title: Paraneoplastic Syndrome of Angiomatoid Fibrous Histiocytoma May Be Caused by EWSR1-CREB1 Fusion-induced Excessive Interleukin-6 Production.
abstract: We describe a 7-year-old girl with angiomatoid fibrous histiocytoma (AFH) presenting severe inflammatory symptoms. The cytokine/chemokine profile of serum samples before and after surgery demonstrated that interleukin (IL)-6 had decreased by the greatest percentage. The AFH cells were immunopathologically positive for IL-6 and Tyr705-phosphorylation of signal transducer and activator of transcription 3. The EWSR1-CREB1 fusion gene detected in the tumor leads to continuous activation of CREB1 and IL-6 production, because the promoter region of IL-6 has a CREB binding site. Thus, IL-6 plays pivotal roles in both paraneoplastic syndrome and the oncogenesis of AFH.
authors.affiliation: Departments of *Pediatrics §Pathology ‡Division of Pediatric Surgery †Division of Molecular Genetics, Institute of DNA Medicine, The Jikei University School of Medicine ∥Department of Pathology, National Center for Child Health and Development Departments of ¶Pediatric Hematology and Oncology Research #Allergy and Immunology, National Research Institute for Child Health and Development, Tokyo, Japan.
authors.forename: Masaharu
authors.initials: M
authors.lastname: Akiyama
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Yamaoka
authors.forename: Yoko
authors.initials: Y
authors.lastname: Mikami-Terao
authors.forename: Kentaro
authors.initials: K
authors.lastname: Yokoi
authors.forename: Takashi
authors.initials: T
authors.lastname: Inoue
authors.forename: Tomomasa
authors.initials: T
authors.lastname: Hiramatsu
authors.forename: Shuichi
authors.initials: S
authors.lastname: Ashizuka
authors.forename: Jyoji
authors.initials: J
authors.lastname: Yoshizawa
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Katagi
authors.forename: Masahiro
authors.initials: M
authors.lastname: Ikegami
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Ida
authors.forename: Atsuko
authors.initials: A
authors.lastname: Nakazawa
authors.forename: Hajime
authors.initials: H
authors.lastname: Okita
authors.forename: Kenji
authors.initials: K
authors.lastname: Matsumoto
mesh_terms: D002648:Child; D005260:Female; D051677:Histiocytoma, Malignant Fibrous / Q000150:complications / Q000235:genetics* / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D015850:Interleukin-6 / Q000096:biosynthesis* / Q000235:genetics; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D010257:Paraneoplastic Syndromes / Q000209:etiology*; D020133:Reverse Transcriptase Polymerase Chain Reaction; D012983:Soft Tissue Neoplasms / Q000150:complications / Q000235:genetics* / Q000473:pathology
chemical_list: C574775:EWSR1-CREB1 fusion protein, human; C508600:IL6 protein, human; D015850:Interleukin-6; D015514:Oncogene Proteins, Fusion