Clinical Outcome and Prognosis of Young Patients with Mycosis Fungoides

Abstract

Background Mycosis fungoides (MF) in young patients is rare and may have atypical presentations. There are limited data in these patients. Objective Determine the clinical outcome and prognosis of young patients with MF. Methods A search of our institutional cancer registry database was conducted for patients diagnosed with MF at ≤ 30 years of age. Results Our study includes 74 patients (median age at diagnosis= 25.5 years). Most patients (n=65, 88%) presented with early stage disease and variants of MF (n=44, 59%) leading to a median delay in diagnosis of 2.5 years. Hypopigmented MF (n=27, 36.5%) was the most common variant affecting predominantly African-American (44.4% vs.19%, p= 0.02) and younger age group (20 vs. 26 years, p<0.001). All patients with hypopigmented MF presented with early stage disease and were less likely to develop progressive disease (PD) compared to other variants (11% vs. 34%, p= 0.03). Overall, nineteen patients (26%) developed PD during median follow-up of 3.5 years which was associated with advanced stage disease (89% vs. 17%, p<0.0001), older age (>20 years) [31% vs. 13%, p=0.08 (NS)], African-American race (52.6% vs. 20%, p= 0.0090) and poikilodermatous presentation (p<0.01). The overall survival was favorable (97.2% and 95.9% at 5 and 10 years) despite delay in diagnosis and atypical presentation. Conclusions PD 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.

The evaluation of variants, subtypes and atypical forms of mycosis fungoides in a tertiary hospital

“…MF is the most common primary cutaneous T-cell lymphoma and its annual incidence is ranged from 0.13 to 0.9 cases per 100.000 people 7 . MF usually presents in the fifth and sixth decades of life and the disease shows male predominance [5][6][7][8] . In classic MF, the patients usually present with slightly erythematous, scaly patches, plaques and tumors located on non-sun exposed areas and clinical features may resemble various inflammatory dermatoses such as atopic dermatitis and psoriasis 2,6,8,9 . In literature, there are studies describing the clinical and epidemiological features of MF patients in countries such as the United States, Kuwait and Iran 7,8,10,11 .…”

“…These variants and subtypes can also be referred to as atypical forms. Clinical variants, subtypes and early MF lesions may mimic other benign dermatoses which might lead to delay in the diagnosis and treatment 5,6 . In our study we aimed to evaluate the demographic, clinical and histopathological features of patients with classical and variants, subtypes or atypical forms of MF in our dermatology department.…”

It looks like childhood eczema but is it?

“…This is a rare disease most commonly seen in older adults, however, may very rarely occur in children and adolescents . The clinical picture may have an appearance similar to psoriasis or nummular eczema in the early stages (Table ).…”

Folliculotropic mycosis fungoides in a pediatric patient with response to psoralen‐ultraviolet A phototherapy

“…In the young population, the hypopigmented variant is a common presentation with an indolent prognosis, but the poikilodermatous one is considered of worse prognosis, as it is the folliculotropic variant. 2 A recent study suggests that the plaque stage of FMF could be differentiated in two stages depending on the cellular infiltra- results than with other therapies. [5][6][7] As far as prognosis is concerned, it is variable, and generally, the cutaneous limited disease tends to wax and wane over decades.…”