肝脏炎性肌纤维母细胞瘤的临床特点及治疗策略

Clinical characteristics and treatment strategies of hepatic inflammatory myofibroblastic tumor

目的探讨肝脏炎性肌纤维母细胞瘤(HIMT)的临床特点及治疗方法。方法回顾性分析中国人民解放军总医院第五医学中心2011年1月到2022年11月诊治的15例HIMT患者的临床资料,总结其临床特征及治疗策略。结果15例中男11例,女4例,平均年龄(51.1±8.7)岁,有症状者8例,发热3例,合并慢性肝炎7例,术前正确诊断3例。3例患者PET/CT检查提示病灶表现为高代谢。免疫组化以Vimentin及平滑肌肌动蛋白(SMA)阳性为主。8例接受手术治疗,至今均未出现复发;7例观察治疗,其中3例病灶自发消退,3例缓慢缩小,1例病灶稳定。结论HIMT没有特异性的临床特征,不明原因感染、创伤和自身免疫功能异常等导致的机体过度炎症反应可能是其病因,治疗策略仍以手术切除为首选,密切观察也是一种可供选择的治疗方法。对无法手术、复发或转移性HIMT患者,基因检测可能有助于全身治疗方案的选择。

Objective To discuss the clinical features and treatment methods of hepatic inflammatory myofibroblastic tumor(HIMT).Methods Retrospective analysis of clinical data of 15 HIMT patients from Jan.2011 to Nov.2022 in the Fifth Medical Center of Chinese PLA General Hospital was conducted.The clinical features and treatment strategies were summarized.Results Among the 15 HIMT patients,there were 11 males and 4 females with a mean age of(51.1±8.7)years,8 symptomatic cases,3 cases with fever,7 cases with combined chronic hepatitis,3 cases with correct preoperative diagnosis.PET/CT examination suggested focal hypermetabolism in 3 patients.Immunohistochemistry was predominantly positive for Vimentin and smooth muscle actin(SMA).Eight cases underwent surgical treatment and none of them had recurrence so far.Seven patients underwent observation and treatment,among them,spontaneous regression of lesions in 3 cases,slow shrinkage in 3 cases,and stable lesions in 1 case.Conclusion HIMT has no specific clinical features,and excessive inflammatory response of the body caused by unexplained infection,trauma and abnormal autoimmune function,may be its etiology.Surgical resection is still the first choice therapy.Close observation is also an alternative treatment method.The genetic testing maybe helpful in the systemic treatment options for inoperable,recurrent or metastatic HIMT patients.