新辅助克唑替尼治疗ALK基因重排的晚期炎性肌纤维母细胞瘤
Neoadjuvant crizotinib in advanced inflammatory myofibroblastic tumour with ALK gene rearrangement.
作者信息
Rafee Shereen, Elamin Yasir Y, Joyce Eimear, Toner Mary, Flavin Richard, McDermott Ronan, Sheehy Niall, Hennessy Bryan, O'Byrne Kenneth, Gleeson Noreen, Osman Nemer
机构信息
St. James's Hospital, Dublin - Ireland.
出版信息
Tumori. 2015 Apr 28;101(2):e35-9. doi: 10.5301/tj.5000245.
BACKGROUND
Inflammatory myofibroblastic tumours (IMTs) are rare sarcomas that were first described in the lung. They are composed of myofibroblastic mesenchymal spindle cells accompanied by an inflammatory infiltrate of plasma cells. Complete resection is the treatment of choice. There is currently no standard treatment for inoperable or recurrent disease. Expression of ALK protein triggered by ALK gene rearrangement at chromosome 2p23 has been found in 36%-60% of IMTs.
CASE REPORT
We report a rapid early response to crizotinib as neoadjuvant therapy, enabling surgical excision of a large ALK-translocated IMT, which resulted in complete disease clearance. To the best of our knowledge, this is the first case in the literature of a patient with IMT in whom crizotinib was used successfully in the neoadjuvant or curative setting.
背景
炎性肌纤维母细胞瘤（IMTs）是一种罕见的肉瘤，最初在肺部被描述。它们由肌纤维母细胞性间充质梭形细胞组成，并伴有浆细胞的炎性浸润。完整切除是首选治疗方法。目前对于无法手术切除或复发性疾病尚无标准治疗方案。在36%-60%的IMTs中发现了由2号染色体p23位点ALK基因重排触发的ALK蛋白表达。
病例报告
我们报告了一例对克唑替尼作为新辅助治疗的快速早期反应，使得一个大型ALK易位的IMT得以手术切除，从而实现了疾病的完全清除。据我们所知，这是文献中首例在新辅助或治愈性治疗中成功使用克唑替尼治疗IMT患者的病例。
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