[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.