胸膜孤立性纤维性肿瘤的临床病理特征和外科治疗

The clinicopathological features and surgical treatment of solitary fibrous tumor of the pleura

目的探讨胸膜孤立性纤维性肿瘤(SFTP)的临床病理特征和外科治疗效果。方法对11例外科切除的 SFTP 患者进行临床、影像、病理学比较分析,并进行免疫组织化学检测及术后随访。结果 11例中男6例,女5例,发病年龄为35～73岁(平均53.7岁)。主要临床表现为咳嗽、胸痛等局部压迫症状,5例伴胸腔积液。3例电视胸腔镜(VATS)下切除肿块,2例 VATS 辅助小切口开胸切除肿块,6例开胸切除肿块。肺楔形切除8例,中下叶切除、下叶切除和胸壁肿块切除各1例。肿块位于脏胸膜9例,脏胸膜下和壁胸膜各1例。病理形态学主要表现为梭形瘤细胞弥漫分布,间质中可见丰富粗大的胶原纤维和厚壁的血管;恶性肿瘤表现为细胞密度高、细胞核异型性、坏死和出现病理性核分裂。免疫组化显示梭形瘤细胞表达波形蛋白(100%,11/11)、CD_(34)(100%,11/11)和 CD_(99)(63.6%,7/11)及 bcl-2(63.6%,7/11),不表达 CD_(31)。病理诊断 SFTP 良性9例,富于细胞型和恶性各1例。术后并发多器官衰竭死亡1例。术后住院天数平均11 d。随访10例,随访时间12～48个月,均未见复发和转移。结论 X 线胸片和 CT 表现为胸膜单个结节或巨大阴影时应考虑 SFTP 的可能;伴有胸腔积液时易误诊;确诊依赖于组织病理学和免疫组织化学检查;VATS 或者 VATS 辅助小切口开胸病灶切除对于直径<5 cm 的肿块是一种很好的手术方式。

Objective To investigate the clinicopathological features and surgical treatment of solitary fibrous tumor of the pleura（SFTP）. Methods The clinical, radiological and pathological features of 11 cases of SFTP were analyzed. The detection of immunohistochemistry, surgical treatment and follow-up data were also reviewed. Results The group consisted of 6 males and 5 females, age ranging from 35 to 73 years （ mean 53. 7 years）. Clinical findings were mainly cough, chest pain and other local symptoms. Five cases had pleurai effusion. Surgical excision was performed by thoracotomy in 6 patients, by video-assisted thoracoscopic surgery （VATS） in 3 patients, and by VATS plus a small thoracotomy in 2 patients. Eight cases received tumor resection by wedge resection of the lung, and the other 3 patients by middle and lower lobe resection, lower lobe resection and chest wall tumor resection, respectively. Nine tumors were located in the visceral pleura, 1 tumor below the visceral pleura, and 1 tumor in parietal pleura. HistopathologicaUy, SFTP consisted mainly of spindle-shaped cells which were diffusely distributed. There were abundant gross collagenons fibers and thick-walled blood vessels around tumor cells. Malignant tumors displayed high cell density, nuclear atypia and patho-caryecinesis. The tumor cells were stained with vimentin（ 100%, 11/11 ）, CD34 （ 100%, 11/11 ）, CD99 （63.6%, 7/11 ）, and bel-2 （ 63. 6%, 7/11 ）, but no expression of CDn. Of the 11 cases, 9 were histologically diagnosed as benign SFTP, 1 as cell-abundant type of SFTP, and another as malignant SFTP. One patient died of multiple organ failure after surgery. The mean duration of hospital stay after surgery was 11 days. Follow-up visit results were available for 10 cases. The follow-up time ranged from 12 to 48 months and all patients remained well without recurrence or metastasis. Conclusions SFTP should be considered when chest X-ray and CT showed single nodule or mass in the pleura. The diagnosis is based on examination of histopathology and immunohistechemistry. For a tumor with a diameter les than 5 cm, resection by VATS or VATS pins small thoracotomy is a good surgical procedure.