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Background: Sinonasal teratocarcinosarcoma (SNTCS) is a rare malignancy of the anterior skull base with only 127 cases described in the English literature. Given the rarity of this tumor, new cases and analysis of published reports may assist in future management of SNTCS. Objectives: 1) Describe findings from a systematic review of all available literature for malignant SNTCS including the clinical presentation, treatment modalities and outcomes. 2) Present two new cases of this rare anterior skull base tumor. 3) Compare treatment outcomes with respect to recurrence and mortality. Methods: A systematic review of all English literature available in 2 comprehensive databases was conducted by two independent reviewers using PRISMA guidelines. 85 publications were identified. Each case was reviewed for demographics, treatment and survival, and aggregate treatment outcomes were compared using Kaplan-Meier analysis. Results: A total of 64 articles meeting inclusion criteria were reported in the literature between 1977-2018. This represented a total of 127 patients, with a strong male predominance (83%) and mean age of 50 years (range 10–82). Mean follow-up was 21 months. Recurrence rate was 38%, with mean survival at 2 years of 55%. Almost all patients underwent surgery as a primary treatment modality (90%). The majority of cases were treated with multimodal therapy, with 55% receiving surgery and radiation and 20% receiving surgery with adjuvant chemoradiation. Kaplan-Meier analysis demonstrated a significant survival advantage for patients treated with combined therapy compared to surgery alone (p < 0.001) but did not show differences in recurrence (p = 0.085). Conclusion: Two-year survival rates for SNTCS are 55%. Multimodality treatment outcomes appear to be superior to surgery alone based on the published data of this rare skull base tumor, although heterogeneity of treatment methods and reporting bias limits the generalizability of these findings. [ABSTRACT FROM AUTHOR]