26例孤立性纤维性肿瘤临床病理及免疫组化特征  

Solitary Fibrous Tumor: The Clinicopathologic and Immunohistochemical Characteristics of 26 Cases

作　　者：陈卉娇[1] 张红英[1] 李响[2] 郭立新[1] 魏兵[1] 郭华[1] 步宏[1] 杨可[1] 刘柏龄[1] 

机构地区：[1]四川大学华西医院病理科,成都610041 [2]四川大学华西医院泌尿科

出　　处：《四川大学学报（医学版）》2004年第5期675-679,共5页Journal of Sichuan University(Medical Sciences)

基　　金：纽约中华医学基金会 ( 0 0 -72 2 )资助

摘　　要：目的　分析孤立性纤维性肿瘤的临床表现、病理形态学、免疫组织化学及生物学行为。方法　对 2 6例孤立性纤维性肿瘤进行肿瘤大体及镜下观察、临床资料分析及随访 ;对 2 3例标本的石蜡切片行 CD34、 vi-mentin、 CD99、 bcl- 2、S- 10 0、SMA、 HBME- 1、EMA和 Ki6 7等免疫组织化学 Envision法染色。结果　本组发病年龄为 15到 6 6岁 ,平均 4 4岁 ;17例为男性 ,9例为女性。发生部位包括鼻腔及鼻咽部、颅内、躯体软组织、纵隔及胸膜等。临床主要表现为局部肿块及其引起的压迫症状。主要病理学改变包括 :无构像的生长模式 (patternlessness) ,细胞密集区与细胞疏松区 ,圆钝的梭形细胞混杂于瘢痕样的胶原中及典型的“血管外皮瘤样”区域。免疫组织化学结果 :vimentin阳性率 10 0 % (2 3/ 2 3) ,CD3482 % (19/ 2 3) ,bcl- 2 87% (2 0 / 2 3) ,CD9910 0 % (2 3/ 2 3) ,SMA 30 % (7/2 3) ,S- 10 0、HBME- 1及 EMA均为阴性。随访 :回访共 13例 ,10例无瘤生存 ,3例于肿瘤复发后死亡。结论　孤立性纤维性肿瘤可发生在全身各部位。需与其他多种梭形细胞肿瘤进行鉴别诊断 ,其组织学特点及免疫组织化学染色可以帮助鉴别。约 10 %～ 2 3%孤立性纤维性肿瘤具恶性的生物学行为 ,表现为局部复发或远处转移 ,其组织学构像?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, long-term follow-up.

关 键 词：孤立性纤维性肿瘤 免疫组织化学 CD34 BCL-2 CD99预后 

分 类 号：R730.4[医药卫生—肿瘤]