Background: Desmoplastic melanoma (DM) represents a relatively rare malignancy. The aim of this study was to describe the incidence and survival of DM in the United States.

Methods: Incidence and survival data were obtained from the National Cancer Institute's Surveillance, Epidemiology and End Results (SEER) program, 1992-2007. Kaplan-Meier and Cox proportional hazards regression methods were used to calculate the survival rates and hazard ratios for DM-specific death.

Results: We identified 1129 DM patients from SEER 13 registries, with 64% in men, 37% in women and most (96.8%) occurring in White populations. The incidence rates per 1,000,000 were 1.3 (female), 3.0 (male) and 2.0 (both). The annual percentage change for incidence was 4.6 (95% confidence interval: 2.9-6.5) from 1992 to 2007. The 5-year and 10-year DM-specific survival rates from SEER 17 registries were 84.8 and 79.2%. The 5-year DM-specific survival rates by stage ranged from 90.9% (local) to 51.5% (distant). Independent predictors of mortality from DM included age, anatomic site, thickness, ulceration, lymph node and surgery.

Conclusions: The incidence of DM has been increasing steadily over the past 15 years. Older age, anatomic site of the head and neck, tumor thickness >2 mm, ulceration, lymph node involvement and non-receipt of surgery are associated with lower survival.

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