/pbc.20831
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发表时间：
2008-01-01
影响因子：
3.2
通讯作者：
Hata, Jun-ichi
中科院分区：
文献类型：
--
作者：
Maeda, Miho;Tsuda, Akio;Hata, Jun-ichi
一名6岁女性因腹痛和右腹肿块入院。她的乳糖脱氢酶水平为1200IU/L，神经元特异性烯醇化酶水平为120 ng/ml。CT扫描显示右肾肿块较大，伴有坏死。行右肾根治性切除术。肿瘤完全被包裹住了。根据小圆形细胞组织学、MIC-2(CD99)强阳性肿瘤细胞和EWS-FLI-1融合转录本，诊断为肾脏尤文肉瘤/原始神经外胚层肿瘤。术后给予诱导和7个周期的化疗随访。她在确诊后90个月内没有复发的证据。
A 6-year-old female was admitted with abdominal pain and a mass in the right abdomen. Her lactose dehydrogenase level was 1,200 IU/L, and neuron specific enolase was 120 ng/ml. Computed tomography scan confirmed a large right renal mass with necrosis. A right radical nephrectomy was performed. The tumor was completely encapsulated. Based on small round cell histology, strong MIC-2 (CD99) positive tumor cells, and EWS-FLI-1 fusion transcript, Ewing sarcoma/primitive neuroectodermal tumor of the kidney was diagnosed. Induction and follow-up with seven cycles of chemotherapy were given after surgery. She has had no evidence of recurrence 90 months from diagnosis.