Mycosis fungoides in patients under 20 years of age: report of 7 cases, review of the literature and study of the clinical course

Dermatology. 1999;199(1):8-14. doi: 10.1159/000018196.

Abstract

Background: Mycosis fungoides (MF) is rare in young patients. Its clinical behavior is still uncertain, as some reports have suggested that it has a more aggressive course than does the adult-onset type.

Aim: To ascertain if early-onset MF represents a heterogeneous group of cutaneous T cell lymphomas.

Materials and methods: Clinical, immunohistopathological and follow-up data of early-onset (<20 years of age) MF cases reported in the literature (n = 42) plus 7 described herein were compared with those of a cohort of adult-onset MF patients (n = 252) diagnosed at our institution since 1975.

Results: The majority of the 49 early-onset MF patients had patch-plaque stage disease at diagnosis. Ten had hypopigmented lesions. The predominant phenotype was CD3+ CD4+CD7-CD8-. Seven patients had a stage progression, 6 with extracutaneous involvement. Five- and 10-year survival rates were 93 and 74%, respectively.

Conclusions: No statistically significant differences were found in the disease course between early- and adult-onset MF.

Publication types

  • Case Reports
  • Comparative Study
  • Review

MeSH terms

  • Adolescent
  • Adult
  • Age of Onset
  • Child
  • Female
  • Follow-Up Studies
  • Humans
  • Immunophenotyping
  • Lymphocytes / immunology
  • Male
  • Mycosis Fungoides / immunology
  • Mycosis Fungoides / pathology*
  • Neoplasm Staging
  • Skin Neoplasms / immunology
  • Skin Neoplasms / pathology*