肾原发性恶性孤立性纤维性肿瘤临床病理观察  

Primary renal malignant solitary fibrous tumor: a clinicopathologic analysis and review of the literature

作　　者：徐德[1] 程君[2] 周建平[2] 尹霞[1] 姜涛[1] XU De CHENG Jun ZHOU Jian-ping YIN Xia JIANG Tao(Department of Pathology, the Affiliated Hospital of Panzhihua University, Panzhihua 617000, China Department of Basic Medicine, Panzhihua University Medical College, Panzhihua 617000, China)

机构地区：[1]攀枝花学院附属医院病理科,四川攀枝花617000 [2]攀枝花学院医学院基础教研究室,四川攀枝花617000

出　　处：《诊断病理学杂志》2016年第9期676-679,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨肾原发性恶性孤立性纤维性肿瘤(MSFT)的临床病理特征及鉴别诊断,提高对肾MSFT的认识。方法观察1例肾MFST的临床、组织学和免疫组化表型特征,并复习国内外文献。结果患者男性,年龄54岁。体检发现左肾包块。CT示左肾下极12.6 cm×8.7 cm×8.3 cm大小肿块影,肿块内见稍低密度坏死灶,增强扫描病灶呈不均匀强化,可见液化坏死灶,考虑左肾癌。术中见左肾下极大小13 cm×9.5 cm×9 cm卵圆形肿块11个,肿瘤边界清楚,实性、质软,切面灰白、灰黄色。镜下见肿瘤细胞呈密集区和稀疏区交替分布,异型性明显,有出血、坏死,核分裂多见,12个/10HPF。免疫组化示肿瘤细胞vimentin、CD99、CD34、bcl-2弥漫强(+),Ki-67增殖指数20%,EMA、AE1/AE3、CD117、DOG1、desmin、SMA、S-100和HMB45(-)。结论肾MSFT非常罕见,临床及影像学易误诊为肾癌,确诊依靠组织形态学和免疫组化染色,组织学需要与肾肉瘤样肾细胞癌和其他恶性梭形细胞肿瘤鉴别。Objective To explore the clinicopathologic characteristics and differential diagnosis of primary renal malignant solitary fibrous tumor（ MSFT）,and to improve our understanding about the primary renal MSFT. Methods We reviewed the clinical characteristics in a case of primary renal MSFT and examined its histological and immunohistochemical features. Results The case of MSFT occurred in a 54 years old male patient. CT scan showed a mass shadow in the left kidney,sized 12. 6 cm × 8. 7 cm × 8. 3 cm,with slightly lower density necrosis. The enhanced scan showed inhomogeneous enhanced lesions and liquefied necrotic foci. Primary diagnosis was left renal cell carcinoma. This soft solid oval mass under left kidney had clear tumor boundary,with a size about 13 cm x 9. 5 cm x 9 cm. The cut section showed pale yellow in color. The microscopic observation showed that the tumor cells were distributed in dense and sparse regions,and the cell morphology was abnormal. There was bleeding,necrosis,and mitotic figures（ 12 /10 HPF）. The expression of Vimentin,CD34,CD99 and Bcl-2 in tumor cells was positive,and the expression of EMA,AE1 / AE3,CD117,desmin,DOG1,SMA,S-100,HMB45 in tumor cells was negative. The Ki-67 proliferation index was 20%.Conclusions Due to the rarity of renal MSFT,it is easy to be misdiagnosed as renal cell carcinoma by clinical and imaging methods. The diagnosis of renal MSFT depends on the histological and immunohistochemical staining. In histology,primary renal MSFT needs to be distinguished from renal sarcoma-like renal cell carcinoma and other malignant spindle cell tumors.

关 键 词：肾肿瘤 恶性孤立性纤维性肿瘤 临床病理 免疫组化 

分 类 号：R737.1[医药卫生—肿瘤]