组织细胞肉瘤6例临床分析及文献复习

Clinical analysis of six histiocytic sarcoma cases and literature review

目的分析组织细胞肉瘤的临床特点、诊断及治疗。方法回顾性分析2002年1月—2016年1月在解放军总医院住院并病理确诊的6例组织细胞肉瘤患者的临床资料、病理、治疗与预后。结果 6例患者中,男4例,女2例;病变部位:肺组织、胸膜、淋巴结、腹膜后软组织、下肢软组织及颅骨。首发临床表现:咳嗽、胸闷、胸痛、发热,左肩胛疼痛、尿频、尿急、皮下结节、走路不稳。初诊误诊4例。病理组织活检:肿瘤性组织细胞弥漫性排列,体积中等偏大,多呈圆形、椭圆形,部分细胞梭形,细胞核大,有显著核仁,核偏位,核分裂相易见;免疫组织化学染色:CD68阳性6例,溶菌酶阳性5例,PAX5、CD1a、CD21和CD30均为阴性。手术联合化疗3例,仅化疗2例,手术联合头部放疗及化疗1例。随访6例,存活3例,死亡3例。结论组织细胞肉瘤可累及多系统,其临床表现无特异性,依病变部位而异,误诊率高。诊断主要依靠组织病理和免疫组化检查。治疗方法以手术为主,可联合放疗和化疗,预后较差。

Objective To analyze the clinical feature and investigate the diagnostic criteria,differential diagnosis,clinical management and prognostic factors of histiocytic sarcoma（HS）.Methods Retrospective analysis clinical data,pathology,therapy and prognosis of six patients with histiocytic sarcoma from 2002 January to 2016 January in Chinese PLA General Hospital.Results In the six cases,including lour male and two lemale,the primary tumors occur respectively in pleura,lung,lymph nodes,soft tissue,peritoneum soft tissue and skull.The early clinical symptoms are two cases of cough,chest tightness,lever;one case of pain,frequent or urgent micturition,walking instability.The typical microscopic pathological feature is moderate large cells,oval or spindle,and large nucleoli.Nuclear fission is common.All cases were reactive for CD68.Five expressed lysozyme and lour expressed CD45 RO.All cases were negative for PAX5,CD1 a,CD21 and CD30.Four of six were misdiagnosed at the preliminary.Three patients were treated with therapy and chemotherapy;two cases were treated only with chemotherapy,and one case with therapy and postoperative radiation.During the follow up,1 case was dead in three months after diagnosis.Conclusion HS can involve multiple systems,its clinical manifestations are nonspecific,vary according to the location of lesions,misdiagnosis rale is high.The diagnosis mainly depends on hislopalhology and immunohistochemistry.Treatment mainly with surgery,combined with radiotherapy and chemotherapy,poor prognosis.