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

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...

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Veröffentlicht in:Journal of pediatric hematology/oncology 2016-05, Vol.38 (4), p.308-311
Hauptverfasser: Gaudichon, Jérémie, Jeanne-Pasquier, Corinne, Deparis, Marianna, Veyssière, Alexis, Heyndrickx, Maxime, Minckes, Odile, Orbach, Daniel
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Sprache:eng
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Zusammenfassung: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.
ISSN:1077-4114
1536-3678
DOI:10.1097/mph.0000000000000498