ABSTRACT
BACKGROUND: Primary cutaneous lymphomas (PCL) are a group of extranodal non-Hodgkin lymphomas presenting with no evidence of extracutaneous disease at the time of diagnosis. Few longitudinal studies of PCL have been conducted, particularly in South American populations. Our objective was to describe the behavior of PCL and evaluate patient survival in a cohort of patients from southern Brazil. METHODS: We conducted a retrospective cohort study of all patients with a histopathological diagnosis of PCL receiving care at a tertiary referral center in southern Brazil from 2000 to 2012. Clinical, laboratory, and histopathological data, treatment variables, and survival curves were analyzed. RESULTS: Eighty-nine patients with PCL were included, with a mean age at diagnosis of 58.9 years. Cutaneous T-cell and natural killer (NK) cell lymphomas (CTCL/NKCL) accounted for 78.4% of cases and cutaneous B-cell lymphomas (CBCL) for 21.6%; 57.4% of patients with CTCL/NKCL and 50% of patients with CBCL were male. Half of all cases were mycosis fungoides (MF). The 5-year survival rate was 74.8% for MF, 61% for Sézary syndrome, 87.5% for primary cutaneous CD30+ lymphoproliferative disorders, and 88.9% for primary cutaneous follicle center lymphoma. CONCLUSIONS: In this cohort of patients with PCL from southern Brazil, CTCL/NKCL was more prevalent than CBCL, and the MF subtype was predominant. Survival curves were similar to those reported in the literature, except for Sézary syndrome, which had a better prognosis in this sample.
