Pooja Virmani1, Laura Levin2, Patricia L Myskowski1,2, Eileen Flores1, Michael A Marchetti1,2, Anna Skripnik Lucas1, Melissa Pulitzer3, Steven Horwitz4, Tanya Trippett5, Alison Moskowitz4, Christiane Querfeld1,6,7. 1. Department of Dermatology, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, New York. 2. Department of Dermatology, Weill Cornell Medical College, New York, New York. 3. Dermatopathology Service, Department of Pathology, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, New York. 4. Lymphoma Services, Department of Medicine, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, New York. 5. Department of Pediatrics, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, New York. 6. Department of Pathology, City of Hope Comprehensive Cancer Center, Duarte, California. 7. Division of Dermatology, City of Hope Comprehensive Cancer Center, Duarte, California.
Abstract
BACKGROUND/ OBJECTIVES: 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. METHODS: A search of our institutional cancer registry database was conducted for patients diagnosed with MF at younger than 30 years of age. RESULTS: Our study included 74 patients (median age at diagnosis 25.5 yrs). Sixty-five (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 (20 vs 26 yrs; 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 3.5 years, which was associated with advanced-stage disease (89% vs 17%; p < 0.001), older age (>20 yrs) (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 5 yrs, 95.9% at 10 yrs) despite the delay in diagnosis and atypical presentation. CONCLUSIONS: 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.
BACKGROUND/ OBJECTIVES: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. METHODS: A search of our institutional cancer registry database was conducted for patients diagnosed with MF at younger than 30 years of age. RESULTS: Our study included 74 patients (median age at diagnosis 25.5 yrs). Sixty-five (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 (20 vs 26 yrs; 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 3.5 years, which was associated with advanced-stage disease (89% vs 17%; p < 0.001), older age (>20 yrs) (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 5 yrs, 95.9% at 10 yrs) despite the delay in diagnosis and atypical presentation. CONCLUSIONS: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.
Authors: Pamela B Allen; Subir Goyal; Tim Niyogusaba; Colin O'Leary; Amy Ayers; Erica S Tarabadkar; Mohammad K Khan; Mary Jo Lechowicz Journal: JAMA Dermatol Date: 2022-09-07 Impact factor: 11.816