Dana-Farber Cancer Institute, Boston, MA 02115, USA.
N Engl J Med. 2010 Oct 28;363(18):1727-33. doi: 10.1056/NEJMoa1007056.
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. (Funded by Pfizer and others; ClinicalTrials.gov number, NCT00585195.).
炎性肌纤维母细胞瘤(IMT)是一种独特的间叶性肿瘤,其特征为梭形细胞增生伴炎症浸润。大约一半的 IMT 存在染色体 2p23 上的间变性淋巴瘤激酶(ALK)基因重排,导致异常的 ALK 表达。我们报告了一例 ALK 易位 IMT 患者对 ALK 抑制剂克唑替尼(PF-02341066,辉瑞)的持续部分缓解,而另一例无 ALK 易位的患者则未观察到活性。这些结果支持 ALK 重排肿瘤对 ALK 介导的信号的依赖性,并为这种软组织肿瘤侵袭性形式的基因组鉴定患者提供了一种治疗策略。(由辉瑞等资助;ClinicalTrials.gov 编号,NCT00585195.)。