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克唑替尼治疗间变性淋巴瘤激酶炎症性肌纤维母细胞瘤——当前经验与未来展望。

Crizotinib in ALK inflammatory myofibroblastic tumors-Current experience and future perspectives.

机构信息

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.

出版信息

Pediatr Blood Cancer. 2018 Apr;65(4). doi: 10.1002/pbc.26920. Epub 2017 Dec 29.

Abstract

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.

摘要

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。

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