儿童和青少年炎性肌纤维母细胞瘤临床病理特征及生存分析

Analysis of outcomes and clinicopathology of children and adolescents with inflammatory myofibroblastoma

目的探讨儿童及青少年炎性肌纤维母细胞瘤(IMTs)的临床病理特征、诊断和治疗。方法回顾性分析2012年8月—2020年6月于中山大学肿瘤防治中心儿童肿瘤科收治的病理确诊为IMT患儿(≤18岁)的临床资料。结果资料完整、长期随访的有13例患儿,其中男性9例,女性4例,中位年龄7.2岁。原发部位:腹腔和盆腔7例,大腿2例,头面部3例,肺部1例。治疗:13例中,采用一线手术治疗为12例,采用一线化疗加靶向药治疗1例。疗效:生存11例,死亡2例。12例直接手术者9例术后无复发,3例复发。肿瘤免疫组化间变性淋巴瘤激酶(ALK)阳性的患儿9例(9/13)。3例服用ALK抑制剂治疗,2例生存,1例死亡。结论IMT是好发于腹部、盆腔的肿瘤。根治性手术是IMT首选的治疗方式。上皮样炎性肌纤维母细胞肉瘤侵袭性高。ALK抑制剂可作为无法手术、有ALK易位患者的治疗选择。

Objective To investigate the clinicopathological characteristics,diagnosis and treatment of inflammatory myofibroblastic tumors(IMTs)in children and adolescents.Methods Retrospectively analyzed the clinical data of pathologically diagnosed children with IMT(≤18 years old)who were admitted to the Pediatric Oncology Department of Sun Yat-sen University Cancer Center from August 2012 through June 2020.Results There were 13 children with long-term follow-up,including 9 males and 4 females,with a median age of 7.2 years.Primary site:7 cases of abdominal cavity and pelvis,2 cases of thighs,3 cases of head and face,1 case of lungs.Treatment:12 patients were treated with first-line surgery,1 patient was administered with first-line chemotherapy and targeted drugs.Efficacy:11 cases survived and 2 died.There were 12 cases of operation,9 cases had no recurrence;3 cases had recurrence.9 cases(9 out of 13)were positive for tumor immunohistochemical anaplastic lymphoma kinase(ALK).3 cases were treated with ALK inhibitors,2 cases survived and 1 case died.Conclusions IMT is a tumor that usually occurs in the abdomen and pelvis.Radical surgery is the preferred treatment for IMT,and epithelioid inflammatory myofibroblastic sarcoma is highly aggressive.ALK inhibitors can be used as a treatment option for inoperable patients with ALK translocations.