{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T01:18:33Z","timestamp":1787015913423,"version":"build-2736575974"},"reference-count":33,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2022,11,3]],"date-time":"2022-11-03T00:00:00Z","timestamp":1667433600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100015636","name":"Ishibashi Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100015636","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002336","name":"Daiichi Sankyo Company","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100002336","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2023,6]]},"abstract":"<jats:p>\n                    Pediatric neoplastic diseases account for about 10% of cases of fever of unknown origin (FUO), and most neoplastic disease cases are leukemia, lymphoma, and neuroblastoma. Brain tumors are rarely reported as the cause of FUO, although craniopharyngioma, metastatic brain tumor, and Castleman's disease have been reported. We report a case of intracranial mesenchymal tumor (IMT) with a\n                    <jats:italic>FET<\/jats:italic>\n                    :\n                    <jats:italic>CREB<\/jats:italic>\n                    fusion gene, which had inflammatory phenotype without neurological signs. A 10\u2010year\u2010old girl was admitted with a 2\u2010month history of intermittent fever and headache, whereas her past history as well as her family history lacked special events. Sepsis work\u2010up showed no pathological organism, and empirical antibiotic therapy was not effective. Bone marrow examination showed a negative result. Cerebrospinal fluid examination showed elevated protein as well as cell counts, and head magnaetic resonance imaging showed a hypervascular mass lesion with contrast enhancement in the left cerebellar hemisphere. The patient underwent tumor excision, which made the intermittent fever disappear. Pathological examinations resembled those of classic angiomatoid fibrous histiocytoma (AFH), but the morphological features were distinct from the AFH myxoid variant; then we performed break\u2010apart fluorescence\n                    <jats:italic>in situ<\/jats:italic>\n                    hybridization and confirmed the tumor harbored the rare\n                    <jats:italic>EWSR1<\/jats:italic>\n                    ::\n                    <jats:italic>CREM<\/jats:italic>\n                    fusion gene (Ewing sarcoma breakpoint region 1 gene (\n                    <jats:italic>EWSR1<\/jats:italic>\n                    ) and cAMP response element binding (\n                    <jats:italic>CREB<\/jats:italic>\n                    ) family gene). Consequently, we diagnosed the condition as IMT with\n                    <jats:italic>EWSR1<\/jats:italic>\n                    ::\n                    <jats:italic>CREM<\/jats:italic>\n                    fusion. Elevated serum concentration of interleukin 6 (IL\u20106) was normalized after tumor resection, which suggested the fever could be caused by tumor\u2010derived IL\u20106. This is the first case of IMT with\n                    <jats:italic>EWSR1<\/jats:italic>\n                    ::\n                    <jats:italic>CREM<\/jats:italic>\n                    fusion that showed paraneoplastic symptoms associated with the IL\u20106\/signal transducer and activator of transcription 3 (STAT3) signaling pathway. Although brain tumors are rarely diagnosed as a responsible disease for FUO, they should be considered as a cause of unknown fever even in the absence of abnormal neurological findings.\n                  <\/jats:p>","DOI":"10.1111\/neup.12877","type":"journal-article","created":{"date-parts":[[2022,11,3]],"date-time":"2022-11-03T22:15:54Z","timestamp":1667513754000},"page":"244-251","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Systemic inflammation caused by an intracranial mesenchymal tumor with a\n                    <i>EWSR1<\/i>\n                    ::\n                    <i>CREM<\/i>\n                    fusion presenting associated with\n                    <scp>IL<\/scp>\n                    \u20106\/\n                    <scp>STAT3<\/scp>\n                    signaling"],"prefix":"10.1111","volume":"43","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3517-9173","authenticated-orcid":false,"given":"Keishiro","family":"Hojo","sequence":"first","affiliation":[{"name":"Department of Pediatrics and Child Health Kurume University School of Medicine  Kurume Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6645-7818","authenticated-orcid":false,"given":"Takuya","family":"Furuta","sequence":"additional","affiliation":[{"name":"Department of Pathology Kurume University School of Medicine  Kurume Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoru","family":"Komaki","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery Kurume University School of Medicine  Kurume Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yukako","family":"Yoshikane","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Fukuoka University Chikushi Hospital  Chikushino Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jin","family":"Kikuchi","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery Kurume University School of Medicine  Kurume Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hideo","family":"Nakamura","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery Kurume University School of Medicine  Kurume Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mizuki","family":"Ide","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Child Health Kurume University School of Medicine  Kurume Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saho","family":"Shima","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Child Health Kurume University School of Medicine  Kurume 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