Pediatric cutaneous lymphomas: rare diseases requiring expert diagnosis and management

Abstract

Mycosis fungoides and lymphomatoid papulosis are the most prevalent pediatric cutaneous lymphoma. Children present with early stage mycosis fungoides and progression to late stage is rare. Skin directed therapy is appropriate. Lymphomatoid papulosis is benign but the histological infiltrate is similar to aggressive systemic T-lymphoma. A history of spontaneously remitting tumors is essential for correct diagnosis and to prevent treatment as systemic lymphoma. Expectant therapy is appropriate in children. There is an association with development of systemic lymphoma in up to 10% with lymphomatoid papulosis and follow-up is required into adulthood. Cutaneous B-cell lymphomas (CBCL) are rare in children. Marginal zone lymphoma is most frequent. It presents with erythematous dermal nodules. Systemic spread is exceptional. All other types of primary cutaneous lymphoma are exceedingly rare in children. It is vital that children are discussed at supraregional multi-disciplinary team with an expertise in cutaneou...