肾脏散发性横纹肌样血管母细胞瘤伴局灶性 CD10 表达：病例报告及文献复习。

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We present here an intriguing case of sporadic renal haemangioblastoma occurring in a 61-year-old male. The tumor consisted of nests of polygonal cells and abundant capillary networks. The neoplastic cells generally showed abundant eosinophilic cytoplasm and prominent eccentric nuclei, resembling the rhabdoid cells. Pronounced intranuclear cytoplasmic pseudoinclusions were another significant feature seen. NSE, a-inhibin and S100 were positive in tumor cells and particularly, focal CD10 expressions were observed. This is possibly the first reported case of a haemangioblastoma showing a rhabdoid phenotype and CD10 immunopositivity. Malignant rhabdoid tumor and renal cell carcinoma with rhabdoid features were probably the most challenging mimics need to be differentiated. The result of focal CD10 staining in our case may further lead to confusion with renal cell carcinoma. To avoid misdiagnosis, more considerations should be attached to the rare neoplasm.

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未注明

本文呈现了一例散发的肾血管母细胞瘤病例，发生于一名 61 岁男性。肿瘤由多边形细胞巢和丰富的毛细血管网络组成。肿瘤细胞通常具有丰富的嗜酸性细胞质和显著偏心的核，类似于横纹肌样细胞。显著的核内细胞质假包涵体是另一个重要特征。肿瘤细胞中 NSE、α-抑制素和 S100 阳性，并且观察到局灶性 CD10 表达。这可能是首例报道的具有横纹肌样表型和 CD10 免疫阳性的血管母细胞瘤。恶性横纹肌样肿瘤和具有横纹肌样特征的肾细胞癌可能是最具挑战性的需要鉴别的模拟物。在我们的病例中局灶性 CD10 染色的结果可能会进一步导致与肾细胞癌的混淆。为避免误诊，应更多地考虑这种罕见肿瘤。