{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,4]],"date-time":"2026-11-04T00:35:38Z","timestamp":1793752538619,"version":"4.6.1"},"reference-count":16,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2020,12,3]],"date-time":"2020-12-03T00:00:00Z","timestamp":1606953600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,1,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Central nervous system neuroblastoma with FOXR2 activation (CNS NB FOXR2) has recently been described as a class of brain tumors sharing common genetic events and a highly similar DNA methylation profile. Most of these tumors have previously been diagnosed as primitive neuroectodermal tumor (PNET). Whereas the entity of PNET has been removed from the WHO classification of brain tumors in its current edition, CNS neuroblastoma was kept as an entity, but still lacks any molecular detail. Here, we describe 8 cases of CNS NB FOXR2 focusing on histomorphological and immunohistochemical features and include magnetic resonance imaging (MRI) for 2 of these cases. MRI revealed large supratentorial masses in superficial location with prominent cysts and necrosis, but little edema. Diffusion and enhancement characteristics were variable. Histological analyses showed that most of the cases displayed neuronal differentiation with necrosis, endothelial proliferation, and high vascularity. Immunohistochemistry revealed strong expression of synaptophysin, MAP2, and OLIG2 as well as moderate proliferation. These findings suggest that tumors with the molecular diagnosis of CNS NB FOXR2 may fit well into the WHO entity of CNS neuroblastoma. Our findings may be helpful when establishing an integrated diagnosis and may be indispensable if molecular data are unavailable.<\/jats:p>","DOI":"10.1093\/jnen\/nlaa128","type":"journal-article","created":{"date-parts":[[2020,12,3]],"date-time":"2020-12-03T20:08:41Z","timestamp":1607026121000},"page":"52-59","source":"Crossref","is-referenced-by-count":36,"title":["Detailed Clinical and Histopathological Description of 8 Cases of Molecularly Defined CNS Neuroblastomas"],"prefix":"10.1093","volume":"80","author":[{"given":"Till","family":"Holsten","sequence":"first","affiliation":[{"name":"Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf"},{"name":"Research Institute, Children's Cancer Center Hamburg"},{"name":"Institute of Neuropathology, University Medical Center Hamburg-Eppendorf , Hamburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabiana","family":"Lubieniecki","sequence":"additional","affiliation":[{"name":"Department of Pediatric Pathology and Neuropathology, Pediatric Hospital \u201cDr. Prof. J.P. Garrahan\u201d , Buenos Aires, Argentina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Spohn","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf"},{"name":"Research Institute, Children's Cancer Center Hamburg"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Mynarek","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brigitte","family":"Bison","sequence":"additional","affiliation":[{"name":"Institute of Diagnostic and Interventional Neuroradiology of the University Hospital W\u00fcrzburg , W\u00fcrzburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulrike","family":"L\u00f6bel","sequence":"additional","affiliation":[{"name":"Department of Radiology, Great Ormond Street Hospital , London, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan","family":"Rutkowski","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulrich","family":"Sch\u00fcller","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf"},{"name":"Research Institute, Children's Cancer Center Hamburg"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2020,12,3]]},"reference":[{"key":"2024032523372178100_nlaa128-B1","doi-asserted-by":"crossref","first-page":"803","DOI":"10.1007\/s00401-016-1545-1","article-title":"The 2016 World Health Organization Classification of Tumors of the Central Nervous System: A summary","volume":"131","author":"Louis","year":"2016","journal-title":"Acta Neuropathol"},{"key":"2024032523372178100_nlaa128-B2","doi-asserted-by":"crossref","first-page":"817","DOI":"10.1007\/s00381-018-3764-3","article-title":"Central nervous system (CNS) neuroblastoma. A case-based update","volume":"34","author":"Bianchi","year":"2018","journal-title":"Childs Nerv Syst"},{"key":"2024032523372178100_nlaa128-B3","doi-asserted-by":"crossref","first-page":"784","DOI":"10.1002\/1097-0142(197708)40:2<784::AID-CNCR2820400228>3.0.CO;2-8","article-title":"Differentiating intracerebral neuroblastoma: Report of a case and review of the literature","volume":"40","author":"Ahdevaara","year":"1977","journal-title":"Cancer"},{"key":"2024032523372178100_nlaa128-B4","first-page":"115","article-title":"Primary cerebral neuroblastoma: CT and MR findings in 12 cases","volume":"11","author":"Davis","year":"1990","journal-title":"Am J Neuroradiol"},{"key":"2024032523372178100_nlaa128-B5","doi-asserted-by":"crossref","first-page":"1060","DOI":"10.1016\/j.cell.2016.01.015","article-title":"New brain tumor entities emerge from molecular classification of CNS-PNETs","volume":"164","author":"Sturm","year":"2016","journal-title":"Cell"},{"key":"2024032523372178100_nlaa128-B6","doi-asserted-by":"crossref","first-page":"735","DOI":"10.1093\/brain\/99.4.735","article-title":"Primary cerebral neuroblastoma. 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