Meng Xiangji, Zhang Lei, Wang Qi, Chen Jimin, Zhang Chunmei, Tao Rongjie, Wang Yong
Department of Neurosurgery, Shandong Cancer Hospital and Institute, Shandong First Medical University and Shandong Academy of Medical Sciences, Jinan, Shandong, 250117, People's Republic of China.
Department of Translational Medicine, Genecast Biotechnology Co., Ltd, Wuxi, Jiangsu, 214104, People's Republic of China.
Onco Targets Ther. 2022 Apr 1;15:313-321. doi: 10.2147/OTT.S343562. eCollection 2022.
Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal tumor that can develop in numerous organs, most commonly in the lungs and rarely in the brain. Here, we reported a 55-year-old patient with nasopharyngeal IMT and the recurrence in the skull base, slope and pterygoid sinus who underwent cranial base and slope tumor resection. Postoperative magnetic resonance imaging (MRI) and multiplex immunohistochemistry (mIHC) showed tumor recurrence and metastasis to the intracalvarium. While genetic testing revealed no significant related gene mutations, tertiary mutations in NSD1 and SOX9 genes were identified in the tumor tissues. The patient achieved partial remission after receiving 7 cycles of immunotherapy (toripalimab 240 mg for 1 cycle followed by 6 cycles of sintilimab 200 mg), and MRI examination indicated an almost complete remission of intracranial IMT after 16 cycles of immunotherapy. In summary, the novel class of immune-targeted agents may be effective in clinical management of rare intracranial IMT.
炎性肌成纤维细胞瘤（IMT）是一种罕见的间充质肿瘤，可发生于多个器官，最常见于肺部，很少发生于脑部。在此，我们报告了一例55岁的鼻咽IMT患者，其颅底、斜坡和翼腭窦复发，接受了颅底和斜坡肿瘤切除术。术后磁共振成像（MRI）和多重免疫组化（mIHC）显示肿瘤复发并转移至颅盖内。虽然基因检测未发现明显的相关基因突变，但在肿瘤组织中鉴定出NSD1和SOX9基因的三级突变。该患者在接受7个周期的免疫治疗（第1周期使用240mg托瑞帕利单抗，随后6个周期使用200mg信迪利单抗）后达到部分缓解，MRI检查显示在16个周期的免疫治疗后颅内IMT几乎完全缓解。总之，新型免疫靶向药物可能对罕见的颅内IMT临床治疗有效。
