title: Clinical presentation, immunopathology, and treatment of juvenile-onset mycosis fungoides: a case series of 34 patients.
abstract: BACKGROUND
  Mycosis fungoides (MF), the most common form of cutaneous T-cell lymphoma, typically presents in middle-aged to elderly individuals.
  
  
  OBJECTIVE
  We sought to study the demographics, clinicopathologic features, treatment response, and prognosis of patients with biopsy-proven MF diagnosed before 20 years of age.
  
  
  METHODS
  Patients were identified from a prospectively collected database for retrospective analysis.
  
  
  RESULTS
  Of 1902 patients with MF, 34 had juvenile-onset MF: 41% were stage IA, 56% were stage IB, and 3% were stage IIB at diagnosis. The male to female ratio was 1.1:1. The median age of symptom onset was 9 years (range 3-19 years), with a delay in diagnosis between 1 month and 14 years. Patients primarily presented with hypopigmented (53%), hyperpigmented (29%), and pink-violaceous (41%) patches/plaques. Immunohistochemistry revealed 39% with CD8(+) immunophenotype, 67% of which had hypopigmented lesions. The phototherapy response rate in 21 patients was 81%. All patients who completely responded to narrowband ultraviolet B phototherapy had hypopigmented MF.
  
  
  CONCLUSIONS
  This is a single cancer center study.
  
  
  CONCLUSIONS
  Juvenile-onset MF presents with early-stage disease with an overrepresentation of hypopigmented MF and CD8(+) immunophenotype. Narrowband ultraviolet B is an effective treatment option for juveniles, especially for those with the hypopigmented variant.
authors.affiliation: Department of Dermatology, University of Texas MD Anderson Cancer Center, Houston, Texas; Department of Dermatology, Wright State University Boonshoft School of Medicine, Dayton, Ohio.
authors.forename: Scarlett
authors.initials: S
authors.lastname: Boulos
authors.affiliation: Department of Dermatology, University of Texas MD Anderson Cancer Center, Houston, Texas.
authors.forename: Reena
authors.initials: R
authors.lastname: Vaid
authors.affiliation: Department of Hematopathology, University of Texas MD Anderson Cancer Center, Houston, Texas.
authors.forename: Tariq N
authors.initials: TN
authors.lastname: Aladily
authors.affiliation: Department of Dermatopathology, University of Texas MD Anderson Cancer Center, Houston, Texas.
authors.forename: Doina S
authors.initials: DS
authors.lastname: Ivan
authors.affiliation: Department of Dermatology, University of Texas MD Anderson Cancer Center, Houston, Texas.
authors.forename: Rakhshandra
authors.initials: R
authors.lastname: Talpur
authors.affiliation: Department of Dermatology, University of Texas MD Anderson Cancer Center, Houston, Texas. Electronic address: mduvic@mdanderson.org.
authors.forename: Madeleine
authors.initials: M
authors.lastname: Duvic
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D016208:Databases, Factual; D005260:Female; D006801:Humans; D017495:Hyperpigmentation / Q000276:immunology / Q000473:pathology; D017496:Hypopigmentation / Q000276:immunology / Q000473:pathology; D016130:Immunophenotyping; D008297:Male; D009182:Mycosis Fungoides / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D011701:PUVA Therapy / Q000379:methods*; D011379:Prognosis; D012189:Retrospective Studies; D012867:Skin / Q000473:pathology; D012878:Skin Neoplasms / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D016896:Treatment Outcome; D014467:Ultraviolet Therapy / Q000379:methods; D014808:Vitamin D Deficiency / Q000276:immunology / Q000473:pathology; D055815:Young Adult
keywords: cutaneous T-cell lymphoma; immunopathology; juvenile onset; mycosis fungoides; narrowband ultraviolet B radiation; presentation; treatment; vitamin-D deficiency