Pediatric Pulmonology, UPMC Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania, USA.
Pediatric Hematology Oncology, UPMC Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania, USA.
Pediatr Pulmonol. 2022 Jan;57(1):330-332. doi: 10.1002/ppul.25742. Epub 2021 Oct 28.
Isolated endobronchial inflammatory myofibroblastic tumors (IMT) are rare, accounting for about 1% of primary endobronchial tumors in children. The mainstay of treatment for this tumor has been surgical resection. Recently, the identification of anaplastic lymphoma kinase (ALK) gene mutations in half of IMTs and promising results of treatment with ALK inhibitors in other ALK-positive tumors have opened the possibility of alternative approaches. We present a 4-year-old child with an ALK-positive endobronchial IMT, treated with endoscopic resection and neoadjuvant therapy with crizotinib, without evidence of tumor recurrence 2 years after the initial resection.
孤立性支气管内炎症性肌纤维母细胞瘤（IMT）较为罕见，约占儿童原发性支气管内肿瘤的 1%。该肿瘤的主要治疗方法是手术切除。最近，在一半的 IMT 中发现间变性淋巴瘤激酶（ALK）基因突变，以及在其他 ALK 阳性肿瘤中使用 ALK 抑制剂治疗取得良好效果，为治疗提供了新的可能。我们报告了一例 4 岁儿童患有 ALK 阳性支气管内 IMT，经内镜下切除和克唑替尼新辅助治疗，初始切除 2 年后未见肿瘤复发。
