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Published on: October 14, 2021
Outcome in 34 patients with juvenile-onset mycosis fungoides: a clinical, immunophenotypic, and molecular study
E Mary Wain1, Guy E Orchard, Sean J Whittaker
1Skin Tumour Unit, St. John's Institute of Dermatology, St. Thomas' Hospital, London, United Kingdom. marywain@doctors.net.uk
Background:
Mycosis fungoides (MF) is predominantly a disease of older patients, but occasionally occurs in children. The aims of the current study were to describe the clinical presentation, pathologic features, and disease progression (DP) in patients who developed MF before age 16 years.
Methods:
A retrospective study was performed. Patients with juvenile-onset MF were identified from our databases. Clinical features were determined from the medical records and patient interviews. Histologic, immunohistochemical, and T-cell receptor (TCR) gene analysis was performed.
Results:
Thirty-four patients were identified: 50% had Stage IA disease, 47% had Stage IB disease, and 3% had Stage IIA disease. The male-to-female ratio was 2:1. Clinical features included hypopigmented lesions (24%), poikiloderma (26%), pilotropic disease (9%), and disease associated with lymphomatoid papulosis (18%). Twenty-eight patients had diagnostic histology, and six patients were included on the basis of compatible histology and a TCR clone in lesional skin. A cytotoxic immunophenotype was observed in 38%, including 71% of patients with hypopigmented lesions. Overall disease-specific survival (DSS) rates at 5 and 10 years were 95% and 93%, respectively. DP rates were 5% at 5 years and 29% at 10 years. Subgroup analysis demonstrated improved DSS and reduced DP in patients with Stage IA disease, those with hypopigmented or poikilodermatous lesions, and those with associated lymphomatoid papulosis.
Conclusions:
The prognosis for juvenile-onset MF is similar to that of adult-onset disease. There was an overrepresentation of a cytotoxic phenotype, which was most marked in hypopigmented variants. Widespread cutaneous disease (Stage IB) indicated a less favorable outcome.
Insights
Juvenile mycosis fungoides (MF) in children has a similar prognosis to adult MF. Early-stage disease and specific lesion types are associated with better outcomes, while widespread skin involvement indicates a less favorable prognosis.
Area of Science:
- Dermatology
- Pediatric Oncology
- Cutaneous Lymphoma Research
Background:
- Mycosis fungoides (MF) is a rare condition in children, typically affecting older adults.
- Understanding juvenile-onset MF is crucial for accurate diagnosis and management in pediatric patients.
Purpose of the Study:
- To characterize the clinical presentation, pathological findings, and disease progression of MF in patients under 16 years old.
- To compare outcomes of juvenile MF with adult-onset disease.
Main Methods:
- Retrospective analysis of 34 patients with juvenile-onset MF.
- Review of clinical records, patient interviews, and histopathological, immunohistochemical, and T-cell receptor (TCR) gene analyses.
Main Results:
- Most patients presented with early-stage disease (IA or IB).
- Common clinical features included hypopigmented lesions and poikiloderma; 18% had associated lymphomatoid papulosis.
- Disease-specific survival rates were high (95% at 5 years, 93% at 10 years), with lower progression rates in early-stage disease and specific lesion types.
Conclusions:
- Juvenile-onset MF prognosis mirrors that of adult-onset MF.
- A cytotoxic immunophenotype was more common in hypopigmented variants.
- Widespread cutaneous disease (Stage IB) correlated with a less favorable outcome.
