Dong Youhong, Zahid Kashif Rafiq, Han Yidi, Hu Pengchao, Zhang Dongdong
Department of Oncology, Xiangyang No. 1 People's Hospital, Hubei University of Medicine, Xiangyang 441000, China.
Department of Hematology, Zhongnan Hospital Affiliated to Wuhan University, No. 169 Donghu Road, Wuhan 430071, China.
Children (Basel). 2022 Feb 24;9(3):307. doi: 10.3390/children9030307.
Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal tumor with intermediate malignancy that tends to affect children primarily. To date, no standardized therapies exist for the treatment of IMT. This study aimed to share experience from China Children's Medical Center for the explorative treatment of IMT.
Patients with newly diagnosed IMT between January 2013 and December 2018 were included. Patients were grouped according to surgical margins and Intergroup Rhabdomyosarcoma Study Group (IRSG) staging. The clinical characteristic, therapeutic schedules, treatment response and clinical outcome were described.
Six patients were enrolled in this study, including two boys and four girls, with a median age of 57 months (range 10-148 months). Among them, five patients were anaplastic lymphoma kinase positive. Four patients achieved complete remission and two patients attained partial remission after treatment with this protocol. All patients were alive after a median follow-up of 4 years (range 3-7 years). The most common treatment-related adverse reaction was myelosuppression.
In this study, we demonstrated that IMT has a good prognosis and the treatment selected according to risk stratification was effective and feasible.
炎性肌纤维母细胞瘤（IMT）是一种罕见的间叶性肿瘤，具有中度恶性，主要影响儿童。迄今为止，尚无治疗IMT的标准化疗法。本研究旨在分享中国儿童医学中心对IMT进行探索性治疗的经验。
纳入2013年1月至2018年12月期间新诊断的IMT患者。根据手术切缘和横纹肌肉瘤协作组（IRSG）分期对患者进行分组。描述了临床特征、治疗方案、治疗反应和临床结果。
本研究共纳入6例患者，其中2例男性，4例女性，中位年龄57个月（范围10 - 148个月）。其中5例患者间变性淋巴瘤激酶阳性。采用该方案治疗后，4例患者达到完全缓解，2例患者达到部分缓解。中位随访4年（范围3 - 7年）后，所有患者均存活。最常见的治疗相关不良反应是骨髓抑制。
在本研究中，我们证明IMT预后良好，根据风险分层选择的治疗方法有效且可行。
