Clinical characteristics and outcome of mycosis fungoides among children, adolescents, and young adults: Single institution study

• Published in: Journal of clinical oncology Vol. 39; no. 15_suppl; p. e19576
• Main Authors: Taylor, Eric, Zhang, Yumeng, Hooper, Madeline, Seminario-Vidal, Lucia, Sokol, Lubomir
• Format: Journal Article
• Language: English
• Published: 20.05.2021
• ISSN: 0732-183X; 1527-7755
• DOI: 10.1200/JCO.2021.39.15_suppl.e19576

Abstract only e19576 Background: Mycosis fungoides (MF) is the most common subtype of cutaneous T cell lymphoma (CTCL) [1]. MF is characterized by the skin infiltrate of small to medium size T lymphocytes with cerebriform nuclei. The incidence of MF increases significantly with age, with a median age at diagnosis of 60 years, and predominantly in white males [2]. The clinical outcome has not been evaluated among children, adolescents, and young adults. This study aimed to evaluate the characteristics and outcomes in this population. Methods: We retrospectively reviewed clinical data on 90 patients with MF at Moffitt Cancer Center between 2000 and 2020. Fifteen patients were diagnosed before the age of 20 years. Our cohort was compared to the largest US epidemiological report by Nath et al. using SEER database, which consists of 4892 patients from 1998-2008 [2]. The Chi-square test was used for comparison. Overall survival (OS) was calculated from diagnosis until death or last contact. OS between different groups was compared using the Kaplan-Meier curve. Hazard ratio and p-value were calculated using the log-rank test. Results: Of 90 patients, the median age at diagnosis was 32 years (range 6-39). The male to female ratio was 1.3:1. White and black races account for 57% and 27% of the patients. Compared to historical control, black patients were significantly higher in the current cohort (27% vs. 13%, p < 0.001). The difference widened in children and adolescents. More patients present with early stage MF in the current cohort (95.6% vs. 83%, p = 0.003). Clinical characteristics, including stage, LDH, WBC, ferritin level, were similar between children/adolescents and young adults. However, our cohort had significantly higher CD8 positive MF, and nearly half of children and adolescents were CD8 positive. Young adults had a higher rate of coexisting skin malignancy; however, this was not statistically significant due to small patient size. Time to diagnosis remained long, especially in children and adolescents’ group. However, the outcome is excellent. At a median follow-up of 4.9 years, four patients deceased, and the median OS was not reached. The prognosis remains poor in patients with advanced disease (HR = 39, p < 0.001) and large cell transformation (LCT) (HR = 8.5, p = 0.009). Conclusions: Compared to historical cohorts, higher proportion of black and female patients were noted in our young cohort with more early-stage and CD8 positive disease. This observation likely represents the different underlying biology of the disease [3]. Clinical outcome in young patients was excellent. However, patients with LCT or advanced stage carry a poor prognosis and need better therapies.