Li Mengmeng, Xing Ruyue, Huang Jiuyan, Shi Chao, Wei Chunhua, Wang Huijuan
Department of Medical Oncology, The Affiliated Cancer Hospital of Zhengzhou University and Henan Caner Hospital, Zhengzhou, China.
Department of Molecular Pathology, The Affiliated Cancer Hospital of Zhengzhou University and Henan Cancer Hospital, Zhengzhou, China.
Front Oncol. 2023 Mar 22;13:1084456. doi: 10.3389/fonc.2023.1084456. eCollection 2023.
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 (). 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 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.
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)的一种侵袭性变体,预后较差。EIMS的特征是上皮样形态、中性粒细胞浸润以及间变性淋巴瘤激酶(ALK)的特定融合伴侣。尽管EIMS没有标准治疗方法,但推荐对这些肿瘤使用ALK酪氨酸激酶抑制剂(TKIs)。本病例描述了一名31岁男性出现的腹部肿块。该患者术后2个月出现复发和多处转移。给予恩沙替尼治疗并检测到ALK融合。观察到部分缓解已达4个月且未再复发。本研究提供了一个靶向治疗持续有效的成功病例。