Clinical features, treatment strategies, and prognosis of epithelioid inflammatory myofibroblastic sarcoma in children: a multicenter experience

作者全名：Cheng, Haiyan; Lin, Yu; Zhu, Jin; Qin, Hong; Yang, Wei; Chang, Xiaofeng; Feng, Jun; Yang, Shen; Fu, Libing; Zhang, Nan; Shi, Kui; Sun, Jian; Su, Yan; Jin, Mei; Wang, Shan; Wang, Huanmin

作者地址：[Cheng, Haiyan; Lin, Yu; Qin, Hong; Yang, Wei; Chang, Xiaofeng; Feng, Jun; Yang, Shen; Wang, Huanmin] Capital Med Univ, Beijing Childrens Hosp, Natl Ctr Childrens Hlth, Dept Oncol Surg, 56 Nanlishi Lu, Beijing 100045, Peoples R China; [Zhu, Jin] Chongqing Med Univ, Childrens Hosp, Dept Pathol, Chongqing, Peoples R China; [Zhu, Jin] Chongqing Med Univ, Coll Basic Med, Dept Pathol, Chongqing, Peoples R China; [Fu, Libing; Zhang, Nan; Wang, Shan] Capital Med Univ, Beijing Childrens Hosp, Natl Ctr Childrens Hlth, Dept Pathol, Beijing, Peoples R China; [Shi, Kui; Su, Yan] Baoding Childrens Hosp, Dept Oncol Surg, Baoding, Peoples R China; [Sun, Jian; Wang, Shan] Childrens Hosp, Chongqing Med Univ, Dept Oncol Surg, 136 Zhongshan 2nd Rd, Chongqing 400014, Peoples R China; [Jin, Mei] Capital Med Univ, Beijing Childrens Hosp, Natl Ctr Childrens Hlth, Dept Med Oncol, Beijing, Peoples R China; [Wang, Huanmin] Capital Med Univ, Beijing Childrens Hosp, Natl Ctr Childrens Hlth, MOE Key Lab Major Dis Children, 56 Nanlishi Lu Xicheng District, Beijing 100045, Peoples R China

通信作者：Wang, HM (通讯作者)，Capital Med Univ, Beijing Childrens Hosp, Natl Ctr Childrens Hlth, Dept Oncol Surg, 56 Nanlishi Lu, Beijing 100045, Peoples R China.; Wang, S (通讯作者)，Childrens Hosp, Chongqing Med Univ, Dept Oncol Surg, 136 Zhongshan 2nd Rd, Chongqing 400014, Peoples R China.; Wang, HM (通讯作者)，Capital Med Univ, Beijing Childrens Hosp, Natl Ctr Childrens Hlth, MOE Key Lab Major Dis Children, 56 Nanlishi Lu Xicheng District, Beijing 100045, Peoples R China.

来源：TRANSLATIONAL PEDIATRICS

ESI学科分类：　

WOS号：WOS:001223633600009

JCR分区：Q2

影响因子：1.5

年份：2024

卷号：13

期号：2

开始页：　

结束页：　

文献类型：Article

关键词：Epithelioid inflammatory myofibroblast sarcoma (EIMS); children; clinical features; precise treatment; prognosis

摘要：Background: Inflammatory myofibroblastic tumors (IMTs) are a spectrum of tumors that range in morphology and biological behavior from benign, intermediate, to apparently malignant and epithelioid inflammatory myofibroblastic sarcoma (EIMS) is one of the malignant subtypes. This study tried to provide experience and new ideas for treating this rare disease. Methods: This study retrospectively analyzed and followed up 12 children with EIMS admitted to Beijing Children's Hospital, Baoding Children's Hospital, and Children's Hospital of Chongqing Medical University from August 2016 to May 2022. Results: Of the 12 children, 7 were male and 5 were female, with a median age of 74.50 [interquartile range (IQR), 61.50-90.00] months. Of these patients, eight had a single lesion and four had multiple lesions. The maximum diameter of the single tumor foci was 19.30 cm, the full meridian of the multiple tumor foci target lesions was 32.67 cm, and the median maximum tumor size was 11.99 (IQR, 7.80-15.70) cm. The site of disease was the abdominopelvic cavity in eight cases, the thoracic cavity in two cases, the maxillofacial region in one case, and the larynx in one case. The clinical manifestations were predominantly elevated body temperature (n=8). There was one case of ROS1 fusion mutation and nine cases of ALK fusion mutation. Of the 12 children, 6 were biopsied at the initial diagnosis and 6 were surgically treated. Follow-up treatment included preoperative neoadjuvant chemotherapy (n=4), peritoneal thermal perfusion therapy (n=2), targeted therapy (n=3), postoperative chemotherapy (n=5), and radiotherapy (n=3). The follow-up time was 14.50 (IQR, 10.50-31.50) months, with eight cases of tumor -free survival, two cases of death, and two cases of loss of follow-up. Conclusions: EIMS in children is extremely rare and clinically aggressive. The clinical presentation is nonspecific, and the initial diagnosis of the tumor is often large. Mutations in the ALK gene are common in EIMS. Surgery is the mainstay of EIMS treatment, and patients benefit from a multidisciplinary combination that includes targeted therapies, with long-term prognosis remaining subject to ongoing follow-up.

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