Total skin electron radiation for patients with erythrodermic cutaneous T-cell lymphoma (Mycosis fungoides and the Sezary syndrome)

BACKGROUND. There is limited published evidence regarding the efficacy of t otal skin electron beam radiation for patients with the diffuse erythroderm ic form of mycosis fungoides. METHODS, Forty-five patients with erythrodermic mycosis fungoides were mana ged at McMaster University in Hamilton, Ontario, Canada (n = 34), and at Ya le University (n = 11) between 1970 and 1996. All received radiation withou t neoadjuvant, concomitant, or adjuvant therapies. The median age was 67 ye ars (range, 42-84 years). The male-to-female ratio was 2.2. Fifteen receive d radiation for the treatment of newly diagnosed disease. There were 28 wit h Stage III (T4 N0-1 MO), 13 with Stage IVA (T4 N2-3 MO), and 4 with Stage IVB (T4 N0-3 M1) disease, and 21 had blood involvement. The median radiatio n dose was 32 gray (Gy) (range, 4.8-40 Gy). The median treatment time was 2 1 days (range, 3-125 days). A technically more intense method of radiation (32-40 Gy and 4-6 MeV electrons) was administered to 23 patients. RESULTS. All patients responded. The rate of complete cutaneous remission w as 60%, with 26% remaining progression free at 5 years. Remission was assoc iated with more intense radiation (P = 0.014 in multivariate analysis with adjustment for blood and staging information). With the more intense radiat ion, 74% attained remission, with 36% remaining progression free at 5 years . For 8 patients with Stage In disease without blood involvement, all enter ed remission, with 69% remaining progression free at 5 years. Twenty of 30 deaths were related to mycosis fungoides. The median overall survival was 3 .4 years, with a 10-year estimate of 28%. The median cause specific surviva l was 5 years, with a 10-year estimate of 43%. Both overall and cause speci fic survival were associated with an absence of blood involvement (both P < 0.03 in multivariate analysis). Age was not a significant factor. Toxiciti es of radiation were acceptable when radiation was administered over 6-9 we eks at 5 fractions per week. CONCLUSIONS. Total skin radiation is an efficient monotherapy for patients with erythrodermic mycosis fungoides. With more intense radiation, the rate of cutaneous remission is 74%, and 27% remain progression free at 10 years . Radiation may be most efficacious in Stage III, with no blood involvement . When there is blood, lymph node, or visceral involvement, combined modali ty therapies should be explored. (C) 1999 American Cancer Society.