Jiang Yi,Yang Chao.Clinical characteristics and treatments of epithelioid inflammatory myofibroblastic sarcoma in children[J].Journal of Clinical Pediatric Surgery,,():169-173.[doi:10.3760/cma.j.cn101785-202503095]
Clinical characteristics and treatments of epithelioid inflammatory myofibroblastic sarcoma in children
- Keywords:
- Epithelioid Inflammatory Myofibroblastic Sarcoma; Signs and Symptoms; Morphological and Microscopic Findings; Surgical Procedures; Operative; Treatment; Treatment Outcome; Child
- Abstract:
- Objective To explore the clinical characteristics,pathological features and treatments of epithelioid inflammatory myofibroblastic sarcoma(EIMS) in children. Methods From March 2013 to March 2022,the relevant clinical data were retrospectively reviewed for 7 children with diagnosed pathologically EIMS at Children’s Hospital of Chongqing Medical University.Demographic profiles,clinical manifestations,laboratory measurements,treatments and outcomes were recorded. Results There were 4 boys and 3 girls with a median age of 7 years and 3 months.The lesions were located in abdominal cavity (n=5),mediastinum (n=1) and pelvis (n=1). Anemia,fever and elevated inflammatory parameters as the major manifestations,ultrasound and CT revealed a solid mass.Pathological examinations indicated fusiform or epithelioid tumor cells often with an infiltration of inflammatory cells.Immunohistochemical stain was frequently positive for VIM,ALK,SMA and CK.Fluorescence in situ hybridization (FISH) detection revealed ALK gene rearrangement (n=5).The protocols included surgery plus chemotherapy(n=4),surgery alone (n=2) and chemotherapy plus surgery and targeted therapy (n=1).The follow-up period was (7-95) month.Except for 1 child relapsing and metastasizing during postoperative chemotherapy and lost to follow-ups after parental giving up treatment,the remainders attained disease-free survival (DFS). Conclusions EIMS is more common in abdominal cavity in children.Its clinical manifestations are diverse and non-specific.It is correlated closely with location and nature of lesions.Immunohistochemical stain and FISH detection help to confirm a diagnosis.Surgery is a major treatment along with chemotherapy or targeted therapy.The efficacy of comprehensive treatment is excellent.
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Memo
收稿日期:2025-3-25。
通讯作者:杨超,Email:400843@hospital.cqmu.edu.cn