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Objectives: Desmoplastic melanoma of the oral cavity is an extremely rare condition that is often confused on initial diagnosis with non-melanoticbenign lesion or spindle cell tumors. The purpose of this article was to raise awareness of the disease using a literature review. Materials and Methods: We analyzed 19 desmoplastic melanoma cases reported in the literature and added our experience. Data on clinical, histopathology,treatment, and survival were retrieved and analyzed. Survival analysis was by the Kaplan-Meier method. Results: Initial clinical and histopathological features were indistinctive, and a definite diagnosis of desmoplastic melanoma at initial assessment waspossible in only 23.5% of cases. Among tests, immunohistochemical studies for S-100 and vimentin were all positive. The 5-year disease-free survivalrate for oral desmoplastic melanoma was 0%, and the 5-year overall survival rate was 55.0%. Conclusion: Oral desmoplastic melanoma has a high percentage of initial misdiagnosis and propensity for local recurrence. Thus, careful initial diagnosisand adequate surgery may result in improved overall survival.

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