{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,3]],"date-time":"2026-02-03T10:58:26Z","timestamp":1770116306424,"version":"3.49.0"},"reference-count":24,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"15","license":[{"start":{"date-parts":[[2025,4,14]],"date-time":"2025-04-14T00:00:00Z","timestamp":1744588800000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Cosmet Laser Ther"],"published-print":{"date-parts":[[2025,4,14]]},"abstract":"<jats:sec>\n                    <jats:title>BACKGROUND<\/jats:title>\n                    <jats:p>Lynch syndrome (LS) is an autosomal dominant disorder caused by impaired DNA mismatch repair during the DNA replication process. The condition increases the risk of gastrointestinal and gynecological cancers as well as oligodendrogliomas and astrocytoma. Herein, the authors report the case of a patient with LS with an unusual tumor with features of both glial and neuronal differentiation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>OBSERVATIONS<\/jats:title>\n                    <jats:p>The authors present the case of a 34-year-old female who presented with headaches and was found to have a large right anterior frontal lobe mass with patchy enhancement. The patient underwent a right-sided craniotomy for tumor resection, with final pathology revealing a low-grade\u2013appearing glioneuronal tumor. Subsequent molecular testing showed an MSH2 mutation in the tumor, and germline testing confirmed a diagnosis of LS. The patient was monitored without adjuvant treatment and subsequently had tumor progression 12 months after her index surgery. A re-resection was performed. Since tumors arising in this setting typically have an aggressive course, Ki-67 was measured, showing an increase from 2%\u20135% to 15%\u201320%. The patient has since been started on concurrent temozolomide and radiation therapy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>LESSONS<\/jats:title>\n                    <jats:p>Although most glioneuronal tumors typically have an indolent course relative to their diffuse glial counterparts, this case suggests that those arising in the setting of LS can have a worse prognosis.<\/jats:p>\n                    <jats:p>\n                      <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" ext-link-type=\"uri\" xlink:href=\"https:\/\/thejns.org\/doi\/10.3171\/CASE24811\">https:\/\/thejns.org\/doi\/10.3171\/CASE24811<\/jats:ext-link>\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.3171\/case24811","type":"journal-article","created":{"date-parts":[[2025,4,14]],"date-time":"2025-04-14T20:16:47Z","timestamp":1744661807000},"source":"Crossref","is-referenced-by-count":0,"title":["Diffuse glioneuronal tumor in Lynch syndrome: illustrative case"],"prefix":"10.3171","volume":"9","author":[{"given":"Stephen","family":"Jaffee","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, Allegheny Health Network Neuroscience Institute, Pittsburgh, Pennsylvania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Trent","family":"Kite","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Allegheny Health Network Neuroscience Institute, Pittsburgh, Pennsylvania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kymberly","family":"Gyure","sequence":"additional","affiliation":[{"name":"Division of Pathology, Allegheny Health Network, Pittsburgh, Pennsylvania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John","family":"Herbst","sequence":"additional","affiliation":[{"name":"Division of Oncology, Allegheny Health Network Cancer Institute, Pittsburgh, Pennsylvania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stephen","family":"Karlovits","sequence":"additional","affiliation":[{"name":"Division of Radiation Oncology, Allegheny Health Network Cancer Institute, Pittsburgh, Pennsylvania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rodney E.","family":"Wegner","sequence":"additional","affiliation":[{"name":"Division of Radiation Oncology, Allegheny Health Network Cancer Institute, Pittsburgh, Pennsylvania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matthew J.","family":"Shepard","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Allegheny Health Network Neuroscience Institute, Pittsburgh, Pennsylvania"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1754","reference":[{"key":"bib1","article-title":"Lynch syndrome (hereditary nonpolyposis colorectal cancer)","author":"Bhattacharya","year":"2024","unstructured":"Bhattacharya PLeslie SWMchugh TW.Lynch syndrome (hereditary nonpolyposis colorectal cancer). In: StatPearls. StatPearls Publishing; 2024."},{"issue":"3","key":"bib2","series-title":"Gastroenterology","first-page":"777","article-title":"American Gastroenterological Association institute guideline on the diagnosis and management of Lynch syndrome","volume":"149","author":"Rubenstein","year":"2015","unstructured":"Rubenstein JHEnns RHeidelbaugh JBarkun A. Clinical Guidelines Committee.American Gastroenterological Association institute guideline on the diagnosis and management of Lynch syndrome. Gastroenterology. 2015;149(3):777-e17."},{"issue":"1","key":"bib3","series-title":"Neuro Oncol","first-page":"167","article-title":"Management of gliomas in patients with Lynch syndrome","volume":"23","author":"Alnahhas","year":"2021","unstructured":"Alnahhas IRayi AOng SGiglio PPuduvalli V.Management of gliomas in patients with Lynch syndrome. Neuro Oncol. 2021;23(1):167-168."},{"issue":"1","key":"bib4","series-title":"CNS Oncol","first-page":"2357532","article-title":"Novel insights toward diagnosis and treatment of glioneuronal and neuronal tumors in young adults","volume":"13","author":"Nunno","year":"2024","unstructured":"Nunno VDAprile MGatto LNovel insights toward diagnosis and treatment of glioneuronal and neuronal tumors in young adults. CNS Oncol. 2024;13(1):2357532."},{"key":"bib5","series-title":"Gliomas","first-page":"211","article-title":"Glioneuronal tumors: insights into a rare tumor entity","author":"Krauze","year":"2021","unstructured":"Krauze AV.Glioneuronal tumors: insights into a rare tumor entity. In: Debinski W, ed. Gliomas. Exon Publications; 2021:211-228."},{"key":"bib6","series-title":"World Neurosurg","first-page":"534","article-title":"Surgical resection and cellular proliferation index predict prognosis for patients with papillary glioneuronal tumor: systematic review and pooled analysis","volume":"107","author":"Ahmed","year":"2017","unstructured":"Ahmed AKDawood HYGerard JSmith TR.Surgical resection and cellular proliferation index predict prognosis for patients with papillary glioneuronal tumor: systematic review and pooled analysis. World Neurosurg. 2017;107:534-541."},{"issue":"6","key":"bib7","series-title":"Neuro Oncol","first-page":"920","article-title":"PARP-mediated PARylation of MGMT is critical to promote repair of temozolomide-induced O6-methylguanine DNA damage in glioblastoma","volume":"23","author":"Wu","year":"2021","unstructured":"Wu SLi XGao Fde Groot JFKoul DYung WKA.PARP-mediated PARylation of MGMT is critical to promote repair of temozolomide-induced O6-methylguanine DNA damage in glioblastoma. Neuro Oncol. 2021;23(6):920-931."},{"issue":"7","key":"bib8","series-title":"Clin Cancer Res","first-page":"1690","article-title":"Restoration of temozolomide sensitivity by PARP inhibitors in mismatch repair deficient glioblastoma is independent of base excision repair","volume":"26","author":"Higuchi","year":"2020","unstructured":"Higuchi FNagashima HNing JKoerner MVAWakimoto HCahill DP.Restoration of temozolomide sensitivity by PARP inhibitors in mismatch repair deficient glioblastoma is independent of base excision repair. Clin Cancer Res. 2020;26(7):1690-1699."},{"issue":"1","key":"bib9","series-title":"Curr Mol Pharmacol","first-page":"102","article-title":"Temozolomide: mechanisms of action, repair and resistance","volume":"5","author":"Zhang","year":"2012","unstructured":"Zhang JStevens MFBradshaw TD.Temozolomide: mechanisms of action, repair and resistance. Curr Mol Pharmacol. 2012;5(1):102-114."},{"issue":"3","key":"bib10","series-title":"Cancers (Basel)","first-page":"580","article-title":"Molecular and metabolic mechanisms underlying selective 5-aminolevulinic acid-induced fluorescence in gliomas","volume":"13","author":"Traylor","year":"2021","unstructured":"Traylor JIPernik MNSternisha ACMcbrayer SKAbdullah KG.Molecular and metabolic mechanisms underlying selective 5-aminolevulinic acid-induced fluorescence in gliomas. Cancers (Basel). 2021;13(3):580."},{"issue":"1","key":"bib11","series-title":"Fam Cancer","first-page":"61","article-title":"Potential risks associated with the use of ionizing radiation for imaging and treatment of colorectal cancer in Lynch syndrome patients","volume":"22","author":"Sun","year":"2023","unstructured":"Sun MMoquet JEllender MPotential risks associated with the use of ionizing radiation for imaging and treatment of colorectal cancer in Lynch syndrome patients. Fam Cancer. 2023;22(1):61-70."},{"issue":"2","key":"bib12","series-title":"Lab Invest","first-page":"160","article-title":"Sporadic and Lynch syndrome-associated mismatch repair-deficient brain tumors","volume":"102","author":"Kim","year":"2022","unstructured":"Kim HLim KYPark JWSporadic and Lynch syndrome-associated mismatch repair-deficient brain tumors. Lab Invest. 2022;102(2):160-171."},{"key":"bib1","article-title":"Lynch syndrome (hereditary nonpolyposis colorectal cancer)","author":"Bhattacharya","year":"2024","unstructured":"Bhattacharya PLeslie SWMchugh TW.Lynch syndrome (hereditary nonpolyposis colorectal cancer). In: StatPearls. StatPearls Publishing; 2024."},{"issue":"3","key":"bib2","series-title":"Gastroenterology","first-page":"777","article-title":"American Gastroenterological Association institute guideline on the diagnosis and management of Lynch syndrome","volume":"149","author":"Rubenstein","year":"2015","unstructured":"Rubenstein JHEnns RHeidelbaugh JBarkun A. Clinical Guidelines Committee.American Gastroenterological Association institute guideline on the diagnosis and management of Lynch syndrome. Gastroenterology. 2015;149(3):777-e17."},{"issue":"1","key":"bib3","series-title":"Neuro Oncol","first-page":"167","article-title":"Management of gliomas in patients with Lynch syndrome","volume":"23","author":"Alnahhas","year":"2021","unstructured":"Alnahhas IRayi AOng SGiglio PPuduvalli V.Management of gliomas in patients with Lynch syndrome. Neuro Oncol. 2021;23(1):167-168."},{"issue":"1","key":"bib4","series-title":"CNS Oncol","first-page":"2357532","article-title":"Novel insights toward diagnosis and treatment of glioneuronal and neuronal tumors in young adults","volume":"13","author":"Nunno","year":"2024","unstructured":"Nunno VDAprile MGatto LNovel insights toward diagnosis and treatment of glioneuronal and neuronal tumors in young adults. CNS Oncol. 2024;13(1):2357532."},{"key":"bib5","series-title":"Gliomas","first-page":"211","article-title":"Glioneuronal tumors: insights into a rare tumor entity","author":"Krauze","year":"2021","unstructured":"Krauze AV.Glioneuronal tumors: insights into a rare tumor entity. In: Debinski W, ed. Gliomas. Exon Publications; 2021:211-228."},{"key":"bib6","series-title":"World Neurosurg","first-page":"534","article-title":"Surgical resection and cellular proliferation index predict prognosis for patients with papillary glioneuronal tumor: systematic review and pooled analysis","volume":"107","author":"Ahmed","year":"2017","unstructured":"Ahmed AKDawood HYGerard JSmith TR.Surgical resection and cellular proliferation index predict prognosis for patients with papillary glioneuronal tumor: systematic review and pooled analysis. World Neurosurg. 2017;107:534-541."},{"issue":"6","key":"bib7","series-title":"Neuro Oncol","first-page":"920","article-title":"PARP-mediated PARylation of MGMT is critical to promote repair of temozolomide-induced O6-methylguanine DNA damage in glioblastoma","volume":"23","author":"Wu","year":"2021","unstructured":"Wu SLi XGao Fde Groot JFKoul DYung WKA.PARP-mediated PARylation of MGMT is critical to promote repair of temozolomide-induced O6-methylguanine DNA damage in glioblastoma. Neuro Oncol. 2021;23(6):920-931."},{"issue":"7","key":"bib8","series-title":"Clin Cancer Res","first-page":"1690","article-title":"Restoration of temozolomide sensitivity by PARP inhibitors in mismatch repair deficient glioblastoma is independent of base excision repair","volume":"26","author":"Higuchi","year":"2020","unstructured":"Higuchi FNagashima HNing JKoerner MVAWakimoto HCahill DP.Restoration of temozolomide sensitivity by PARP inhibitors in mismatch repair deficient glioblastoma is independent of base excision repair. Clin Cancer Res. 2020;26(7):1690-1699."},{"issue":"1","key":"bib9","series-title":"Curr Mol Pharmacol","first-page":"102","article-title":"Temozolomide: mechanisms of action, repair and resistance","volume":"5","author":"Zhang","year":"2012","unstructured":"Zhang JStevens MFBradshaw TD.Temozolomide: mechanisms of action, repair and resistance. Curr Mol Pharmacol. 2012;5(1):102-114."},{"issue":"3","key":"bib10","series-title":"Cancers (Basel)","first-page":"580","article-title":"Molecular and metabolic mechanisms underlying selective 5-aminolevulinic acid-induced fluorescence in gliomas","volume":"13","author":"Traylor","year":"2021","unstructured":"Traylor JIPernik MNSternisha ACMcbrayer SKAbdullah KG.Molecular and metabolic mechanisms underlying selective 5-aminolevulinic acid-induced fluorescence in gliomas. Cancers (Basel). 2021;13(3):580."},{"issue":"1","key":"bib11","series-title":"Fam Cancer","first-page":"61","article-title":"Potential risks associated with the use of ionizing radiation for imaging and treatment of colorectal cancer in Lynch syndrome patients","volume":"22","author":"Sun","year":"2023","unstructured":"Sun MMoquet JEllender MPotential risks associated with the use of ionizing radiation for imaging and treatment of colorectal cancer in Lynch syndrome patients. Fam Cancer. 2023;22(1):61-70."},{"issue":"2","key":"bib12","series-title":"Lab Invest","first-page":"160","article-title":"Sporadic and Lynch syndrome-associated mismatch repair-deficient brain tumors","volume":"102","author":"Kim","year":"2022","unstructured":"Kim HLim KYPark JWSporadic and Lynch syndrome-associated mismatch repair-deficient brain tumors. Lab Invest. 2022;102(2):160-171."}],"container-title":["Journal of Neurosurgery: Case Lessons"],"original-title":[],"link":[{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-case-lessons\/9\/15\/article-CASE24811.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-case-lessons\/9\/15\/article-CASE24811.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-case-lessons\/9\/15\/article-CASE24811.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,2]],"date-time":"2026-02-02T23:40:19Z","timestamp":1770075619000},"score":1,"resource":{"primary":{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-case-lessons\/9\/15\/article-CASE24811.xml"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,4,14]]},"references-count":24,"journal-issue":{"issue":"15"},"URL":"https:\/\/doi.org\/10.3171\/case24811","relation":{},"ISSN":["2694-1902"],"issn-type":[{"value":"2694-1902","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,4,14]]}}}