中国近25年脾脏炎性肌纤维母细胞瘤的流行病学特征及诊治经验

摘要

Objective: To investigate the epidemiological features, diagnosis and treatment of splenic inflammatory myofibroblastic tumor in China during the past 25 years. Methods: The clinical data of 64 patients diagnosed splenic inflammatory myofibroblastic tumor (Splenic inflammatory pseudotumor) published from 1985 to 2010 from various databases, including China National Knowledge Infrastructure (CNKI), VIP, and the epidemiological features, diagnosis and treatment of SIMT patients were retrospectively analyzed. Results: There were 64 patients, including 39 males and 25 females (1.56:1). And their age spread from 24 to 74 with an average of 49.2. Most of patients had not special symptoms, and were diagnosed through routine examinations such as type-B ultrasonic or CT scan. Epigastric pain, debilitation, low-grade fever and some other untypical symptoms might appear in some patients. A solitary splenic lesion might be seen during medical ima-geologcal examination. Histological types: hypocellular fibrous pattern (n=26), myxoid/vascular pattern (n=12), compact spindle cell pattern (n=5). Immunophenotypic findings show that the spindle cells were positive for Vimentin, SMA in most cases. For most patients, splenectomy were the mainly treatment, and there were no signs of relapse and metastasis after the operation. Conclusion: Splenic inflammatory myofibroblastic tumor is extremely rare in china, and there are not special clinical symptoms or signs. It can be identified by characteristics of medical imaging, histologic and immunophenotypic findings with other splenic lesions. But it is difficult to define its nature pre-operatively. Splenectomy is the mainly treatment with a satisfactory prognosis.%目的:探讨中国近25年来脾脏炎性肌纤维母细胞瘤(SIMT)的流行病学特征及诊治经验.方法:联合检索中国知网、维普等多家中文数据库获取国内近25年有关SIMT的病例资料进行回顾性分析.结果:本组64例中,男39例,女25例,男女比例1.56:1.年龄24～74岁,平均49.2岁.多为查体时B超或CT发现,部分表现为上腹部不适、乏力、低热等非特异性症状.影像学检查提示脾内单发或多发,界限多清楚占位性病变.有明确病理结果描述者43例:少细胞纤维型26例,黏液样/血管型1 2例,丰富梭形细胞型5例；免疫组化示多数病例梭形细胞对Vimentin、SMA等抗体阳性.治疗方式主要为脾脏切除,随访无复发及转移.结论:SIMT极为罕见,临床表现无特异性,通过影像学检查、病理组织学特点及免疫组化结果可与其他脾脏占位性病变相鉴别,术前诊断较为困难,治疗主要为脾切除术,预后良好.