title: Pulmonary inflammatory myofibroblastic tumor harboring EML4-ALK fusion gene.
abstract: Inflammatory myofibroblastic tumor is a rare tumor deriving from mesenchymal tissue. Approximately 50% of inflammatory myofibroblastic tumors harbor an anaplastic lymphoma kinase fusion gene. Pulmonary inflammatory myofibroblastic tumors harboring tropomyosin3-anaplastic lymphoma kinase or protein tyrosine phosphatase receptor-type F polypeptide-interacting protein-binding protein 1-anaplastic lymphoma kinase have been reported previously. However, it has not been reported that inflammatory myofibroblastic tumors harbor echinoderm microtubule-associated protein-like 4-anaplastic lymphoma kinase fusion gene which is considered to be very specific to lung cancers. A few tumors harboring echinoderm microtubule-associated protein-like 4-anaplastic lymphoma kinase fusion gene other than lung cancers have been reported and the tumors were all carcinomas. A 67-year-old man had been followed up for a benign tumor for approximately 3 years before the tumor demonstrated malignant transformation. Lobectomy and autopsy revealed that an inflammatory myofibroblastic tumor harboring echinoderm microtubule-associated protein-like 4-anaplastic lymphoma kinase fusion gene had transformed into an undifferentiated sarcoma. This case suggests that echinoderm microtubule-associated protein-like 4-anaplastic lymphoma kinase fusion is an oncogenic event in not only carcinomas but also sarcomas originating from stromal cells.
authors.affiliation: *Department of Pathology, Japanese Red Cross Nagoya Daiichi Hospital, 3-35 Michishita-cho, Nakamura-ku, Nagoya-city, Aichi 453-8511, Japan. itom@nagoya-1st.jrc.or.jp.
authors.forename: Akihiko
authors.initials: A
authors.lastname: Sokai
authors.forename: Makiko
authors.initials: M
authors.lastname: Enaka
authors.forename: Risa
authors.initials: R
authors.lastname: Sokai
authors.forename: Shoichi
authors.initials: S
authors.lastname: Mori
authors.forename: Shunsuke
authors.initials: S
authors.lastname: Mori
authors.forename: Masaharu
authors.initials: M
authors.lastname: Gunji
authors.forename: Masahiko
authors.initials: M
authors.lastname: Fujino
authors.forename: Masafumi
authors.initials: M
authors.lastname: Ito
mesh_terms: D000368:Aged; D001344:Autopsy; D017809:Fatal Outcome; D006801:Humans; D007249:Inflammation; D008175:Lung Neoplasms / Q000235:genetics* / Q000378:metabolism / Q000473:pathology*; D008297:Male; D009379:Neoplasms, Muscle Tissue / Q000235:genetics* / Q000378:metabolism / Q000473:pathology*; D015514:Oncogene Proteins, Fusion / Q000235:genetics*
chemical_list: C522504:EML4-ALK fusion protein, human; D015514:Oncogene Proteins, Fusion
keywords: EML4-ALK; inflammatory myofibroblastic tumor; malignant transformation