Long-term outcomes in juvenile-onset mycosis fungoides.

BACKGROUND

Mycosis fungoides is the most common cutaneous T-cell lymphoma in the pediatric population.

OBJECTIVE

We aimed to characterize the long-term outcomes associated with juvenile-onset mycosis fungoides (jMF).

METHODS

Patients were identified from a retrospective database. Clinical and histopathologic data were obtained from medical records, and survival was assessed through contact by telemedicine. Stage progression was defined as progression to a more advanced tumor, node, metastasis, and blood stage or death from disease.

RESULTS

From 1987 to 2023, 118 patients were diagnosed with jMF: 97% of patients (115/118) had early-stage (IA-IIA) disease at diagnosis and 3% (3/118) had advanced-stage (IIB-IVB) disease. Thirteen percent of patients (15/118 patients) developed disease progression with a median time to progression of 2 years (range: 2 months to 13 years). Median follow-up time for survival assessment was 7.2 years (range: 2 months to 32 years). Overall, 5-year and 10-year survival rates were 99% and 98%, respectively, and 5-year and 10-year disease-specific survival rates were 100%. No patients died of disease.

LIMITATIONS: This is a single-center retrospective study.

CONCLUSION

jMF often presents with early-stage disease and has an excellent prognosis with a 5-year and 10-year overall survival of 99% and of 98%, respectively. Disease progression is uncommon and did not lead to disease-specific death in our cohort.