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目的:探讨恶性孤立性纤维性肿瘤(malignant solitary fibrous tumor,MSFT)的临床病理特征、诊断、鉴别诊断、分子遗传学、治疗及预后。方法:对2例MSFT进行光镜、免疫组织化学研究,并复习相关文献。结果:男性2例,年龄分别为66岁及48岁。发生部位:骶前1例,临床表现为进行性二便障碍3年;后上纵隔1例,临床表现为活动后心慌半个月。肿瘤大小分别为10 cm×9 cm×6 cm及12 cm×10 cm×9 cm。镜检:细胞稀少区和细胞丰富区交替分布,两者之间有粗的玻璃样变胶原纤维分隔。密集区肿瘤细胞丰富,呈束状、漩涡状排列,可见血管外皮瘤样结构;瘤细胞呈梭形、短梭形,核染色质粗,异型性明显,有肿瘤性坏死、较多量核分裂(>4/10 HPF),局部出血。免疫表型:肿瘤细胞CD34,Bcl-2,CD99及Vimentin弥漫阳性;P53部分阳性,上皮细胞膜抗原局灶阳性;ki-67增殖指数20%～30%;S100,Desmin,C K及胶质纤维酸性蛋白阴性。结论:M SFT是一种少见的间叶肿瘤,确诊主要依靠形态学及免疫组织化学,需与血管外皮细胞瘤、恶性纤维组织细胞瘤、纤维肉瘤、滑膜肉瘤、恶性间皮瘤、恶性外周神经鞘膜瘤和脑膜瘤等相鉴别。MSFT的预后不良,易复发和转移,根治性切除是主要治疗手段,术后应长期随访。
Objective: To investigate the clinicopathological features, diagnosis, differential diagnosis, molecular genetics, treatment and prognosis of malignant solitary fibrous tumor (MSFT). Methods: Two cases of MSFT were examined by light microscopy and immunohistochemistry, and the related literatures were reviewed. Results: Two males were aged 66 and 48 years old respectively. Occur site: sacral anterior, clinical manifestations of progressive biliary disorders for 3 years; 1 case after the mediastinum, clinical manifestations of activity after half a month of palpitation. The tumor sizes were 10 cm × 9 cm × 6 cm and 12 cm × 10 cm × 9 cm, respectively. Microscopic examination: the cells are sparsely populated area and the cell-rich area alternating distribution between the two coarse glass-like collagen fibers separated. Tumor cells in the dense area were rich in bundles and arranged in a swirling pattern with vascular rumor-like structures. The tumor cells were fusiform, short fusiform, with coarse nuclear chromatin, obvious atypia, tumor necrosis, 4/10 HPF), local bleeding. Immunophenotype: The tumor cells were diffusely positive for CD34, Bcl-2, CD99 and Vimentin; P53 was partially positive and epithelial membrane antigen was focal; Ki-67 proliferation index was 20% -30%; S100, Desmin, CK and glial fibrillary acidic Protein negative. Conclusion: M SFT is an uncommon mesenchymal tumor. The diagnosis depends mainly on morphology and immunohistochemistry. M SFT is associated with hemangiopericytoma, malignant fibrous histiocytoma, fibrosarcoma, synovial sarcoma, malignant mesothelioma, malignant peripheral Nerve sheath tumor and meningioma phase identification. MSFT poor prognosis, easy to relapse and metastasis, radical resection is the main treatment, should be long-term follow-up.