Li XiaoQing, Zheng JingFan, Li XinYi, Chen YuYu, Liu Kang, Li FangChao, Lu Zhong
School of Clinical Medicine, Weifang Medical University, Weifang, Shandong, China.
Department of Oncology, Affiliated Hospital Of Weifang Medical University, Weifang, Shandong, China.
Front Oncol. 2023 Oct 5;13:1252221. doi: 10.3389/fonc.2023.1252221. eCollection 2023.
Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a highly aggressive malignant subtype of inflammatory myofibroblastoma (IMT) associated with poor prognosis. IMT can occur in various parts of the body, most frequently in the lungs, followed by the mesentery, omentum, retroperitoneum, and pelvis, among other areas; however, it is exceptionally rare in the stomach. Anaplastic lymphoma kinase (ALK) is a critical driver of lung cancer development and is currently the "gold standard" target for non-small cell lung cancer treatment. However, there are few reports on the use of ALK inhibitors for EIMS, necessitating further investigation. A male patient with postoperative inflammatory myofibroblastic sarcoma of the stomach received postoperative chemotherapy and had a stable outcome. However, a repeat CT scan performed 11 months later revealed disease progression. The patient later underwent immunohistochemistry testing that indicated ALK positivity, and next-generation sequencing revealed STRN-ALK fusion. Ensartinib 225 mg qd was administered as recommended, and the patient experienced only mild pruritus and no adverse effects such as rash. Eight months after CT follow-up, the patient's subseptal soft tissue nodules had decreased, and the outcome was assessed as a partial response. The findings of this case report introduce a novel strategy for treating ALK-positive EIMS that utilizes ensartinib, a drug with previously demonstrated success in the treatment of ALK-positive cancer.
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)的一种高度侵袭性恶性亚型,预后较差。IMT可发生于身体的各个部位,最常见于肺部,其次是肠系膜、大网膜、腹膜后和骨盆等部位;然而,在胃中极为罕见。间变性淋巴瘤激酶(ALK)是肺癌发展的关键驱动因素,目前是非小细胞肺癌治疗的“金标准”靶点。然而,关于使用ALK抑制剂治疗EIMS的报道很少,需要进一步研究。一名胃炎性肌成纤维细胞肉瘤术后男性患者接受了术后化疗,病情稳定。然而,11个月后进行的重复CT扫描显示疾病进展。该患者随后接受免疫组化检测,结果显示ALK阳性,下一代测序显示存在STRN-ALK融合。按照推荐剂量给予恩沙替尼225mg每日一次,患者仅出现轻度瘙痒,未出现皮疹等不良反应。CT随访8个月后,患者的隔下软组织结节减少,评估结果为部分缓解。本病例报告的结果介绍了一种治疗ALK阳性EIMS的新策略,即使用恩沙替尼,该药物此前已在治疗ALK阳性癌症方面取得成功。