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[孤立性纤维性肿瘤:26例临床病理及免疫组化特征]

[Solitary fibrous tumor: the clinicopathologic and immunohistochemical characteristics of 26 cases].

作者信息

Chen Hui-jiao, Zhang Hong-ying, Li Xiang, Guo Li-xin, Wei Bing, Guo Hua, Bu Hong, Yang Ke, Liu Bai-ling

机构信息

Department of Pathology, West China Hospital, Sichuan University, Chengdu 610041, China.

出版信息

Sichuan Da Xue Xue Bao Yi Xue Ban. 2004 Sep;35(5):675-9.

Abstract

OBJECTIVE

To study the clinicopathologic and immunohistochemical features of solitary fibrous tumor (SFT) and its biologic behaviour.

METHODS

Clinicopathologic data of 26 cases were collected and analyzed. Among them, immunohistochemical staining (Envision method) for CD34, vimentin, CD99, bcl-2, S-100, SMA, HBME-1, EMA and Ki67 were performed in 23 cases.

RESULTS

The 17 male and 9 female patients were between 15 and 66 years of age (mean, 44). Their SFTs were located in nose and nasopharynx, cranial cavity, soft tissue, mediastinum, and parietal and visceral pleura. The main clinical manifestations were local mass and pressure symptom. The characteristic microscopic features included patternless growth pattern, alternating hyper- and hypo-cellular areas, blunt spindle cells within keloid-like hyalinization, and hemangiopericytoma-like regions. Positive immunohistochemical staining: vimentin 100%(23/23), CD34 82%(19/23), bcl-2 87%(20/23), CD99 100%(23/23), SMA 30%(7/23). All cases were negative for S-100, HBME-1 and EMA. Follow-up information on 13 cases revealed that 3 patients had had relapse and died, the other 10 patients were alive without evidence of recurrence.

CONCLUSION

SFT is a rare mesenchymal spindle cell tumor which may be found in various parts of human body and needs to be distinguished from other spindle cell tumors by differential diagnosis. The immunophenotype can be of help in this connexion. About 10% to 23% SFTs have malignant behaviour, manifesting as local recurrence or metastasis. The behavior of SFT is unpredictable, which requires careful, longterm follow-up.

摘要

目的

研究孤立性纤维性肿瘤(SFT)的临床病理及免疫组化特征及其生物学行为。

方法

收集并分析26例患者的临床病理资料。其中23例进行了CD34、波形蛋白、CD99、bcl-2、S-100、平滑肌肌动蛋白(SMA)、人鼠杂交瘤单克隆抗体(HBME-1)、上皮膜抗原(EMA)及Ki67的免疫组化染色(Envision法)。

结果

17例男性和9例女性患者,年龄15至66岁(平均44岁)。其SFT位于鼻及鼻咽部、颅腔、软组织、纵隔以及壁层和脏层胸膜。主要临床表现为局部肿块及压迫症状。特征性镜下表现包括无规律生长模式、细胞疏密交替区域、瘢痕疙瘩样玻璃样变内的短梭形细胞以及血管外皮细胞瘤样区域。免疫组化染色阳性:波形蛋白100%(23/23),CD34 82%(19/23),bcl-2 87%(20/23),CD99 100%(23/23),SMA 30%(7/23)。所有病例S-100、HBME-1及EMA均为阴性。13例患者的随访信息显示,3例患者复发并死亡,另外10例患者存活且无复发迹象。

结论

SFT是一种罕见的间叶性梭形细胞肿瘤,可发生于人体各个部位,需通过鉴别诊断与其他梭形细胞肿瘤区分。免疫表型在这方面有帮助。约10%至23%的SFT具有恶性行为,表现为局部复发或转移。SFT的行为难以预测,需要仔细、长期的随访。

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