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Cytomorphology and immunohistochemistry of extrarenal rhabdoid tumor: A case report with review of literature

机译:肾外横纹肌瘤的细胞形态学和免疫组化:1例报道并文献复习

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Extrarenal rhabdoid tumor (ERRT) is a rare, aggressive tumor with extremely poor prognosis. We report a case of ERRT with intraspinal extension in a 1.5-year-old child diagnosed by fine needle aspiration cytology (FNAC) and immunohistochemistry. The child presented with a right lumbar region lump of two months duration. Ultrasound guided FNAC was performed and cell block was prepared. Smears were highly cellular and showed a dispersed population of large round cells having abundant pale eosinophillic cytoplasm, centrally to eccentrically placed nucleus with large prominent nucleoli. Immunohistochemistry was carried out on cell block which was positive for epithelial membrane antigen EMA and Vimentin. It was negative for leucocyte common antigen [LCA], wilms tumor 1, WT1, desmin and neuron specific enolaseNSE, thus ruling out other tumors like lymphoma, Wilms tumor, rhabdomyosarcoma, and neuroblastoma. A final diagnosis of ERRT was given. ERRT is an extremely rare tumor of retroperitoneal area; it should be included in the differential diagnosis of malignant round cell tumor in children. Cell block in this case is mandatory for putting up the panel of immunohistochemistry which can clinch the diagnosis of rhabdoid tumor and treatment can be started as early as possible.
机译:肾外横纹肌样瘤(ERRT)是一种罕见的侵袭性肿瘤,预后极差。我们报告了一个1.5岁的细针穿刺细胞学(FNAC)和免疫组化诊断为ERRT并伴有椎内延伸的病例。这名儿童出现了两个月大的右腰区肿块。进行超声引导的FNAC并制备细胞块。涂片是高度细胞化的,并显示散布的圆形大细胞群,具有丰富的苍白嗜酸性细胞质,中心至偏心放置的核具有大的突出核仁。在上皮膜抗原EMA和波形蛋白阳性的细胞块上进行免疫组织化学。白细胞共同抗原[LCA],野生型肿瘤1,WT1,结蛋白和神经元特异性烯醇化酶NSE阴性,因此排除了其他肿瘤,如淋巴瘤,威尔姆斯瘤,横纹肌肉瘤和神经母细胞瘤。最终确定了ERRT。 ERRT是一种非常罕见的腹膜后区域肿瘤。它应包括在儿童恶性圆形细胞瘤的鉴别诊断中。在这种情况下,细胞阻断对于建立免疫组织化学研究小组是必不可少的,它可以帮助诊断横纹肌瘤,并且可以尽早开始治疗。

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