To test the applicability and prognostic value of the new TNM classification system for primary cutaneous lymphomas other than mycosis fungoides and Sézary syndrome in patients with primary cutaneous anaplastic large cell lymphoma (C-ALCL) and to evaluate the prognostic significance of other clinical variables, in particular the site of presentation.
Retrospective cohort analysis.
Dutch Cutaneous Lymphoma Group database.
One hundred thirty-five patients with C-ALCL.
Main Outcome Measures
Clinical variables, including T category and site of presentation.
Eighty patients (59.3%) presented with T1 disease, 37 (27.4%) with T2 disease, and 18 (13.3%) with T3 disease. Median follow-up was 56 months (range, 11-288 months). Five-year disease-specific survival (DSS) was 93% for T1 disease, 93% for T2 disease, and 77% for T3 disease (P = .19). Patients with skin lesions on a leg had reduced 5-year DSS compared with lesions on other sites (82% for leg vs 95% for head and neck, 96% for trunk, and 95% for arm; P = .23). Patients with leg involvement (n = 32) had significantly worse 5-year DSS than did patients without leg involvement (n = 103; 76% vs 96%; P = .03 after adjustment for T category).
The new TNM system can be applied well to patients with C-ALCL and may provide prognostic information, in particular when combined with site of presentation. Patients with T2 or T3 disease with skin lesions on the leg may have reduced survival and require close surveillance during follow-up.