Primary cutaneous anaplastic large-cell lymphoma: a review of the SEER database from 2005 to 2016

Introduction. Primary cutaneous anaplastic large-cell lymphoma (PC-ALCL) is a rare T-cell lymphoma. A prior analysis of the Surveillance, Epidemiology, and End Results (SEER) database reported only 157 cases of localized primary cutaneous CD30+ T-cell lymphoproliferative disorders (PC-ALCL and lymphomatoid papulosis) from 1973 to 2004. Our analysis of the SEER database since 2004 is the largest to date and our results improve our understanding of this disease and their potential prognostic factors. Methods. We used the SEER database to retrospectively identify patients. Survival was analysed using the Kaplan-Meier method, and log-rank tests were used to compare survival distributions. Results. There were 501 cases of PC-ALCL recorded from 2005 to 2016. Overall survival rates at 5 and 10 years were found to be 80.6% (95% CI 76.3%-84.3%) and 61.5% (95% CI 54.1%-68.1%) respectively. Age >= 60 years [hazard ratio (HR) = 1.09, P = 0.001 and use of chemotherapy (HR = 1.86, P = 0.01)] were associated with lower overall survival. In contrast to the 1973-2004 cohort, the head and neck site was not significantly associated with prognosis on multivariate analysis. Conclusion. PC-ALCL has been increasingly recognized over the past decade. Age > 60 years and use of chemotherapy are associated with a worse outcome. Contrary to prior studies, location was not associated with poor survival.