title: A case report of epithelioid inflammatory myofibroblastic sarcoma with RANBP2-ALK fusion gene treated with the ALK inhibitor, crizotinib.
abstract: Epithelioid inflammatory myofibroblastic sarcoma is a variant of inflammatory myofibroblastic tumor with aggressive clinical course associated with RANBP2-ALK fusion. The present report describes a case of a 22-year-old Japanese man with a pelvic mesenchymal neoplasm. The feature of the neoplasms, including epithelioid morphology, anaplastic lymphoma kinase staining on the nuclear membrane, and results from the reverse transcriptase-polymerase chain reaction, led to diagnosis of epithelioid inflammatory myofibroblastic sarcoma with RANBP2-ALK fusion. Despite two surgical excision procedures, local recurrence rapidly occurred, and the tumor developed resistance to conventional chemotherapy with doxorubicin. Subsequent administration of crizotinib, an oral anaplastic lymphoma kinase inhibitor, resulted in tumor shrinkage. Distinguishing epithelioid inflammatory myofibroblastic sarcoma from conventional inflammatory myofibroblastic tumor is important, and crizotinib is a promising treatment for this aggressive tumor.
authors.affiliation: Department of Clinical Oncology, Osaka City General Hospital, Osaka.
authors.forename: Shiro
authors.initials: S
authors.lastname: Kimbara
authors.affiliation: Department of Clinical Oncology, Osaka City General Hospital, Osaka.
authors.forename: Koji
authors.initials: K
authors.lastname: Takeda
authors.affiliation: Department of Pathology, Osaka City General Hospital, Osaka.
authors.forename: Hiroko
authors.initials: H
authors.lastname: Fukushima
authors.affiliation: Department of Gastroenterological Surgery, Osaka City General Hospital, Osaka, Japan.
authors.forename: Toru
authors.initials: T
authors.lastname: Inoue
authors.affiliation: Department of Clinical Oncology, Osaka City General Hospital, Osaka.
authors.forename: Hideaki
authors.initials: H
authors.lastname: Okada
authors.affiliation: Department of Clinical Oncology, Osaka City General Hospital, Osaka.
authors.forename: Yumi
authors.initials: Y
authors.lastname: Shibata
authors.affiliation: Department of Clinical Oncology, Osaka City General Hospital, Osaka.
authors.forename: Utae
authors.initials: U
authors.lastname: Katsushima
authors.affiliation: Department of Clinical Oncology, Osaka City General Hospital, Osaka.
authors.forename: Asuka
authors.initials: A
authors.lastname: Tsuya
authors.affiliation: Department of Clinical Oncology, Osaka City General Hospital, Osaka.
authors.forename: Shinya
authors.initials: S
authors.lastname: Tokunaga
authors.affiliation: Department of Clinical Oncology, Osaka City General Hospital, Osaka.
authors.forename: Haruko
authors.initials: H
authors.lastname: Daga
authors.affiliation: Department of Pathology, Osaka City General Hospital, Osaka.
authors.forename: Takahiro
authors.initials: T
authors.lastname: Okuno
authors.affiliation: Department of Pathology, Osaka City General Hospital, Osaka.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Inoue
mesh_terms: D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D000077547:Crizotinib; D006801:Humans; D007249:Inflammation; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000188:drug therapy* / Q000235:genetics; D009379:Neoplasms, Muscle Tissue / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology; D015514:Oncogene Proteins, Fusion / Q000037:antagonists & inhibitors* / Q000235:genetics*; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use*; D011720:Pyrazoles / Q000627:therapeutic use*; D011725:Pyridines / Q000627:therapeutic use*; D020133:Reverse Transcriptase Polymerase Chain Reaction; D012509:Sarcoma / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D015514:Oncogene Proteins, Fusion; D047428:Protein Kinase Inhibitors; D011720:Pyrazoles; D011725:Pyridines; C000591371:RANBP2-ALK fusion protein, human; D000077547:Crizotinib
keywords: RANBP2-ALK; crizotinib; epithelioid inflammatory myofibroblastic sarcoma