Cutaneous peripheral T-cell lymphoma, not otherwise specified: A single-center prognostic analysis.

Background: Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) is a rare and aggressive disease either originating in or secondarily involving the skin.

Objective: We sought to assess clinical, histopathologic, and prognostic features of patients with cutaneous PTCL-NOS.

Methods: This was a retrospective chart review of patients with cutaneous PTCL-NOS between 1993 and 2013.

Results: Thirty patients with PTCL-NOS were included. Fourteen had skin-only disease and 15 had concurrent skin and systemic disease at presentation. In primary cutaneous PTCL-NOS, the overall survival rate at 5 years was 61% (95% confidence interval, 37-100%; number still at risk, 2). The median overall survival was 5.6 years. Patients were diagnosed a median of 2.4 months from symptom onset. Patients with concurrent disease died a median of 2.1 years after diagnosis. The estimated overall survival rate at 5 years after diagnosis was 29% (95% confidence interval, 13-67%; number at risk, 3). The median overall survival was 3.9 years. Patients were diagnosed a median of 6 months from symptom onset, with a 53% increased risk of death for each year from symptom onset to diagnosis.

Limitations: This was a retrospective study with a limited number of cases.

Conclusions: Age at diagnosis and B-symptoms predict poor survival in patients with cutaneous PTCL-NOS. In addition, poorer survival is observed in patients with multifocal lesions and concomitant skin and systemic PTCL-NOS.