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成年患者中具有独特病理特征的ALK重排相关性肾细胞癌(RCC)

ALK rearrangements-associated renal cell carcinoma (RCC) with unique pathological features in an adult.

作者信息

Jeanneau Marie, Gregoire Valerie, Desplechain Claude, Escande Fabienne, Tica Dan Petre, Aubert Sebastien, Leroy Xavier

机构信息

Department of Pathology, Univ. Lille, CHU, F-59000 Lille, France.

Sipath-Unilabs Laboratory, 63063 Clermont-Ferrand, France.

出版信息

Pathol Res Pract. 2016 Nov;212(11):1064-1066. doi: 10.1016/j.prp.2016.07.015. Epub 2016 Aug 2.

DOI:10.1016/j.prp.2016.07.015
PMID:27554841
Abstract

We report an exceptional case of renal cell carcinoma (RCC) associated with ALK translocation in an adult. The tumor was located in the left kidney and measured 4cm. The tumor was composed of sheets of large eosinophilic cells with frequently intracytoplasmic vacuoles. The nuclei were large with a nucleolar grade 3. At immunohistochemistry, tumor cells were diffusely positive for PAX8 and vimentin and focally stained with CK7. ALK immunostaining was diffuse and fluorescent in situ hybridization showed a rearrangement of ALK in numerous tumor cells. To date, less than 17 cases of RCCs with ALK rearrangements are reported in children and adults. The prognosis remains uncertain but ALK rearrangement may be of potential value for target therapy with crizotinib.

摘要

我们报告了一例成人肾细胞癌(RCC)伴有ALK易位的罕见病例。肿瘤位于左肾,大小为4厘米。肿瘤由大片嗜酸性大细胞组成,胞质内常有空泡。细胞核大,核仁分级为3级。免疫组化显示,肿瘤细胞PAX8和波形蛋白弥漫性阳性,CK7局灶性染色。ALK免疫染色弥漫,荧光原位杂交显示许多肿瘤细胞中ALK发生重排。迄今为止,儿童和成人中报道的伴有ALK重排的肾细胞癌病例少于17例。预后仍不确定,但ALK重排可能对克唑替尼靶向治疗具有潜在价值。

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