肝脏炎性肌纤维母细胞瘤研究进展

炎性肌纤维母细胞瘤,是指由分化的肌纤维母细胞性梭形细胞组成,常伴大量炎细胞和/或淋巴细胞的一种间叶性肿瘤.炎性肌纤维母细胞瘤病因未明,可见于身体各个部位,发病年龄多见于儿童和青少年.肝脏炎性肌纤维母细胞瘤多发于肝右叶,组织活检、CT和MRI有助于诊断和鉴别诊断,且免疫组化常表达波形蛋白(Vimentin),平滑肌肌动蛋白(SMA),肌特异性肌动蛋白(MSA),大部分患者经手术治疗后预后良好.本文复习国内外文献对发生于肝脏的炎性肌纤维母细胞瘤的病因及发病机制、临床表现、影像学特点、组织学形态、免疫组织化学及电镜特征、诊断及鉴别诊断、治疗等问题作系统综述,以期提高对肝脏炎性肌纤维母细胞瘤的认识.

Advancement of research on inflammatory myofibroblastic tumor of the liver

Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm composed of trans- formed myofibroblastic spindle cells with infil- tration of a large number of inflammatory cells and/or lymphocytes.The etiology of IMT re- mains unknown.IMT occurs at various sites and often affects young adults and children.IMT of the liver mostly arises in the right lobe of liver. Biopsy,computed tomography (CT) and mag- netic resonance imaging (MRI) are beneficial to diagnosis and differential diagnosis.Vimentin, smooth muscle actin (SMA),muscle-specific actin (MSA) are often positive by immunohis- tochemistry.The prognosis of most patients is favorable after surgery.In this article,we re- viewed the overseas and domestic literatures and summarized the advancement of research on the IMT of the liver about etiology,patho- genesis,clinical presentations,imaging feature, histological appearance,immunohistochemical and electron microscopic feature,diagnosis,dif- ferential diagnosis and therapy.