Crizotinib in ALK-rearranged inflammatory myofibroblastic tumor.
Crizotinib in ALK-rearranged inflammatory myofibroblastic tumor.
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DOI：
10.1056/nejmoa1007056
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发表时间：
2010-10-28
期刊：
影响因子：
--
通讯作者：
Shapiro GI
中科院分区：
文献类型：
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作者：
Butrynski JE;D'Adamo DR;Hornick JL;Dal Cin P;Antonescu CR;Jhanwar SC;Ladanyi M;Capelletti M;Rodig SJ;Ramaiya N;Kwak EL;Clark JW;Wilner KD;Christensen JG;Jänne PA;Maki RG;Demetri GD;Shapiro GI
炎性肌纤维母细胞肿瘤（IMT）是一种独特的间叶性肿瘤，其特征是梭形细胞增殖和炎症浸润。大约一半的 IMT 携带 2p23 染色体上的间变性淋巴瘤激酶 (ALK) 位点重排，导致 ALK 表达异常。我们报告，一名患有 ALK 易位 IMT 的患者对 ALK 抑制剂克唑替尼（PF-02341066，辉瑞）有持续的部分反应，而另一名没有发生 ALK 易位的患者则没有观察到活性。这些结果支持 ALK 重排肿瘤对 ALK 介导的信号传导的依赖性，并为基因组鉴定的患有这种侵袭性软组织肿瘤的患者提出了治疗策略。
Inflammatory myofibroblastic tumor (IMT) is a distinctive mesenchymal neoplasm characterized by a spindle-cell proliferation with an inflammatory infiltrate. Approximately half of IMTs carry rearrangements of the anaplastic lymphoma kinase (ALK) locus on chromosome 2p23, causing aberrant ALK expression. We report a sustained partial response to the ALK inhibitor crizotinib (PF-02341066, Pfizer) in a patient with ALK-translocated IMT, as compared with no observed activity in another patient without the ALK translocation. These results support the dependence of ALK-rearranged tumors on ALK-mediated signaling and suggest a therapeutic strategy for genomically identified patients with the aggressive form of this soft-tissue tumor.