Mycosis fungoides (MF), the most common form of cutaneous T-cell lymphoma, typically presents in middle-aged to elderly individuals.
We sought to study the demographics, clinicopathologic features, treatment response, and prognosis of patients with biopsy-proven MF diagnosed before 20 years of age.
Patients were identified from a prospectively collected database for retrospective analysis.
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.
This is a single cancer center study.
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.Le texte complet de cet article est disponible en PDF.
Key words : cutaneous T-cell lymphoma, immunopathology, juvenile onset, mycosis fungoides, narrowband ultraviolet B radiation, presentation, treatment, vitamin-D deficiency
Abbreviations used : MF, NB, PUVA, TCR, UV
| Supported by the Sherry L. Anderson Research Fund.
| Conflicts of interest: None declared.
| Reprints not available from the authors.