Paraneoplastic Syndrome of Angiomatoid Fibrous Histiocytoma May Be Caused by EWSR1-CREB1 Fusion-induced Excessive Interleukin-6 Production

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 immunopatholog...

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Veröffentlicht in:Journal of pediatric hematology/oncology 2015-10, Vol.37 (7), p.554-559
Hauptverfasser: 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
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container_end_page 559
container_issue 7
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container_title Journal of pediatric hematology/oncology
container_volume 37
creator 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
description 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.
doi_str_mv 10.1097/MPH.0000000000000390
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subjects Child
Female
Histiocytoma, Malignant Fibrous - complications
Histiocytoma, Malignant Fibrous - genetics
Histiocytoma, Malignant Fibrous - pathology
Humans
Immunohistochemistry
In Situ Hybridization, Fluorescence
Interleukin-6 - biosynthesis
Interleukin-6 - genetics
Oncogene Proteins, Fusion - genetics
Paraneoplastic Syndromes - etiology
Reverse Transcriptase Polymerase Chain Reaction
Soft Tissue Neoplasms - complications
Soft Tissue Neoplasms - genetics
Soft Tissue Neoplasms - pathology
title Paraneoplastic Syndrome of Angiomatoid Fibrous Histiocytoma May Be Caused by EWSR1-CREB1 Fusion-induced Excessive Interleukin-6 Production
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