纵隔原发性恶性纤维组织细胞瘤的诊断与治疗

Diagnosis and Treatment of Primary Malignant Fibrous Histiocytoma in the Mediastinum

目的:探讨纵隔恶性纤维组织细胞瘤临床特点、病理特征、诊断和治疗。方法:回顾分析1992—2005年6例纵隔原发性恶性纤维组织细胞瘤的临床和病理资料。结果:全组均行手术治疗,姑息切除1例,纵隔肿瘤切除4例,纵隔肿瘤加右肺下叶背段和后基底段切除1例。术后病理检查确诊。术后无并发症和死亡。术前诊断为气管囊肿1例;支气管肺囊肿1例,肺恶性纤维组织细胞瘤1例;纵隔肿瘤3例。肿瘤细胞呈多形性,以梭形纤维母细胞和组织细胞样细胞为主,并混有各种过渡型细胞、未分化及多核巨细胞等,3例免疫组化检查示肿瘤细胞Vimentin、CD68、AACT及AAT均表达阳性。结论:该病临床症状无特征性,术前确诊极为困难,应结合临床表现和形态学及免疫组化标记综合考虑。该病应手术治疗,彻底切除是手术的关键。该病恶性度高,易复发和转移,预后不佳。

Objective：To study the clinical and histopathological characteristics of primary malignant fibrous histiocytoma in the mediastinum and to discuss the diagnostic criterion and thearapy. Methods：Clinical and histopathological record of six cases of primary malignant fibrous histioeytoma in the mediastinum in our hospital from 1992 to 2005 were reviewed, which included one case bronchus lung cyst before surgery,one case trachea cyst or teratoma,one case primary malignant lung fibrous histiocytoma and three cases mediastinum tumor. Results： All the patients received surgery including appeasement resection in one, mediastinum tumor resection in four; mediastinum tumor and lobus inferior segmentum apicale（SVI） and segmentum basale posterius（SX） resection in one, The postoperative diagnosis was confirmed by pathological examination. There were no postoperative complication and mortality. Microscopic findings showed that the tumor consisted of diffuse neoplastic histiocytes and spindle shaped fibroblasts arranged in a prominent storiform pattern intermingled with various transition cell,no differentiation cell and numerous multinucleared tumor giant cells. Tumor cells of three cases were positive for vimentin, CD68, AACT and AAT. Conclusion：Preoperative diagnosis of MFH is very difficult. All the clinical manifestation, morphological characteristics and immunohistochemical detection should be taken into account to confirm the diagnosis. The main treatment of MFH resorts to surgery and the key to cure is thorough resection. The disease has high degree malignancy, is easy to recur and metastize , usually with poor prognosis.