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目的探讨胸膜外恶性孤立性纤维性肿瘤(SFT)的临床病理特征、诊断、鉴别诊断及预后。方法回顾性分析2例胸外恶性孤立性纤维性肿瘤,并复习相关文献。结果 2例患者均为男性,年龄分别为59岁和76岁,1例肿瘤位于右顶部大脑镰旁,另1例位于右肩部,肿瘤大小分别为3 cm×3 cm×2 cm和8 cm×6 cm×3 cm。镜下肿瘤组织呈席纹状、编织状、漩涡状结构,散在有胶原分布。瘤细胞以梭形细胞为主,细胞核呈短梭型或卵圆形,颅内肿瘤细胞异型性不明显,肩部肿瘤细胞异型性非常明显,可见巨核、染色质粗颗粒状的细胞。2例核分裂象均易见。免疫组化:肿瘤细胞vimentin、CD34、STAT6、bcl-2和CD99均(+),S-100、desmin、EMA和Actin均(-)。结论发生在颅内及肩部的恶性SFT临床较少见,易与其它软组织肿瘤混淆,需与脑膜瘤、周围神经鞘膜瘤、多形性脂肪肉瘤、滑膜肉瘤鉴别,确诊需要依靠病理学形态及免疫表型。
Objective To investigate the clinicopathological features, diagnosis, differential diagnosis and prognosis of extrapleural malignant solitary fibrous tumor (SFT). Methods Two cases of malignant thoracic solitary fibrous tumor were retrospectively reviewed and related literatures were reviewed. Results Both patients were male and were 59 and 76 years old respectively. One tumor was located beside the falx on the right top and the other was located on the right shoulder. The tumor size was 3 cm × 3 cm × 2 cm and 8 cm, respectively × 6 cm × 3 cm. Microscopic tumor tissue was satin stripes, braided, swirling structure, scattered collagen distribution. Spindle cells dominated tumor cells were short spindle or oval, intracranial tumor cell atypia is not obvious, atypical shoulder tumor cells is very obvious, showing the megakaryocyte, chromatin coarse granular cells. 2 cases of mitosis are easy to see. Immunohistochemistry: Both tumor cells vimentin, CD34, STAT6, bcl-2 and CD99 (+), S-100, desmin, EMA and Actin were (-). Conclusions Malignant SFT occurred in the intracranial and the shoulder is rare and easily confused with other soft tissue tumors. It needs to be differentiated from meningioma, peripheral nerve sheath tumor, pleomorphic liposarcoma and synovial sarcoma. The diagnosis depends on the pathology Morphology and immunophenotype.