{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,21]],"date-time":"2026-07-21T06:20:47Z","timestamp":1784614847347,"version":"3.55.0"},"reference-count":67,"publisher":"MDPI AG","issue":"23","license":[{"start":{"date-parts":[[2021,11,26]],"date-time":"2021-11-26T00:00:00Z","timestamp":1637884800000},"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: Primary skull base chondrosarcomas (SBCs) can severely affect patients\u2019 quality of life. Surgical-resection and radiotherapy are feasible but may cause debilitating complications. We systematically reviewed the literature on primary SBCs. Methods: PubMed, EMBASE, Scopus, Web-of-Science, and Cochrane were searched following the PRISMA guidelines to include studies of patients with primary SBCs. Clinical characteristics, management strategies, and treatment outcomes were analyzed. Results: We included 33 studies comprising 1307 patients. Primary SBCs mostly involved the middle-fossa (72.7%), infiltrating the cavernous-sinus in 42.4% of patients. Cranial-neuropathies were reported in 810 patients (62%). Surgical-resection (93.3%) was preferred over biopsy (6.6%). The most frequent open surgical approaches were frontotemporal-orbitozygomatic (17.6%) and pterional (11.9%), and 111 patients (21.3%) underwent endoscopic-endonasal resection. Post-surgical cerebrospinal-fluid leaks occurred in 36 patients (6.5%). Radiotherapy was delivered in 1018 patients (77.9%): photon-based (41.4%), proton-based (64.2%), and carbon-based (13.1%). Severe post-radiotherapy complications, mostly hypopituitarism (15.4%) and hearing loss (7.1%) were experienced by 251 patients (30.7%). Post-treatment symptom-improvement (46.7%) and reduced\/stable tumor volumes (85.4%) showed no differences based on radiotherapy-protocols (p = 0.165; p = 0.062). Median follow-up was 67-months (range, 0.1\u2013376). SBCs recurrences were reported in 211 cases (16.1%). The 5-year and 10-year progression-free survival rates were 84.3% and 67.4%, and overall survival rates were 94% and 84%. Conclusion: Surgical-resection and radiotherapy are effective treatments in primary SBCs, with acceptable complication rates and favorable local tumor control.<\/jats:p>","DOI":"10.3390\/cancers13235960","type":"journal-article","created":{"date-parts":[[2021,11,29]],"date-time":"2021-11-29T01:49:58Z","timestamp":1638150598000},"page":"5960","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":52,"title":["Primary Skull Base Chondrosarcomas: A Systematic Review"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0711-3975","authenticated-orcid":false,"given":"Paolo","family":"Palmisciano","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, Trauma Center, Gamma Knife Center, Cannizzaro Hospital, 95126 Catania, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ali S.","family":"Haider","sequence":"additional","affiliation":[{"name":"Texas A&M University College of Medicine, Houston, TX 77030, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4640-226X","authenticated-orcid":false,"given":"Mohammadmahdi","family":"Sabahi","sequence":"additional","affiliation":[{"name":"Neurosurgery Research Group (NRG), Student Research Committee, Hamadan University of Medical Sciences, Hamadan 65141, Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chibueze D.","family":"Nwagwu","sequence":"additional","affiliation":[{"name":"Emory University School of Medicine, Atlanta, GA 30322, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9365-4115","authenticated-orcid":false,"given":"Othman","family":"Bin Alamer","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, King Abdullah International Medical Research Center, Riyadh 11451, Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9465-2506","authenticated-orcid":false,"given":"Gianluca","family":"Scalia","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Highly Specialized Hospital of National Importance \u201cGaribaldi\u201d, 95126 Catania, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1573-431X","authenticated-orcid":false,"given":"Giuseppe E.","family":"Umana","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Trauma Center, Gamma Knife Center, Cannizzaro Hospital, 95126 Catania, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4946-1524","authenticated-orcid":false,"given":"Aaron A.","family":"Cohen-Gadol","sequence":"additional","affiliation":[{"name":"Department of Neurological Surgery, Indiana University School of Medicine, Indianapolis, IN 46077, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7122-4349","authenticated-orcid":false,"given":"Tarek Y.","family":"El Ahmadieh","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery and Brain Metastasis Center, Memorial Sloan Kettering Cancer Center, New York, NY 10065, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenny","family":"Yu","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery and Brain Metastasis Center, Memorial Sloan Kettering Cancer Center, New York, NY 10065, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5083-9416","authenticated-orcid":false,"given":"Omar N.","family":"Pathmanaban","sequence":"additional","affiliation":[{"name":"Manchester Skull Base Unit, Department of Neurosurgery, Manchester Centre for Clinical Neurosciences, Geoffrey Jefferson Brain Research Centre, Salford Royal Hospital, Manchester Academic Health Science Centre, University of Manchester, Manchester M13 9WL, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,11,26]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1007\/BF01774391","article-title":"Intracranial malignant cartilaginous tumours. 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