title: Angiomatoid fibrous histiocytoma with t(2;22)(q33;q12.2) and EWSR1 gene rearrangement.
abstract: Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor of low malignant potential. The tumor is mostly seen in the deep dermis and subcutis of the lower extremities in children and young adults. Histologically, the tumor forms lobulated sheets of plump round to spindle cells surrounded by a fibrous pseudocapsule and lymphoid cuff. The cytogenetic and molecular hallmarks of AFH are not well defined. Only 4 of 30 reported cases of AFH have had karyotypic information. We present a case of AFH in the inguinal region of a 12-year-old girl. The tumor showed characteristic histological features, t(2;22)(q33;q12.2), and EWSR gene rearrangement by fluorescence in situ hybridization.
authors.affiliation: Department of Pathology, Children's Mercy Hospitals and Clinics, School of Medicine, University of Missouri-Kansas City, Kansas City, MO, USA. lshao@cmh.edu
authors.forename: Lei
authors.initials: L
authors.lastname: Shao
authors.forename: Vivekanand
authors.initials: V
authors.lastname: Singh
authors.forename: Linda
authors.initials: L
authors.lastname: Cooley
mesh_terms: D002148:Calmodulin-Binding Proteins / Q000235:genetics*; D002648:Child; D002889:Chromosomes, Human, Pair 2 / Q000235:genetics*; D002892:Chromosomes, Human, Pair 22 / Q000235:genetics*; D017362:Cyclic AMP Response Element-Binding Protein / Q000235:genetics; D005260:Female; D050939:Gene Fusion; D015321:Gene Rearrangement; D051677:Histiocytoma, Malignant Fibrous / Q000235:genetics* / Q000601:surgery; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D007621:Karyotyping; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins / Q000235:genetics*; D012983:Soft Tissue Neoplasms / Q000235:genetics* / Q000601:surgery; D014178:Translocation, Genetic; D016896:Treatment Outcome
chemical_list: C115163:CREB1 protein, human; D002148:Calmodulin-Binding Proteins; D017362:Cyclic AMP Response Element-Binding Protein; C500986:EWSR1 protein, human; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins