title: Drastic initial response and subsequent response to two ALK inhibitors in a patient with a highly aggressive ALK-rearranged inflammatory myofibroblastic tumor arising in the pleural cavity.
abstract: A 57-year-old male current smoker was diagnosed with an aggressive variant of ALK-rearranged inflammatory myofibroblastic tumor (IMT) arising in the pleural cavity. First line treatment with ASP3026 was initiated at a dose of 125mg once daily. A follow-up CT scan revealed drastic regression of the pleural lesion. After disease progression with ASP3026 treatment, LDK378 (ceritinib) was initiated at a dose of 750mg once daily. A follow-up CT scan revealed a second drastic regression of the pleural lesion. Furthermore, it is noteworthy that this case represents the use of serum hyaluronan levels to assist in monitoring of treatment efficacy in an IMT. Herein, we present the first case of a patient with a highly aggressive ALK-rearranged IMT arising in the pleural cavity, who showed both initial and subsequent drastic response to two ALK inhibitors while being monitored for serum hyaluronan.
authors: Division of Thoracic Oncology, Shizuoka Cancer Center, Shizuoka, Japan. Electronic address: a.ono@scchr.jp.
authors: Akira
authors: A
authors: Ono
authors: Division of Thoracic Oncology, Shizuoka Cancer Center, Shizuoka, Japan.
authors: Haruyasu
authors: H
authors: Murakami
authors: Drug Discovery and Development Division, Shizuoka Cancer Center Research Institute, Shizuoka, Japan.
authors: Masakuni
authors: M
authors: Serizawa
authors: Division of Thoracic Oncology, Shizuoka Cancer Center, Shizuoka, Japan.
authors: Kazushige
authors: K
authors: Wakuda
authors: Division of Thoracic Oncology, Shizuoka Cancer Center, Shizuoka, Japan.
authors: Hirotsugu
authors: H
authors: Kenmotsu
authors: Division of Thoracic Oncology, Shizuoka Cancer Center, Shizuoka, Japan.
authors: Tateaki
authors: T
authors: Naito
authors: Division of Thoracic Oncology, Shizuoka Cancer Center, Shizuoka, Japan.
authors: Tetsuhiko
authors: T
authors: Taira
authors: Drug Discovery and Development Division, Shizuoka Cancer Center Research Institute, Shizuoka, Japan; Third Department of Internal Medicine, Wakayama Medical University, Wakayama, Japan.
authors: Yasuhiro
authors: Y
authors: Koh
authors: Division of Thoracic Surgery, Shizuoka Cancer Center, Shizuoka, Japan.
authors: Yasuhisa
authors: Y
authors: Ohde
authors: Division of Diagnostic Pathology, Shizuoka Cancer Center, Shizuoka, Japan.
authors: Takashi
authors: T
authors: Nakajima
authors: Division of Diagnostic Radiology, Shizuoka Cancer Center, Shizuoka, Japan.
authors: Masahiro
authors: M
authors: Endo
authors: Division of Thoracic Oncology, Shizuoka Cancer Center, Shizuoka, Japan.
authors: Toshiaki
authors: T
authors: Takahashi
mesh_terms: D000077548:Anaplastic Lymphoma Kinase; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001706:Biopsy; D017809:Fatal Outcome; D015321:Gene Rearrangement; D006801:Humans; D008297:Male; D008875:Middle Aged; D009379:Neoplasms, Muscle Tissue / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics*; D035422:Pleural Cavity / Q000000981:diagnostic imaging / Q000473:pathology*; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use*; D020794:Receptor Protein-Tyrosine Kinases / Q000235:genetics*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D047428:Protein Kinase Inhibitors; C000626173:ALK protein, human; D000077548:Anaplastic Lymphoma Kinase; D020794:Receptor Protein-Tyrosine Kinases
keywords: ALK; ASP3026; Ceritinib; Hyaluronan; Inflammatory myofibroblastic tumor; NF1