[Primary centrofollicular cutaneous cell lymphoma. Clinical description, treatment results, and follow up of 21 cases].

Background: The goal of this study was to describe the clinical, biological, features and the outcome of 21 patients with primary cutaneous centro-follicular lymphoma (PCL-CF).

Patients And Methods: A group of 21 consecutive patients with PCL-CF (median age 56 years) presenting with single (11/21) or multiple (10/21) cutaneous lesions observed between January 1980 and June 2000 were described. The histologic features, treatment modalities and outcome were shown.

Results: The patients mainly presented with cutaneous lesions on the head and trunk (19/21). Histological findings were grade I, II and III (REAL/WHO classification) in 4, 8 and 9 patients respectively. Pattern was follicular (12/21) and diffuse (9/21). Treatment of PCL-CF consisted in restricted field radiotherapy (RT), chemotherapy (CHT) and combined modalities (CM) in 11, 5 and 5 cases respectively. The response to treatment was: 17 complete responses, CR (13 RT, 2CHT, 2CM), 3 partial responses, PR (1 CHT, 2 CM) and 1 non response, NR (1CHT). The relapse rate was 23.8% with a median follow-up of 48 months; the estimated 4 years event free survival (EFS) and overall survival (OS) were 68% and 90% respectively.

Discussion And Conclusions: Our experience demonstrates that PCL-CF are lymphoproliferative disorders with a good prognosis after adequate therapy and that the histologic features did not influence significantly both response and survival.