Extracutaneous mycosis fungoides

Abstract

The clinical course of 40 patients with histologically documented extracutaneous mycosis fungoides (ECMF) was reviewed. Thirty one patients had documentation of nodal disease only (Stage IVA). Nine patients had histologic evidence of visceral involvement (Stage IVB). A wide variety of topical, regional and/or systemic therapies were used in the management of these patients. The median survival for the entire group was 14.5 months. Eleven patients (28%) obtained a complete response (CR) and had a median survival of 21 months as compared with 8 months among 29 patients not obtaining a CR. One patient is without evidence of disease (NED) at 10 years. Multiple prognostic factors were examined with respect to survival. Prognostic factors found to be significant at the P = 0.05 level included ECMF at presentation of skin disease (versus at the time of relapse), the ability to achieve a CR, and management incorporating the use of topical therapy, especially electron beam treatment. Tabulation of all trials of chemotherapy either at diagnosis of ECMF or subsequently revealed that cyclophosphamide, vincristine and prednisone (CVP) and cyclophosphamide, vincristine, prednisone, and bleomycin (COP-Bleo) were the most effective chemotherapeutic combinations. In a multivariate analysis with survival as the endpoint, the best model consisted of only two covariates: ECMF at presentation of skin disease, and the use of topical therapy. Other covariates found not to be significant at the P = 0.05 level included age, gender, clinical extent of cutaneous and extracutaneous disease, Stage IVA versus Stage IVB disease, the presence of Sezary cells in the peripheral blood smear, and management incorporating the use of systemic therapy.