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CASE REPORT article

Front. Oncol.
Sec. Pediatric Oncology
Volume 14 - 2024 | doi: 10.3389/fonc.2024.1417918
This article is part of the Research Topic Multi-omics Approaches to Identify Immune Profiles and Therapeutic Targets for Rare Cancers View all articles

Rare giant epithelioid inflammatory myofibroblastic sarcoma of the abdominal cavity in a child: a case report and review of the literature

Provisionally accepted
Jinzhou Li Jinzhou Li Haixing Su Haixing Su *Sheng Zhang Sheng Zhang *Xianyun Chen Xianyun Chen *Chongzhi Hou Chongzhi Hou *Tao Cheng Tao Cheng *
  • Other, Xi'an, China

The final, formatted version of the article will be published soon.

    Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a distinct subtype of inflammatory myofibroblastoma tumor (IMT), recognized as a rare malignant tumor characterized by anaplastic lymphoma kinase (ALK) positivity, significant aggressiveness, treatment challenges, and a poor prognosis. We report the case of an 8-year-old boy presenting with abdominal pain and vomiting. Computed tomography (CT) of the abdomen revealed a large tumor, and pathology results following a biopsy confirmed the diagnosis of EIMS. The child underwent radical tumor resection, and genetic testing identified the presence of the RANBP2-ALK fusion. To our knowledge, this represents the largest pediatric case of abdominal EIMS documented in the literature. Currently, there is no standard therapy for EIMS; however, existing studies advocate for the use of ALK tyrosine kinase inhibitors (TKIs) in its treatment. This case was reported to be in remission following treatment with crizotinib, thereby contributing to the understanding of the specific pathology of EIMS and facilitating accurate diagnosis and targeted therapy.

    Keywords: Childhood tumor, Epithelioid inflammatory myofibroblastic sarcoma, Anaplastic lymphoma kinase, crizotinib, targeted therapy

    Received: 15 Jul 2024; Accepted: 13 Nov 2024.

    Copyright: © 2024 Li, Su, Zhang, Chen, Hou and Cheng. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence:
    Haixing Su, Other, Xi'an, China
    Sheng Zhang, Other, Xi'an, China
    Xianyun Chen, Other, Xi'an, China
    Chongzhi Hou, Other, Xi'an, China
    Tao Cheng, Other, Xi'an, China

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