RESUMO
BACKGROUND: Hypopigmented mycosis fungoides (HMF) is an uncommon variant of mycosis fungoides. AIMS: To study the clinical and histopathology presentation in children with HMF. METHOD: We reviewed 9 children diagnosed with HMF. The clinical data were collected and analyzed. RESULT: Eight boys and 1 girl were included, with a median onset age of 7.4 year old and median age of diagnosis of 10.5 year old. Multiple hypopigmented patches were observed in all patients, and 5 patients exhibited multiple scaly erythema at the center of hypopigmented patches. Histopathology showed atypical lymphocytes with hyperchromatic, irregular, and cerebriform nuclei, infiltrated in the epidermis and dermis. Pautrier's microabscesses was noted in 6 of 9 patients, and papillary dermal fibroplasia was noted in 6 of 9 patients. CD8 predominance was detected in 4 of 6 patients. Four patients were simultaneously subjected to skin biopsy on hypopigmented patches and scaly erythema simultaneously. Compared with hypopigmented specimens, erythema biopsy detected deeper and denser infiltration of atypical lymphoid cells in 3 of 4 patients, higher CD4+/CD8+ ratio in 4 of 4 patients, more CD5 loss in 2 of 4 patients, and more CD7 loss in 2 of 4 patients. TCR gene monoclonal rearrangement was detected in 2 of 5 patients. Narrowband ultraviolet B phototherapy was applied in 7 patients. One of 7 patients achieved complete response, and 6 of 7 patients achieved partial response. No recurrence was noted with the median follow-up period of 6 months. CONCLUSION: HMF could occur in young patients, with indolent and benign course. HMF could gradually seem as scaly erythema based on hypopigmented patches. The histopathology indicated a more advanced stage of the scaly erythema lesions than hypopigmented patches.
Assuntos
Hipopigmentação/patologia , Micose Fungoide/patologia , Neoplasias Cutâneas/patologia , Pigmentação da Pele , Biomarcadores Tumorais/genética , Criança , Pré-Escolar , Feminino , Rearranjo Gênico do Linfócito T , Genes Codificadores dos Receptores de Linfócitos T , Humanos , Hipopigmentação/genética , Hipopigmentação/imunologia , Hipopigmentação/radioterapia , Linfócitos do Interstício Tumoral/imunologia , Masculino , Micose Fungoide/genética , Micose Fungoide/imunologia , Micose Fungoide/radioterapia , Neoplasias Cutâneas/genética , Neoplasias Cutâneas/imunologia , Neoplasias Cutâneas/radioterapia , Pigmentação da Pele/efeitos da radiação , Resultado do Tratamento , Terapia UltravioletaRESUMO
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.