Clinical presentation, immunopathology, and treatment of juvenile-onset mycosis fungoides: a case series of 34 patients

J Am Acad Dermatol. 2014 Dec;71(6):1117-26. doi: 10.1016/j.jaad.2014.07.049. Epub 2014 Sep 26.

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.

Limitations: This is a single cancer center study.

Conclusion: 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.

Keywords: cutaneous T-cell lymphoma; immunopathology; juvenile onset; mycosis fungoides; narrowband ultraviolet B radiation; presentation; treatment; vitamin-D deficiency.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Age of Onset
  • Biopsy
  • Child
  • Child, Preschool
  • Cross-Sectional Studies
  • Databases, Factual
  • Female
  • Humans
  • Hyperpigmentation / immunology
  • Hyperpigmentation / pathology
  • Hypopigmentation / immunology
  • Hypopigmentation / pathology
  • Immunophenotyping
  • Male
  • Mycosis Fungoides* / drug therapy
  • Mycosis Fungoides* / immunology
  • Mycosis Fungoides* / pathology
  • PUVA Therapy / methods*
  • Prognosis
  • Retrospective Studies
  • Skin / pathology
  • Skin Neoplasms* / drug therapy
  • Skin Neoplasms* / immunology
  • Skin Neoplasms* / pathology
  • Treatment Outcome
  • Ultraviolet Therapy / methods
  • Vitamin D Deficiency / immunology
  • Vitamin D Deficiency / pathology
  • Young Adult