Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm that is characterized by a proliferation of myofibroblastic and fibroblastic spindle cells, accompanied by an inflammatory infiltrate that is abundant in plasma cells, lymphocytes, and eosinophils. IMT can arise in various anatomical locations but most commonly occurs in the abdominal cavity, retroperitoneum, and lung, particularly in children and young adults. IMT typically demonstrates local invasion or recurrence, whereas metastasis is rare. IMTs pose a diagnostic challenge because of their overlapping morphological characteristics with a heterogeneous group of nonneoplastic and neoplastic lesions. Precise diagnosis is crucial for optimal management and accurate prognostication. Despite recent advancements in IMT diagnosis and treatment, its biological complexity and clinical management remain challenging due to significant histological heterogeneity and molecular genetic diversity. This review provides comprehensive updates on the clinical, molecular, and pathological characteristics of IMT, highlighting the diagnostic approaches and key differential diagnoses.
炎性肌纤维母细胞瘤是一种罕见的肿瘤,其特征为肌纤维母细胞和纤维母细胞性梭形细胞增生,并伴有富含浆细胞、淋巴细胞和嗜酸性粒细胞的炎性浸润。该肿瘤可发生于多种解剖部位,但最常见于腹腔、腹膜后及肺部,尤其好发于儿童和青年群体。炎性肌纤维母细胞瘤通常表现为局部侵袭或复发,转移较为罕见。由于其形态学特征与多种非肿瘤性及肿瘤性病变存在重叠,该肿瘤的诊断颇具挑战性。精确诊断对于优化治疗及准确预后评估至关重要。尽管近年来在炎性肌纤维母细胞瘤的诊断与治疗方面取得进展,但由于其显著的组织学异质性和分子遗传多样性,其生物学复杂性及临床管理仍面临挑战。本综述系统更新了炎性肌纤维母细胞瘤的临床、分子及病理学特征,重点阐述了诊断方法及关键鉴别诊断要点。