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目的探讨形成脂肪的孤立性纤维性肿瘤(FFSFT)的临床病理学特征、免疫学表型、诊断与鉴别诊断。方法对2例FFSFT进行临床病理学和免疫表型分析,并复习相关文献。结果 2例FFSFT患者均为男性,年龄分别为59和46岁。肿瘤分别位于左颈上至枕下区和右腹股沟,均实行全部肿瘤切除。大体检查,肿瘤界限清楚,切面灰白、灰黄色,质软、韧。大小分别为10 cm×6 cm×5 cm、4 cm×3.5 cm×2 cm。镜下肿瘤由成熟的脂肪细胞和血管外周细胞瘤样结构组成。血管外周细胞瘤样结构区域的肿瘤细胞呈梭形或卵圆形,细胞界限不清,胞质淡红染,细胞核呈梭形,偶见核仁。细胞形态温和,无细胞异型性和坏死,核分裂象罕见。免疫组织化学染色显示,肿瘤细胞弥漫高表达CD34、CD99、bcl-2和vimentin。结论 FFSFT是一种极为罕见的良性肿瘤,完整切除是其首选治疗方式。FFSFT需要和神经鞘瘤、血管平滑肌脂肪瘤、梭形细胞脂肪瘤等鉴别。
Objective To investigate the clinicopathological features, immunological phenotype, diagnosis and differential diagnosis of fat-formed solitary fibrous tumor (FFSFT). Methods Two cases of FFSFT were analyzed by clinicopathological and immunophenotype, and the related literatures were reviewed. Results Both FFSFT patients were male and their age were 59 and 46 respectively. Tumors were located on the left neck to the suboccipital and right groin, all the implementation of all tumor resection. General examination, clear tumor boundaries, gray section, gray, soft, tough. The sizes are 10 cm × 6 cm × 5 cm and 4 cm × 3.5 cm × 2 cm, respectively. Microscopic tumors consist of mature adipocytes and perivascular tumor-like structures. Tumor cells in the region of vascular peritumoral tumor-like structure are fusiform or oval, the cell boundaries are unclear, the cytoplasm is stained lightly, the cell nucleus is fusiform and the nucleoli are occasionally seen. Cell morphology is mild, cell-free atypia and necrosis, meiosis is rare. Immunohistochemical staining showed that the tumor cells diffuse high expression of CD34, CD99, bcl-2 and vimentin. Conclusion FFSFT is a very rare benign tumor. Complete resection is the first choice of treatment. FFSFT needs and schwannoma, angiomyolipoma, spindle cell lipoma and other identification.