Paraneoplastic Syndrome of Angiomatoid Fibrous Histiocytoma May Be Caused by EWSR1-CREB1 Fusion-induced Excessive Interleukin-6 Production. Issue 7 (October 2015)

• Record Type: Journal Article
• Authors: Akiyama, Masaharu; Yamaoka, Masayoshi; Mikami-Terao, Yoko; Yokoi, Kentaro; Inoue, Takashi; Hiramatsu, Tomomasa; Ashizuka, Shuichi; Yoshizawa, Jyoji; Katagi, Hiroaki; Ikegami, Masahiro; Ida, Hiroyuki; Nakazawa, Atsuko; Okita, Hajime; Matsumoto, Kenji
• Is Part Of: Journal of pediatric hematology/oncology. Volume 37:Issue 7(2015)
• Publication Date: 2015-10
• Subjects: Pediatric hematology -- Periodicals; Tumors in children -- Periodicals; 618.9215
• DOI: 10.1097/MPH.0000000000000390
• Languages: English
• ISSNs: 1077-4114

Abstract

<abstract> <title> <x xml:space="preserve">Abstract</x> </title> <sec> <p>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 <italic>EWSR1-CREB1</italic> fusion gene detected in the tumor leads to continuous activation of <italic>CREB1</italic> 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.</p> </sec> </abstract>