Chennouf Anas, Arslanian Elizabeth, Roberge David, Berthelet France, Bojanowski Michel, Bahary Jean-Paul, Masucci Laura, Belanger Karl, Florescu Marie, Wong Philip
CRCHUM, Université de Montréal.
Pathology, Centre Hospitalier de l'Université de Montréal (CHUM).
Cureus. 2017 Mar 2;9(3):e1068. doi: 10.7759/cureus.1068.
Inflammatory myofibroblastic tumors (IMT) of the central nervous system (CNS) are rare entities that have a predilection for local recurrences. Approximately half of the inflammatory myofibroblastic tumors contain translocations that result in the over-expression of the anaplastic lymphoma kinase (ALK) gene. We hereby present the case of a patient diagnosed with a left parieto-occipital IMT that recurred after multiple surgeries and radiotherapy. Immuno-histochemical examination of the tumor demonstrated ALK overexpression and the presence of an ALK rearrangement observed in lung cancers. The patient was subsequently started on an ALK inhibitor. A response evaluation criteria in solid tumors (RECIST) partial response was observed by the seventh month of ALK inhibition and the tumor remained in control for 14 months. The current case reiterates the activity of ALK inhibitors within the CNS and suggests that radiotherapy may potentiate the permeability of ALK inhibitors in CNS tumors addicted to ALK signalling.
中枢神经系统（CNS）的炎性肌纤维母细胞瘤（IMT）是一种罕见的疾病，容易局部复发。大约一半的炎性肌纤维母细胞瘤含有导致间变性淋巴瘤激酶（ALK）基因过度表达的易位。我们在此报告一例被诊断为左顶枕叶IMT的患者，该患者在多次手术和放疗后复发。对肿瘤进行免疫组织化学检查显示ALK过度表达，并且在肺癌中观察到ALK重排。该患者随后开始使用ALK抑制剂。在ALK抑制的第七个月观察到实体瘤疗效评价标准（RECIST）部分缓解，并且肿瘤在14个月内保持控制状态。目前的病例再次证明了ALK抑制剂在中枢神经系统中的活性，并表明放疗可能增强ALK抑制剂在依赖ALK信号传导的中枢神经系统肿瘤中的渗透性。
