Virmani Pooja, Levin Laura, Myskowski Patricia L, Flores Eileen, Marchetti Michael A, Lucas Anna Skripnik, Pulitzer Melissa, Horwitz Steven, Trippett Tanya, Moskowitz Alison, Querfeld Christiane
Department of Dermatology, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, New York.
Department of Dermatology, Weill Cornell Medical College, New York, New York.
Pediatr Dermatol. 2017 Sep;34(5):547-553. doi: 10.1111/pde.13226. Epub 2017 Aug 14.
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.
A search of our institutional cancer registry database was conducted for patients diagnosed with MF at younger than 30 years of age.
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.
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.
背景/目的：年轻患者的蕈样肉芽肿（MF）较为罕见，可能有非典型表现。针对这些患者的数据有限。本研究目的是确定年轻MF患者的临床结局和预后。
检索我院机构癌症登记数据库中诊断为MF且年龄小于30岁的患者。
我们的研究纳入了74例患者（诊断时中位年龄25.5岁）。65例（88%）为早期疾病及MF变异型（n = 44 [59%]），导致诊断中位延迟2.5年。色素减退性MF（n = 27 [36.5%]）是最常见的变异型，主要影响非裔美国人（44.4%对19%；p = 0.02）和更年轻的患者（20岁对26岁；p < 0.001）。所有色素减退性MF患者均为早期疾病，与其他变异型患者相比，发生疾病进展（PD）的可能性更小（11%对34%；p = 0.03）。19例患者（26%）在中位随访3.5年期间发生PD，这与晚期疾病（89%对17%；p < 0.001）、年龄较大（>20岁）（31%对13%；p = 0.08）、非裔美国人种族（52.6%对20%；p = 0.009）及皮肤异色症表现（p < 0.01）相关。尽管存在诊断延迟和非典型表现，但总体生存率良好（5年时为97.2%，10年时为95.9%）。
疾病进展与年龄较大、非裔美国人种族、皮肤异色症变异型及晚期疾病相关。色素减退性变异型在年轻患者中是常见表现，疾病进程较为惰性。我们的研究证实年轻MF患者总体预后良好。
