Paraneoplastic syndrome of angiomatoid fibrous histiocytoma may be caused by EWSR1-CREB1 fusion-induced excessive interleukin-6 production

Masaharu Akiyama, Masayoshi Yamaoka, Yoko Mikami-Terao, Kentaro Yokoi, Takashi Inoue, Tomomasa Hiramatsu, Shuichi Ashizuka, Jyoji Yoshizawa, Hiroaki Katagi, Masahiro Ikegami, Hiroyuki Ida, Atsuko Nakazawa, Hajime Okita, Kenji Matsumoto

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4 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)554-559
Number of pages6
JournalJournal of Pediatric Hematology/Oncology
Volume37
Issue number7
DOIs
Publication statusPublished - 2015 Oct 1

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Keywords

  • Angiomatoid fibrous histiocytoma
  • EWSR1-CREB1 fusion gene
  • Interleukin-6
  • Paraneoplastic syndrome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

Cite this

Akiyama, M., Yamaoka, M., Mikami-Terao, Y., Yokoi, K., Inoue, T., Hiramatsu, T., Ashizuka, S., Yoshizawa, J., Katagi, H., Ikegami, M., Ida, H., Nakazawa, A., Okita, H., & Matsumoto, K. (2015). Paraneoplastic syndrome of angiomatoid fibrous histiocytoma may be caused by EWSR1-CREB1 fusion-induced excessive interleukin-6 production. Journal of Pediatric Hematology/Oncology, 37(7), 554-559. https://doi.org/10.1097/MPH.0000000000000390