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A Wilms Tumor with Spinal Cord Compression: An Extrarenal Origin?

机译:脊髓压迫的威尔姆氏肿瘤:肾外起源吗?

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摘要

Spinal cord compression in Wilms' tumor (WT) is an extremely rare event that can have a very poor prognosis if not taken care of rapidly. Most cases reported in the literature involve widely metastatic patient with bone or paraspinal metastases or occasionally intradural metastasis. Here, we present the case of a 3-year-old girl of WT confirmed by biopsy, with spinal cord compression due to the direct contiguous spread of a tumor through 2 vertebral foramina. Abdominal ultrasonography and magnetic resonance imaging performed for an abdominal mass revealed a large heterogeneous tumor near the upper pole of the left kidney. A nodular infiltration extended through the T11-L1 and L1-L2 neural foramina, forming an intraspinal mass that compressed the spinal cord. Major paresthesia subsequently occurred, requiring urgent treatment with corticosteroids and chemotherapy. The evolution was rapidly satisfying. After six courses of chemotherapy, a left nephrectomy was performed. Macroscopic examination identified a large tumor attached to the kidney without renal infiltration. Microscopical examination concluded to a nephroblastoma with regressive changes, of intermediate risk. Evolution at 6 months is satisfactory, with no neurological deficit. The histological aspect of the tumor and the clinical outcome suggest that she had an extrarenal WT that spread through the vertebral foramina and was secondarily attached to the kidney.
机译:威尔姆斯肿瘤(WT)中的脊髓压迫是极为罕见的事件,如果不及时治疗,其预后很差。文献中报道的大多数病例涉及广泛转移的患者,该患者发生骨或椎旁转移或偶发硬膜内转移。在这里,我们呈现一例经活检证实为WT的3岁女孩的病例,由于肿瘤通过2个椎间孔直接连续扩散,脊髓受压。对腹部肿块进行的腹部超声检查和磁共振成像显示,左肾上极附近有较大的异质性肿瘤。结节性浸润延伸通过T11-L1和L1-L2神经孔,形成压缩脊髓的脊髓内块。随后发生大感觉异常,需要紧急用皮质类固醇和化学疗法治疗。演变迅速令人满意。经过六个疗程的化疗后,进行了左肾切除术。肉眼检查发现附着在肾脏上的大肿瘤没有肾脏浸润。镜检总结为肾母细胞瘤具有退行性改变,具有中等风险。 6个月时的进化令人满意,没有神经系统缺陷。肿瘤的组织学特征和临床结果表明,她的肾外WT扩散通过椎骨孔,其次是附着在肾脏上。

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