{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,18]],"date-time":"2026-10-18T02:45:17Z","timestamp":1792291517096,"version":"4.4.0"},"reference-count":46,"publisher":"MDPI AG","issue":"14","license":[{"start":{"date-parts":[[2022,7,16]],"date-time":"2022-07-16T00:00:00Z","timestamp":1657929600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Background: Ollier disease (OD) is a rare nonhereditary type of dyschondroplasia characterized by multiple enchondromas, with typical onset in the first decade of life. Surgery is the only curative treatment for primary disease and its complications. Patients with OD are at risk of malignant transformation of enchondromas and of occurrence of other neoplasms. Methods: A wide literature review disclosed thirty cases of glioma associated with OD, most of them belonging to the pre-molecular era. Our own case was also included. Demographic, clinical, pathologic, molecular, management, and outcome data were analyzed and compared to those of sporadic gliomas. Results: Gliomas associated with OD more frequently occur at younger age, present higher rates of multicentric lesions (49%), brainstem localizations (29%), and significantly lower rates of glioblastomas (7%) histotype. The IDH1 R132H mutation was detected in 80% of gliomas of OD patients and simultaneously in enchondromas and gliomas in 100% of cases. Conclusions: The molecular data suggest a higher risk of occurrence of glioma in patients with enchondromas harboring the IDH1 R132H mutation than those with the IDH1 R132C mutation. Thus, we suggest considering the IDH1 R132H mutation in enchondromas of patients with OD as a predictive risk factor of occurrence of glioma.<\/jats:p>","DOI":"10.3390\/cancers14143464","type":"journal-article","created":{"date-parts":[[2022,7,17]],"date-time":"2022-07-17T21:00:28Z","timestamp":1658091628000},"page":"3464","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Brain Gliomas and Ollier Disease: Molecular Findings as Predictive Risk Factors?"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4918-6857","authenticated-orcid":false,"given":"Sergio","family":"Corvino","sequence":"first","affiliation":[{"name":"Department of Neurosciences, Reproductive and Odontostomatological Sciences, Neurosurgical Clinic, School of Medicine, University of Naples \u201cFederico II\u201d, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7755-0105","authenticated-orcid":false,"given":"Giuseppe","family":"Mariniello","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Reproductive and Odontostomatological Sciences, Neurosurgical Clinic, School of Medicine, University of Naples \u201cFederico II\u201d, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe","family":"Corazzelli","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Reproductive and Odontostomatological Sciences, Neurosurgical Clinic, School of Medicine, University of Naples \u201cFederico II\u201d, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raduan Ahmed","family":"Franca","sequence":"additional","affiliation":[{"name":"Department of Advanced Biomedical Sciences, Section of Pathology, School of Medicine, University of Naples \u201cFederico II\u201d, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marialaura","family":"Del Basso De Caro","sequence":"additional","affiliation":[{"name":"Department of Advanced Biomedical Sciences, Section of Pathology, School of Medicine, University of Naples \u201cFederico II\u201d, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rosa","family":"Della Monica","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine and Medical Biotechnology, School of Medicine, University of Naples \u201cFederico II\u201d, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lorenzo","family":"Chiariotti","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine and Medical Biotechnology, School of Medicine, University of Naples \u201cFederico II\u201d, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Maiuri","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Reproductive and Odontostomatological Sciences, Neurosurgical Clinic, School of Medicine, University of Naples \u201cFederico II\u201d, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,7,16]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"647","DOI":"10.1007\/s00381-009-0873-z","article-title":"Ollier disease","volume":"25","author":"Massimi","year":"2009","journal-title":"Childs Nerv. Syst."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1186\/1750-1172-1-37","article-title":"Ollier disease","volume":"1","author":"Silve","year":"2006","journal-title":"Orphanet. J. Rare Dis."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"645","DOI":"10.1002\/pbc.21050","article-title":"Acute myelogenous leukemia associated with Ollier disease","volume":"50","author":"White","year":"2008","journal-title":"Pediatr. Blood Cancer"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1605","DOI":"10.1002\/1097-0142(19840401)53:7<1605::AID-CNCR2820530731>3.0.CO;2-N","article-title":"Enchondromatosis (Ollier\u2019s disease) and ovarian juvenile granulosa cell tumor","volume":"53","author":"Tamimi","year":"1984","journal-title":"Cancer"},{"key":"ref_5","first-page":"903","article-title":"Identical twins with Ollier\u2019s disease and intracranial gliomas: Case report","volume":"34","author":"Chang","year":"1994","journal-title":"Neurosurgery"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"688","DOI":"10.1007\/s002560050460","article-title":"Simultaneous occurrence of a supra- and an infratentorial glioma in a patient with Ollier\u2019s disease: More evidence for non-mesodermal tumor predisposition in multiple enchondromatosis","volume":"27","author":"Hofman","year":"1998","journal-title":"Skelet. Radiol."},{"key":"ref_7","first-page":"1261","article-title":"Multiple enchondromatosis Ollier\u2019s disease with two primary brain tumors","volume":"25","author":"Mahafza","year":"2004","journal-title":"Saudi Med. J."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1007\/BF01808830","article-title":"Thalamic gliomas: A clinicopathologic analysis of 20 cases with reference to patient age","volume":"139","author":"Nishio","year":"1997","journal-title":"Acta Neurochir."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"400","DOI":"10.1227\/00006123-198709000-00023","article-title":"A case of Ollier\u2019s disease associated with two intracranial gliomas","volume":"21","author":"Rawlings","year":"1987","journal-title":"Neurosurgery"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"S607","DOI":"10.4103\/2152-7806.189731","article-title":"Ollier disease with anaplastic astrocytoma: A review of the literature and a unique case","volume":"7","author":"Gajavelli","year":"2016","journal-title":"Surg. Neurol. Int."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"376","DOI":"10.1007\/BF00314239","article-title":"Ollier\u2019s disease and Maffucci\u2019s syndrome: Distinct entities or a continuum. Case report: Enchondromatosis complicated by an intracranial glioma","volume":"235","author":"Mellon","year":"1988","journal-title":"J. Neurol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1007\/BF00389685","article-title":"Dyschondroplasia with glioma of the brain. Third histologically verified case (author\u2019s transl)","volume":"93","author":"Becker","year":"1979","journal-title":"Arch. Orthop. Trauma. Surg."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1097\/00041327-199903000-00021","article-title":"Neuro-ophthalmologic manifestations of Maffucci\u2019s syndrome and Ollier\u2019s disease","volume":"19","author":"Balcer","year":"1999","journal-title":"J. Neuroophthalmol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"58","DOI":"10.4103\/0971-3026.95406","article-title":"Multifocal intracranial astrocytoma in a pediatric patient with Ollier disease","volume":"22","author":"Bathla","year":"2012","journal-title":"Indian J. Radiol. Imaging"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1016\/0720-048X(91)90115-C","article-title":"Multiple enchondromatosis (Ollier\u2019s disease) complicated by malignant astrocytoma","volume":"12","author":"Bendel","year":"1991","journal-title":"Eur. J. Radiol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1007\/s11060-008-9583-8","article-title":"Cerebellar anaplastic astrocytoma in a teenager with Ollier Disease","volume":"89","author":"Walid","year":"2008","journal-title":"J. Neurooncol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"2299","DOI":"10.1016\/j.radcr.2021.05.046","article-title":"Diffuse midline glioma in Ollier disease: A case report and a brief review of the literature","volume":"16","author":"Karabulut","year":"2021","journal-title":"Radiol. Case Rep."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"269","DOI":"10.2106\/00004623-198769020-00016","article-title":"The malignant potential of enchondromatosis","volume":"69","author":"Schwartz","year":"1987","journal-title":"J. Bone Jt. Surg. Am."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1227\/00006123-199009000-00029","article-title":"A case of dyschondroplasia associated with brain stem glioma: Diagnosis by stereotactic biopsy","volume":"27","author":"Patt","year":"1990","journal-title":"Neurosurgery"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"222","DOI":"10.1007\/s003810050377","article-title":"Diffuse brain stem tumor in an adolescent with multiple enchondromatosis (Ollier\u2019s disease)","volume":"15","author":"Frappaz","year":"1999","journal-title":"Childs Nerv. Syst."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"363","DOI":"10.3171\/2009.5.PEDS08422","article-title":"Pediatric thalamic glioblastoma associated with Ollier disease (multiple enchondromatosis): A rare case of concurrence","volume":"4","author":"Ranger","year":"2009","journal-title":"J. Neurosurg. Pediatr."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"595","DOI":"10.3892\/ol_00000105","article-title":"Diffuse gliomas in an adolescent with multiple enchondromatosis (Ollier\u2019s disease)","volume":"1","author":"Hori","year":"2010","journal-title":"Oncol. Lett."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1007\/s10014-017-0303-y","article-title":"Enchondromatosis-associated oligodendroglioma: Case report and literature review","volume":"35","author":"Achiha","year":"2018","journal-title":"Brain Tumor Pathol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1016\/j.jocn.2011.06.019","article-title":"Multifocal supratentorial diffuse glioma in a young patient with Ollier disease","volume":"19","author":"Pearce","year":"2012","journal-title":"J. Clin. Neurosci."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1097\/01.wnq.0000214039.38720.b4","article-title":"Ollier Disease Anaplastic Mixed Oligoastrocytoma A Rare Association With Brain Tumors","volume":"16","author":"Koc","year":"2006","journal-title":"Neurosurg. Q."},{"key":"ref_26","first-page":"66","article-title":"Ollier\u2019s disease with intracranial glioma","volume":"12","author":"Simsek","year":"2002","journal-title":"Child\u2019s Nerv. Syst."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"106","DOI":"10.1016\/S0303-8467(98)00072-9","article-title":"A case of Ollier\u2019s disease associated with two intracerebral low-grade gliomas","volume":"101","year":"1999","journal-title":"Clin. Neurol. Neurosurg."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"1093","DOI":"10.1002\/ajmg.a.61530","article-title":"Natural history of Ollier disease and Maffucci syndrome: Patient survey and review of clinical literature","volume":"182","author":"Robbins","year":"2020","journal-title":"Am. J. Med. Genet. A"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"1771","DOI":"10.1634\/theoncologist.2011-0200","article-title":"Incidence, predictive factors, and prognosis of chondrosarcoma in patients with Ollier disease and Maffucci syndrome: An international multicenter study of 161 patients","volume":"16","author":"Verdegaal","year":"2011","journal-title":"Oncologist"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1186\/s40478-016-0302-y","article-title":"Characteristics of gliomas in patients with somatic IDH mosaicism","volume":"4","author":"Bonnet","year":"2016","journal-title":"Acta Neuropathol. Commun."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"469","DOI":"10.1038\/nature26000","article-title":"DNA methylation-based classification of central nervous system tumours","volume":"555","author":"Capper","year":"2018","journal-title":"Nature"},{"key":"ref_32","doi-asserted-by":"crossref","unstructured":"Della Monica, R., Cuomo, M., Buonaiuto, M., Costabile, D., Franca, R.A., Del Basso De Caro, M., Catapano, G., Chiariotti, L., and Visconti, R. (2022). MGMT and Whole-Genome DNA Methylation Impacts on Diagnosis, Prognosis and Therapy of Glioblastoma Multiforme. Int. J. Mol. Sci., 23.","DOI":"10.3390\/ijms23137148"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"1231","DOI":"10.1093\/neuonc\/noab106","article-title":"The 2021 WHO Classification of Tumors of the Central Nervous System: A summary","volume":"23","author":"Louis","year":"2021","journal-title":"Neuro Oncol."},{"key":"ref_34","unstructured":"(2016). WHO Classification of Tumours of the Central Nervous System, International Agency for Research on Cancer. [4th ed.]."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1038\/s41571-020-00447-z","article-title":"EANO guidelines on the diagnosis and treatment of diffuse gliomas of adulthood","volume":"18","author":"Weller","year":"2021","journal-title":"Nat. Rev. Clin. Oncol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"765","DOI":"10.1056\/NEJMoa0808710","article-title":"IDH1 and IDH2 mutations in gliomas","volume":"360","author":"Yan","year":"2009","journal-title":"N. Engl. J. Med."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"334","DOI":"10.1002\/path.2913","article-title":"IDH1 and IDH2 mutations are frequent events in central chondrosarcoma and central and periosteal chondromas but not in other mesenchymal tumours","volume":"224","author":"Amary","year":"2011","journal-title":"J. Pathol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"1262","DOI":"10.1038\/ng.994","article-title":"Ollier disease and Maffucci syndrome are caused by somatic mosaic mutations of IDH1 and IDH2","volume":"43","author":"Amary","year":"2011","journal-title":"Nat. Genet."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"727","DOI":"10.1002\/ana.22106","article-title":"Natural history of incidental World Health Organization grade II gliomas","volume":"68","author":"Pallud","year":"2010","journal-title":"Ann. Neurol."},{"key":"ref_40","first-page":"10","article-title":"Understanding inherited genetic risk of adult glioma\u2014a review","volume":"3","author":"Rice","year":"2016","journal-title":"Neurooncol. Pract."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"2481","DOI":"10.1056\/NEJMoa1402121","article-title":"Comprehensive, Integrative Genomic Analysis of Diffuse Lower-Grade Gliomas","volume":"372","author":"Brat","year":"2015","journal-title":"N. Engl. J. Med."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"1256","DOI":"10.1038\/ng.1004","article-title":"Somatic mosaic IDH1 and IDH2 mutations are associated with enchondroma and spindle cell hemangioma in Ollier disease and Maffucci syndrome","volume":"43","author":"Pansuriya","year":"2011","journal-title":"Nat. Genet."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"8522","DOI":"10.1038\/s41598-021-88023-y","article-title":"A survival analysis of surgically treated incidental low-grade glioma patients","volume":"11","author":"Zeng","year":"2021","journal-title":"Sci. Rep."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"365","DOI":"10.3171\/2011.9.JNS111068","article-title":"Natural history and surgical management of incidentally discovered low-grade gliomas","volume":"116","author":"Potts","year":"2012","journal-title":"J. Neurosurg."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"2237","DOI":"10.1007\/s00701-013-1886-7","article-title":"The silent phase of diffuse low-grade gliomas. Is it when we missed the action?","volume":"155","author":"Pallud","year":"2013","journal-title":"Acta Neurochir."},{"key":"ref_46","first-page":"vdab101","article-title":"Systematic review-Time to malignant transformation in low-grade gliomas: Predicting a catastrophic event with clinical, neuroimaging, and molecular markers","volume":"3","author":"Satar","year":"2021","journal-title":"Neurooncol. Adv."}],"container-title":["Cancers"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2072-6694\/14\/14\/3464\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T23:52:09Z","timestamp":1760140329000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2072-6694\/14\/14\/3464"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,7,16]]},"references-count":46,"journal-issue":{"issue":"14","published-online":{"date-parts":[[2022,7]]}},"alternative-id":["cancers14143464"],"URL":"https:\/\/doi.org\/10.3390\/cancers14143464","relation":{},"ISSN":["2072-6694"],"issn-type":[{"value":"2072-6694","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,7,16]]}}}