Li Jinzhou, Su Haixing, Zhang Sheng, Chen Xianyun, Hou Chongzhi, Cheng Tao
Department of General Surgery, Xi'an Children's Hospital/Children's Hospital Affiliated to Xi'an Jiaotong University, Xi'an, China.
Front Oncol. 2024 Dec 5;14:1417918. doi: 10.3389/fonc.2024.1417918. eCollection 2024.
Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a distinct subtype of inflammatory myofibroblastoma tumor (IMT) that is recognized as a rare malignant tumor characterized by anaplastic lymphoma kinase (ALK) positivity, significant aggressiveness, treatment challenges, and a poor prognosis. We report on 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 the pathology results following a biopsy confirmed the diagnosis of EIMS. The patient underwent radical tumor resection, and genetic testing identified the presence of the - 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.
上皮样炎性肌成纤维细胞肉瘤（EIMS）是炎性肌成纤维细胞瘤（IMT）的一种独特亚型，被认为是一种罕见的恶性肿瘤，其特征为间变性淋巴瘤激酶（ALK）阳性、具有显著侵袭性、治疗具有挑战性且预后较差。我们报告了一例8岁男孩，其表现为腹痛和呕吐。腹部计算机断层扫描（CT）显示有一个大肿瘤，活检后的病理结果确诊为EIMS。该患者接受了根治性肿瘤切除术，基因检测发现存在 - 融合。据我们所知，这是文献中记载的最大的小儿腹部EIMS病例。目前，EIMS尚无标准治疗方法；然而，现有研究主张在其治疗中使用ALK酪氨酸激酶抑制剂（TKIs）。据报道，该病例在接受克唑替尼治疗后病情缓解，从而有助于对EIMS的特定病理学的理解，并促进准确诊断和靶向治疗。
