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一名青少年女性转移性ALK重排炎性肌纤维母细胞瘤对克唑替尼的完全且重复缓解

Complete and Repeated Response of a Metastatic ALK-rearranged Inflammatory Myofibroblastic Tumor to Crizotinib in a Teenage Girl.

作者信息

Gaudichon Jérémie, Jeanne-Pasquier Corinne, Deparis Marianna, Veyssière Alexis, Heyndrickx Maxime, Minckes Odile, Orbach Daniel

机构信息

*Oncology-Hematology Unit Departments of §Maxillofacial and Plastic Surgery ∥Thoracic Surgery, Caen University Hospital ‡Department of Pathology, Centre François Baclesse, Caen †Pediatric, Adolescent and Young Adult Oncology Department, Institut Curie, Paris, France.

出版信息

J Pediatr Hematol Oncol. 2016 May;38(4):308-11. doi: 10.1097/MPH.0000000000000498.

Abstract

Inflammatory myofibroblastic tumors (IMT) are rare tumors in children and young adults, considered by the World Health Organization to be intermediate malignancies and rarely metastasizing, with the presence of an anaplastic lymphoma kinase rearrangement in about 50% of the cases. We report the case of a teenager who presented with a metastatic aggressive IMT that was life-threatening despite multiple treatments, and which responded repeatedly to anaplastic lymphoma kinase-targeted crizotinib therapy. Crizotinib induced drastic primary tumor regression, which was sufficient to allow surgical resection and to control distant disease. This case shows that crizotinib is a promising therapy in IMT, even in adolescents and young adults.

摘要

炎性肌纤维母细胞瘤(IMT)在儿童和年轻人中是罕见肿瘤,被世界卫生组织视为中间型恶性肿瘤,很少发生转移,约50%的病例存在间变性淋巴瘤激酶重排。我们报告了一例青少年患者,其患有转移性侵袭性IMT,尽管接受了多种治疗仍危及生命,但对间变性淋巴瘤激酶靶向药物克唑替尼治疗反复有反应。克唑替尼使原发性肿瘤显著消退,足以进行手术切除并控制远处疾病。该病例表明,克唑替尼在IMT治疗中是一种有前景的疗法,即使在青少年和年轻成人中也是如此。

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