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.
journal_name
J Am Acad Dermatoljournal_title
Journal of the American Academy of Dermatologyauthors
Boulos S,Vaid R,Aladily TN,Ivan DS,Talpur R,Duvic Mdoi
10.1016/j.jaad.2014.07.049subject
Has Abstractpub_date
2014-12-01 00:00:00pages
1117-26issue
6eissn
0190-9622issn
1097-6787pii
S0190-9622(14)01780-0journal_volume
71pub_type
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journal_title：Journal of the American Academy of Dermatology
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