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[肾恶性横纹肌样瘤:2例报告并文献复习]

[Malignant rhabdoid tumor of the kidney: 2 cases and a review of the literature].

作者信息

Picazo García M L, Vicandi Plaza B, Fraga Fernández J, Burgos Lizalde E, Contreras Rubio F

机构信息

Departamento de Anatomía Patológica, Hospital La Paz, Madrid.

出版信息

An Esp Pediatr. 1988 Jun;28(6):540-4.

PMID:2848433
Abstract

Report of the microscopic, ultrastructural and immunohistochemical findings of two malignant rhabdoid tumors of the kidney in two males aged 2 1/2 and 19 months. The incidence of this tumor in our files is 1.9% of all the renal tumors of infancy. Both cases were formally considered to be rhabdomyosarcomatous variants of nephroblastomas. Microscopically a slightly cohesive polygonal cell proliferation is shown. The cells have intracytoplasmatic hyaline inclusions ultrastructurally identifiable as intermediate filament bundles, showing positivity to vimentin in the immunohistochemical technique. Malignant rhabdoid tumors are referred to as very aggressive tumors with a poor response to treatment and a high mortality rate. Association with central nervous tumors and hypercalcemia has been reported. The clinical and pathologic features make it possible to differentiate them from nephroblastomas, although the histogenesis is not definitely established.

摘要

两名年龄分别为2岁半和19个月的男性肾恶性横纹肌样瘤的显微镜检查、超微结构检查及免疫组化检查结果报告。该肿瘤在我们的病例档案中占婴儿期所有肾肿瘤的1.9%。这两例病例曾被正式认为是肾母细胞瘤的横纹肌肉瘤样变体。显微镜下可见轻度聚集的多边形细胞增殖。这些细胞有胞质内透明包涵体,超微结构上可识别为中间丝束,在免疫组化技术中对波形蛋白呈阳性反应。恶性横纹肌样瘤被认为是极具侵袭性的肿瘤,对治疗反应不佳且死亡率高。有报道称其与中枢神经肿瘤和高钙血症有关。尽管组织发生尚未明确确定,但临床和病理特征使其有可能与肾母细胞瘤相鉴别。

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