title: Epithelioid inflammatory myofibroblastic sarcoma with recurrence after extensive resection: significant clinicopathologic characteristics of a rare aggressive soft tissue neoplasm.
abstract: A case of epithelioid inflammatory myofibroblastic scarcoma (EIMS) developing in an 8-year-old boy who presented with a bulky intra-abdominal occupying lesion with recurrence undergoing a radical resection was reported. Histologically, the tumor cells arranged in cords, strands or sheets of round-to-epithelioid cells with a vesicular nuclear chromatin pattern, prominent nucleoli and weakly eosinophic or basophilic cytoplasm embedded in the abundant myxoid stroma with lymphocytes infiltration. They were positive for ALK, Desmin, SMA, CD30, but negative for AE1/AE3, LCA, CD2, CD3, CD5, CD7, S-100, CD34, CD31, EMA, MyoD1, and myogenin. An elevated proliferation index was demonstrated by Ki-67 comparing the first and the second lesion. Fluorescence in situ (FISH) showed the presence of chromosomal translocation involving ALK. This case show EIMS is a rare variant of inflammatory myofibroblatic tumor with aggressive biological behavior and unfavourable prognosis. To be familiar with its significant clinicopathologic characteristics could prompt us to take it into consideration when facing the relevant dieases.
authors.affiliation: Department of Pathology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Basic Medicine College of Zhengzhou University China.
authors.forename: Jun
authors.initials: J
authors.lastname: Zhou
authors.affiliation: Department of Pathology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Basic Medicine College of Zhengzhou University China.
authors.forename: Guozhong
authors.initials: G
authors.lastname: Jiang
authors.affiliation: Department of Pathology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Basic Medicine College of Zhengzhou University China.
authors.forename: Dandan
authors.initials: D
authors.lastname: Zhang
authors.affiliation: Department of Pathology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Basic Medicine College of Zhengzhou University China.
authors.forename: Lan
authors.initials: L
authors.lastname: Zhang
authors.affiliation: Department of Pathology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Basic Medicine College of Zhengzhou University China.
authors.forename: Jingjing
authors.initials: J
authors.lastname: Xu
authors.affiliation: Department of Pathology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Basic Medicine College of Zhengzhou University China.
authors.forename: Shenglei
authors.initials: S
authors.lastname: Li
authors.affiliation: Department of Pathology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Basic Medicine College of Zhengzhou University China.
authors.forename: Wencai
authors.initials: W
authors.lastname: Li
authors.affiliation: Department of Pathology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Basic Medicine College of Zhengzhou University China.
authors.forename: Yihui
authors.initials: Y
authors.lastname: Ma
authors.affiliation: Department of Pathology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Basic Medicine College of Zhengzhou University China.
authors.forename: Ahong
authors.initials: A
authors.lastname: Zhao
authors.affiliation: Department of Pathology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Basic Medicine College of Zhengzhou University China.
authors.forename: Zhihua
authors.initials: Z
authors.lastname: Zhao
mesh_terms: D000008:Abdominal Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D000077548:Anaplastic Lymphoma Kinase; D014408:Biomarkers, Tumor / Q000032:analysis / Q000235:genetics; D001706:Biopsy; D049109:Cell Proliferation; D002648:Child; D003937:Diagnosis, Differential; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D007249:Inflammation / Q000235:genetics / Q000473:pathology; D008297:Male; D058628:Myofibroblasts / Q000737:chemistry / Q000473:pathology; D009364:Neoplasm Recurrence, Local; D011237:Predictive Value of Tests; D020794:Receptor Protein-Tyrosine Kinases / Q000235:genetics; D012509:Sarcoma / Q000737:chemistry / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D012983:Soft Tissue Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D014178:Translocation, Genetic; D016896:Treatment Outcome; D047368:Tumor Burden
chemical_list: D014408:Biomarkers, Tumor; C000626173:ALK protein, human; D000077548:Anaplastic Lymphoma Kinase; D020794:Receptor Protein-Tyrosine Kinases
keywords: ALK; Epithelioid inflammatory myofibroblastic scarcoma; FISH; soft tissue neoplasm