Juvenile-onset mycosis fungoides progression not common, survival high: study

Juvenile-onset mycosis fungoides (jMF) is usually diagnosed during its early stage and has an excellent prognosis, with overall survival (OS) of 99 percent at 5 years and 98 percent at 10 years, reveals a study.

Researchers identified patients from a retrospective database and obtained clinical and histopathologic data from medical records. They assessed survival through contact by telemedicine.

Stage progression was defined as progression to a more advanced tumour, node, metastasis, and blood stage or death from disease.

Some 118 patients were diagnosed with jMF from 1987 to 2023. Of these, 115 (97 percent) had early-stage (IA-IIA) disease at diagnosis and three (3 percent) had advanced-stage (IIB-IVB) disease. Disease progression occurred in 15 patients (13 percent), with a median time to progression of 2 years.

The median follow-up time for survival assessment was 7.2 years. The OS rates at 5 and 10 years was 99 percent and 98 percent, respectively, with a 100-percent disease-specific survival rate for both periods. Notably, none of the patients succumbed to the disease.

“Disease progression is uncommon and did not lead to disease-specific death in our cohort,” the researchers said.

The study was limited by its single-centre retrospective design.

“Mycosis fungoides is the most common cutaneous T-cell lymphoma in the paediatric population,” according to the researchers.