AUTHOR=Li Mengmeng , Xing Ruyue , Huang Jiuyan , Shi Chao , Wei Chunhua , Wang Huijuan 

TITLE=Case report: Epithelioid inflammatory myofibroblastic sarcoma treated with an ALK TKI ensartinib

JOURNAL=Frontiers in Oncology

VOLUME=13

YEAR=2023

URL=https://www.frontiersin.org/journals/oncology/articles/10.3389/fonc.2023.1084456

DOI=10.3389/fonc.2023.1084456

ISSN=2234-943X

ABSTRACT=<p>Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is an aggressive variant of inflammatory myofibroblastic tumor (IMT) and has a poor prognosis. EIMS is characterized by epithelioid morphology, neutrophilic infiltrate and specific fusion partners of anaplastic lymphoma kinase (<italic>ALK</italic>). Despite no standard therapy for EIMS, ALK tyrosine kinase inhibitors (TKIs) are recommended for these tumors. The present case describes an abdominal mass that presented in a 31-year-old male. The patient suffered from recurrence and multiple metastases 2 months after surgery. Ensartinib was administered and <italic>RANBP2-ALK</italic> fusion was detected. A partial response has been observed for 4 months and there has been no recurrence. This study provided a successful case with sustained response of targeted therapy.</p>