孤立性纤维性肿瘤的临床病理研究

Clinicopathologic and immunohistochemical study of 18 solitary fibrous tumors cases

目的 探讨孤立性纤维性肿瘤（SFT）的临床及病理形态特征、诊断和鉴别诊断。方法 收集发生于不同部位的18例SFT患者,进行临床、组织学形态和免疫组化染色观察,并复习相关文献。结果 SFT患者临床主要表现为缓慢生长的肿块,全身各个解剖部位皆可发生,发病高峰在40~70岁。镜下由交替分布的细胞丰富区和细胞稀疏区组成,被纤维性间质分隔,胶原纤维丰富,并具有血管外皮瘤样分支状血管。免疫组化：瘤细胞特征性强阳性表达CD34和Bcl-2,Vimentin和CD99也呈阳性表达,不表达CK、S-100和SMA。但是,CD34和Bcl-2并不总是阳性,尤其是在恶性SFT患者。若CD34阴性,Bcl-2一般呈弥漫强阳性表达。结论 SFT是一种在形态学和免疫表型上比较有特征性的肿瘤,需要与其它梭形细胞肿瘤相鉴别。大部分病例完整切除可以治愈,但需要长期随访。

Objective To investigate the clinicopathologic characteristic, diagnosis and differential diagnosis of solitary fibrous tumors（SFTs）. Methods Eighteen SFT cases were analyzed based on clinical presentation, morphology, immunohistochemistry with review of the related literatures. Results The common clinical symptom of SFT was a slowly growing mass occurred to almost all anatomical sites in the patients. Histologically, the most essential characteristic was nonatypical spindle-shaped tumor cells on a collagenous background. Keloid-type collagen, hypocellular and hypercellular areas could be observed in all the cases. On immuno-histochemical analysis, it was found that mesenchymal markers such as CD34, bcl-2 , vimentin and CD99 were positive, and CK, S-100 protein and desmin were negative. If the result for CD34 was negative, bcl-2 would be strongly expressed. Conclusion Complete resection of SFT could usually cure the disease, while careful follow-up is still necessary. Morphological and pathological features are of great importance for differential diagnosis of the disease.