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肾脏原发恶性孤立性纤维瘤。

De novo malignant solitary fibrous tumor of the kidney.

机构信息

Department of Pathology, Chang Gung Memorial Hospital, Keelung, Taiwan.

出版信息

Diagn Pathol. 2011 Oct 5;6:96. doi: 10.1186/1746-1596-6-96.

Abstract

The kidney is a relatively infrequent site for solitary fibrous tumor (SFT). Among the previously reported cases, only two cases of malignant renal SFT developing via dedifferentiation from a pre-existing benign SFT have been reported. Here we reported a case of de novo malignant renal SFT clinically diagnosed as renal cell carcinoma in a 50-year-old woman. The tumor was circumscribed but unencapsulated and showed obvious hemorrhagic necrosis. Microscopically, the tumor was composed of patternless sheets of alternating hypercellular and hypocellular areas of spindle cells displaying mild to moderate nuclear atypia, frequent mitoses up to 8 per 10 high power fields, and a 20% Ki-67 proliferative index. Immunohistochemical studies revealed reactivity for CD34, CD99 and vimentin, with no staining for all other markers, confirming the diagnosis of SFT. No areas of dedifferentiation were seen after extensive sampling. Based on the pathologic and immunohistochemical features, a diagnosis of de novo malignant renal SFT was warranted. Our report expands the spectrum of malignant progression in renal SFTs. Even though this patient has been disease-free for 30 months, long-term follow-up is still mandatory.

摘要

肾脏是孤立性纤维瘤(SFT)相对少见的部位。在以前报道的病例中,仅有两例恶性肾 SFT 通过从先前存在的良性 SFT 去分化发展而来的报道。本文报道了一例 50 岁女性新发的恶性肾 SFT,临床诊断为肾细胞癌。肿瘤边界清楚但无包膜,并有明显出血坏死。镜下,肿瘤由交替的细胞丰富区和细胞稀疏区的无模式梭形细胞构成,显示轻度至中度核异型性,常见核分裂象,每 10 高倍视野多达 8 个,Ki-67 增殖指数为 20%。免疫组化研究显示 CD34、CD99 和波形蛋白阳性,所有其他标志物均无染色,证实了 SFT 的诊断。广泛取样后未见去分化区域。基于病理和免疫组化特征,诊断为新发的恶性肾 SFT。本报告扩展了肾 SFT 恶性进展的范围。尽管该患者无病生存 30 个月,但仍需长期随访。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6685/3195699/6e41d7074c89/1746-1596-6-96-1.jpg

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