Chaudhary Poras
Department of Surgery, Lady Hardinge Medical College and Associated Dr. Ram Manohar Lohia Hospital, New Delhi, India.
Ann Gastroenterol. 2015 Jan-Mar;28(1):49-54.
Inflammatory myofibroblastic tumors (IMTs), also known as inflammatory pseudotumors and inflammatory fibrosarcomas, are uncommon mesenchymal tumors composed of myofibroblastic spindle cells admixed with lymphocytes, plasma cells and eosinophils. Once thought to be reactive, these lesions are now considered to be neoplastic. These tumors can occur throughout the body, most commonly in the lung, mesentery and omentum. Patients commonly present with painless abdominal mass or with intestinal obstruction. IMTs may be multicentric, have a high local recurrence rate and may metastasize in rare cases. The lesions show wide variability in their histologic features and cellularity, and marked inflammatory infiltration, predominantly of plasmatocytes and lymphocytes, and occasionally neutrophils and eosinophils. Anaplastic lymphoma kinase (ALK) rearrangements and/or ALK1 and p80 immunoreactivity are reported in 33-67% of the tumors. Owing to the rarity of these lesions, there are no specific imaging findings that distinguish IMTs from other mesenteric masses. Complete surgical resection is the treatment of choice. Local recurrence rates are high, and re-excision is the preferred therapy for local recurrences. ALK-positive tumors show good response to ALK inhibitors. Current knowledge and comprehensive review of the available literature on IMTs is herein presented.
炎性肌纤维母细胞瘤(IMTs),也被称为炎性假瘤和炎性纤维肉瘤,是一种罕见的间叶性肿瘤,由肌纤维母细胞性梭形细胞与淋巴细胞、浆细胞和嗜酸性粒细胞混合组成。这些病变曾经被认为是反应性的,现在被认为是肿瘤性的。这些肿瘤可发生于全身,最常见于肺、肠系膜和网膜。患者通常表现为无痛性腹部肿块或肠梗阻。IMTs可能是多中心性的,局部复发率高,在罕见情况下可能发生转移。这些病变在组织学特征和细胞密度上表现出很大的变异性,并有明显的炎症浸润,主要是浆细胞和淋巴细胞,偶尔还有中性粒细胞和嗜酸性粒细胞。据报道,33%-67%的肿瘤存在间变性淋巴瘤激酶(ALK)重排和/或ALK1及p80免疫反应性。由于这些病变罕见,没有特定的影像学表现能将IMTs与其他肠系膜肿块区分开来。完整的手术切除是首选的治疗方法。局部复发率高,再次切除是局部复发的首选治疗方法。ALK阳性肿瘤对ALK抑制剂显示出良好的反应。本文介绍了关于IMTs的现有知识及对相关文献的全面综述。