眼眶炎性肌纤维母细胞瘤的临床和基础研究进展

Clinical and basic research progress of orbital inflammatory myofibroblastic tumor

炎性肌纤维母细胞瘤(IMT)在当前世界卫生组织软组织肿瘤组织学分型中被归类为纤维母细胞性或肌纤维母细胞性肿瘤中间型抑或偶见转移型。IMT由分化的肌纤维母细胞伴大量浆细胞、淋巴细胞和(或)嗜酸性粒细胞组成。它常发生于软组织和内脏中,而少见于眼部。IMT临床表现多样,可局部复发,少数可伴有全身症状和远端转移,罕见发生恶变。既往研究结果已明确,IMT是一种肿瘤性改变而非炎症过程,但其发病机制、是否存在可识别的组织学及预后特征等问题尚未明确。由于IMT具有潜在恶性的生物学行为,及时诊断和合理治疗此种肿瘤对患者预后至关重要。本文中笔者将从眼眶IMT的发现史、病因、发病机制、临床表现、影像学表现、病理组织学特征、诊断、鉴别诊断、治疗及预后进行综述。

The inflammatory myofibroblastic tumor(IMT)is classified as intermediate type of fibroblastic/myofibroblastic tumor(metastatic type is occasionally observed)in the current WHO soft tissue tumor histological classification.IMT consists of differentiated myofibroblastic spindle cells with numerous plasma cells,lymphocytes,and(or)eosinophils.It often occurs in the soft tissues and viscera,but rarely in the eyes.IMT has various clinical manifestations,some of which may locally recur,few of which may be accompanied by systemic symptoms and distal metastasis,and malignant transformation is rare.Literatures have demonstrated that IMT is a neoplastic change instead of the reactive process;however,its pathogenesis,and whether there is a recognizable histological prognosis feature have not yet determined.Due to the potentially malignant biological behavior of IMT,the prompt diagnosis and reasonable treatment of this tumor are essential for the patient′s prognosis.The research history,etiology and pathogenesis,clinical manifestations,imaging manifestations,histopathological characteristics,diagnosis and differential diagnosis,treatment and prognosis of orbital inflammatory myofibroblastic tumor were reviewed in this paper.