Fujiki Toshihiro, Sakai Yuta, Ikawa Yasuhiro, Takenaka Mika, Noguchi Kazuhiro, Kuroda Rie, Abe Takatoshi, Nomura Kozo, Sakai Seisho, Wada Taizo
Department of Pediatrics, Kanazawa University, Kanazawa, Ishikawa, Japan.
Department of Pediatric Surgery, Kanazawa University, Kanazawa, Ishikawa, Japan.
Pediatr Blood Cancer. 2023 Apr;70(4):e30172. doi: 10.1002/pbc.30172. Epub 2023 Jan 12.
An inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm characterized by the proliferation of myofibroblasts and inflammatory cell infiltration. Although radical resection is the only established treatment strategy for IMT, it can cause functional disorders when vital organs are affected. We describe a case of pediatric IMT of the bladder with FN1-ALK (fibronectin 1-anaplastic lymphoma kinase) fusion. Radical resection might lead to urinary disturbance due to the large tumor size at diagnosis. However, the tumor was successfully treated with alectinib, a second-generation ALK inhibitor, followed by transurethral resection of the bladder tumor without any complications.
炎性肌纤维母细胞瘤（IMT）是一种间叶性肿瘤，其特征为肌成纤维细胞增殖和炎性细胞浸润。尽管根治性切除是IMT唯一已确立的治疗策略，但当重要器官受到影响时，可能会导致功能障碍。我们描述了一例患有FN1-ALK（纤连蛋白1-间变性淋巴瘤激酶）融合的小儿膀胱IMT病例。由于诊断时肿瘤体积较大，根治性切除可能会导致尿路紊乱。然而，该肿瘤通过第二代ALK抑制剂阿来替尼成功治疗，随后经尿道切除膀胱肿瘤，未出现任何并发症。
