Department of Pediatric Surgery and Pediatric Urology, Johann Wolfgang Goethe University, Frankfurt, Germany.
Division of Pediatric Stem Cell Transplantation and Immunology, Hospital for Children and Adolescents*, Johann Wolfgang Goethe University, Frankfurt, Germany.
Pediatr Blood Cancer. 2018 Apr;65(4). doi: 10.1002/pbc.26920. Epub 2017 Dec 29.
Inflammatory myofibroblastic tumor (IMT) and its subtype epithelioid inflammatory myofibroblastic sarcoma (EIMS) are rare soft-tissue tumors. As about 50% of IMT and 100% of EIMS contain activating rearrangements of the anaplastic lymphoma kinase (ALK) gene, targeted kinase inhibition of ALK by compounds such as crizotinib is a potential treatment option. We performed a literature review and analyzed a total of 30 patients with IMT/EIMS treated with crizotinib. A total of 12 patients achieved complete or partial remission. As preliminary data are promising, a prospective study evaluating crizotinib treatment in patients with unresectable/multifocal ALK IMT/EIMS is warranted.
炎性肌纤维母细胞瘤(IMT)及其上皮样炎性肌纤维母细胞瘤(EIMS)亚型是罕见的软组织肿瘤。由于约 50%的 IMT 和 100%的 EIMS 含有间变性淋巴瘤激酶(ALK)基因的激活重排,因此,克唑替尼等化合物针对 ALK 的靶向激酶抑制是一种潜在的治疗选择。我们进行了文献回顾,并分析了总共 30 例接受克唑替尼治疗的 IMT/EIMS 患者。共有 12 例患者获得完全或部分缓解。由于初步数据很有前景,因此有必要进行一项评估不可切除/多灶性 ALK IMT/EIMS 患者克唑替尼治疗的前瞻性研究。