Clinical characteristics and outcome of patients with extracutaneous mycosis fungoides

Purpose: To identify prognostic factors predictive of outcome in patients w ith extracutaneous (stage IV) mycosis fungoides (588) and to evaluate the r isk of progression to extracutaneous disease by initial extent of skin invo lvement, Patients and Methods: One hundred twelve patients with extracutaneous disea se at presentation or with progression and 434 patients with initial cutane ous-only disease were identified. Actuarial survival curves were plotted ac cording to the Kapian-Meier technique. Results: The median survival of all stage IV patients was 13 months from th e date of first treatment for stage IV disease, Sex, race, age, extent of s kin involvement, and peripheral blood Sezary cell involvement were not sign ificant to survival outcome, Eleven patients (10%) had a complete response to therapy resulting in a significantly improved median survival compared w ith patients with a partial or no response (1.70 v 0.91 years, P = .047 and 1.70 v 0.57 years, P = .011, respectively), At 20 years from diagnosis, th e risk for progression to extracutaneous disease by Initial extent of skin involvement was 0% for limited patch/plaque, 10% for generalized patch/plaq ue, 35.5% for tumorous disease, and 41% for erythrodermic involvement. Conclusion: This was a larger scale study over ct longer time period than h ad been completed previously on extracutaneous MF. Prognostic factors impor tant in the cutaneous stages of disease are no longer significant once extr acutaneous disease develops, Patients who had a more favorable response to therapy may have had a biologically less aggressive disease than their less fortunate counterparts, The risk of developing stage IV MF is highest in p atients presenting with tumorous or erythrodermic skin disease and is lowes t in patients with limited skin involvement. (C) 2001 by American Society o f Clinical Oncology.