Clinical Outcome and Prognosis of Young Patients with Mycosis Fungoides.

Abstract

Mycosis fungoides (MF) in young patients is rare and may have atypical presentations. There are limited data in these patients. The objective was to determine the clinical outcome and prognosis of young patients with MF. A search of our institutional cancer registry database was conducted for patients diagnosed with MF at younger than 30years of age. Our study included 74 patients (median age at diagnosis 25.5yrs). Sixty-five (88%) presented with early stage disease and variants of MF (n=44 [59%]), leading to a median delay in diagnosis of 2.5years. Hypopigmented MF (n=27 [36.5%]) was the most common variant, affecting predominantly African American (44.4% vs 19%; p=0.02) and younger (20 vs 26yrs; p<0.001) patients. All patients with hypopigmented MF presented with early stage disease and were less likely to develop progressive disease (PD) than those with other variants (11% vs 34%; p=0.03). Nineteen patients (26%) developed PD during a median follow-up of 3.5years, which was associated with advanced-stage disease (89% vs 17%; p<0.001), older age (>20yrs) (31% vs 13%; p=0.08), African American race (52.6% vs 20%; p=0.009), and poikilodermatous presentation (p<0.01). Overall survival was good (97.2% at 5yrs, 95.9% at 10yrs) despite the delay in diagnosis and atypical presentation. Progressive disease is associated with older age, African American race, the poikilodermatous variant, and advanced-stage disease. The hypopigmented variant is a common presentation in young patients and has an indolent disease course. Our study confirms an overall favorable prognosis in young patients with MF.