title: Epithelioid inflammatory myofibroblastic sarcoma treated with ALK inhibitor: a case report and review of literature.
abstract: Epithelioid inflammatory myofibroblastic sarcoma is extremely rare and belongs to a variant of inflammatory myofibrobalstic tumor with aggressive clinical course. We describe a case of a 22 years old man presented with an abdominal huge tumor. Microscopically, the neoplasm cells were rounded and epithelioid in shape. Abundant interstitial edema and less myxoid stroma were also present together with an inflammatory infiltrate. Fluorescence in situ hybridization revealed that ALK gene presented mutation. After surgery the patient received chemotherapy with an anaplastic lymphoma kinase (ALK) inhibitor, crizotinib. The patient continues to be alive with disease for 16 months and has no recurrence. Although EIMS has a poor prognosis, this is the few successful case with sustained response of targeted therapy.
authors.affiliation: Department of Pathology, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University Zhengzhou, China.
authors.forename: Qiuyu
authors.initials: Q
authors.lastname: Liu
authors.affiliation: Department of Pathology, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University Zhengzhou, China.
authors.forename: Yunzhen
authors.initials: Y
authors.lastname: Kan
authors.affiliation: Department of Pathology, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University Zhengzhou, China.
authors.forename: Yuewu
authors.initials: Y
authors.lastname: Zhao
authors.affiliation: Department of Pathology, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University Zhengzhou, China.
authors.forename: Hui
authors.initials: H
authors.lastname: He
authors.affiliation: Department of Pathology, Henan Provincial People's Hospital, People's Hospital of Zhengzhou University Zhengzhou, China.
authors.forename: Lingfei
authors.initials: L
authors.lastname: Kong
mesh_terms: D000008:Abdominal Neoplasms / Q000188:drug therapy* / Q000201:enzymology / Q000235:genetics / Q000473:pathology / Q000601:surgery; D000077548:Anaplastic Lymphoma Kinase; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D017024:Chemotherapy, Adjuvant; D000077547:Crizotinib; D004252:DNA Mutational Analysis; D015622:Epithelioid Cells / Q000187:drug effects* / Q000201:enzymology / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D008297:Male; D058990:Molecular Targeted Therapy; D009154:Mutation; D058628:Myofibroblasts / Q000187:drug effects* / Q000201:enzymology / Q000473:pathology; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use*; D011720:Pyrazoles / Q000627:therapeutic use*; D011725:Pyridines / Q000627:therapeutic use*; D020794:Receptor Protein-Tyrosine Kinases / Q000037:antagonists & inhibitors* / Q000235:genetics / Q000378:metabolism; D012509:Sarcoma / Q000188:drug therapy* / Q000201:enzymology / Q000235:genetics / Q000473:pathology / Q000601:surgery; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D047368:Tumor Burden; D055815:Young Adult
chemical_list: D000970:Antineoplastic Agents; D047428:Protein Kinase Inhibitors; D011720:Pyrazoles; D011725:Pyridines; D000077547:Crizotinib; C000626173:ALK protein, human; D000077548:Anaplastic Lymphoma Kinase; D020794:Receptor Protein-Tyrosine Kinases
keywords: ALK inhibitor; Epithelioid inflammatory myofibroblastic sarcoma; RANBP2-ALK fusion; crizotinib
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