Journal Article

Pulmonary Inflammatory Myofibroblastic Tumor Harboring EML4-ALK Fusion Gene

Akihiko Sokai, Makiko Enaka, Risa Sokai, Shoichi Mori, Shunsuke Mori, Masaharu Gunji, Masahiko Fujino and Masafumi Ito

in Japanese Journal of Clinical Oncology

Volume 44, issue 1, pages 93-96
Published in print January 2014 | ISSN: 0368-2811
Published online November 2013 | e-ISSN: 1465-3621 | DOI: https://dx.doi.org/10.1093/jjco/hyt173
Pulmonary Inflammatory Myofibroblastic Tumor Harboring EML4-ALK Fusion Gene

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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.

Keywords: inflammatory myofibroblastic tumor; EML4-ALK; malignant transformation

Journal Article.  1579 words.  Illustrated.

Subjects: Medical Oncology

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