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散发性肾血管母细胞瘤:三例临床病理研究及文献复习。

Sporadic hemangioblastoma of the kidney: a clinicopathologic study of three cases and a literature review.

机构信息

Department of Pathology, Shanxian Central Hospital, Heze, China.

Department of Ultrasound, Shanxian Central Hospital, Heze, China.

出版信息

J Int Med Res. 2021 Jul;49(7):3000605211027774. doi: 10.1177/03000605211027774.

Abstract

Much attention has been paid to renal hemangioblastoma, but there are still challenges in its differential diagnosis. Three cases (2 men, 1 woman; age: 40-56 years) presented with renal tumors. The tumors were surrounded by a thick fibrous capsule, well-demarcated from the surrounding renal parenchyma, and composed of sheets or nests of polygonal to short spindle-shaped tumor cells with a rich capillary network. In cases 1 and 3, the large polygonal tumor cells contained abundant pale or eosinophilic cytoplasm, and some possessed intracytoplasmic lipid vacuoles. In case 2, tumor cells were characterized by a uniform size, mild, clear, or lightly stained cytoplasm, and typical "clear cell" appearance. Immunohistochemistry revealed that the polygonal stromal cells were strongly and diffusely positive for α-inhibin, neuron-specific enolase (NSE), S100 protein, and vimentin. Cluster of differentiation (CD)10 and paired box gene (PAX)8 were positive, while epithelial membrane antigen (EMA) and cytokeratin (CK) were focally positive in case 3. CD34 and CD31 outlined the contours and distribution of the vascular networks. Renal hemangioblastoma is rare and prone to misdiagnosis; more attention should be paid to the morphological features and reasonable application of immunohistochemistry in the diagnosis of hemangioblastoma.

摘要

人们对肾脏血管平滑肌脂肪瘤给予了高度关注,但在其鉴别诊断方面仍存在挑战。本研究报道了 3 例肾脏肿瘤患者(2 男 1 女;年龄:40-56 岁)。肿瘤被厚纤维囊包围,与周围肾实质界限清楚,由片状或巢状多边形至短梭形肿瘤细胞组成,富含毛细血管网。在病例 1 和 3 中,大的多边形肿瘤细胞含有丰富的淡染或嗜酸性胞质,部分细胞含有细胞内脂滴。在病例 2 中,肿瘤细胞具有均匀的大小、轻度、透明或淡染的胞质,呈现典型的“透明细胞”外观。免疫组织化学显示,多边形基质细胞强烈且弥漫性地表达α-抑制素、神经元特异性烯醇化酶(NSE)、S100 蛋白和波形蛋白。分化簇(CD)10 和配对盒基因(PAX)8 阳性,而上皮膜抗原(EMA)和细胞角蛋白(CK)在病例 3 中局灶性阳性。CD34 和 CD31 描绘了血管网络的轮廓和分布。肾脏血管平滑肌脂肪瘤罕见,易误诊;在诊断血管平滑肌脂肪瘤时,应更加关注其形态学特征和免疫组织化学的合理应用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c129/8283055/0e42c28fb45a/10.1177_03000605211027774-fig1.jpg

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