LAUSR

Simple Summary Primary skull base chondrosarcomas (SBCs) may carry significant tumor-burden by causing severe cranial nerve neuropathies. Current treatment strategies mainly focus on surgical resection and radiotherapy protocols, with a wide range of findings in terms of efficacy and safety. The aim of our systematic review was to comprehensively analyze the current literature on primary SBCs, describing clinical and radiological characteristics, available management strategies, treatment outcomes, and prognoses. We found that most primary SBCs show benign slow-growing patterns but may cause neurological deficits by compressing critical neurovascular structures. Open surgical approaches favor maximal resection with acceptable complication rates, but only a few studies reported the use of newer endoscopic approaches. Proton-based, photon-based, and carbon-based radiotherapy protocols may also allow safe and effective local tumor control as adjuvant treatments or stand-alone strategies in patients not eligible to undergo surgery. Overall, primary SBCs’ prognoses proved to be favorable and comparable to benign skull base neoplasms. Abstract Background: Primary skull base chondrosarcomas (SBCs) can severely affect patients’ 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, 0.1–376). 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.