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 cutaneous lymphoma to ensure accurate diagnosis and correct management.
Financial & competing interests disclosure
The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.
No writing assistance was utilized in the production of this manuscript.
Key issues
• Primary cutaneous lymphomas are probably underdiagnosed in children due similarities with common inflammatory dermatoses and infrequent skin biopsies.
• Most cases of pediatric skin lymphoma are early stage mycosis fungoides (MF) which rarely develop to advanced disease in childhood but requires follow up into adulthood and disease progression may occur in later life.
• Multiple skin biopsies may be required and must be sent for hematoxylin and eosin, immunohistochemistry and T-cell clonality studies.
• Children with suspected cutaneous lymphoma should be discussed by a supraregional multi-disciplinary panel to establish diagnosis and formulate management plan. Follow-up in pediatrics is appropriate.
• The hypopigmented form of MF is more common in children.
• Early stage MF is managed with skin directed therapy and late stage requires systemic treatment.
• Erythrodermic MF and Sézary syndrome must be considered in the differential diagnosis of children with erythroderma that fail to respond to conventional treatment of eczema or psoriasis.
• Lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma must be differentiated from systemic CD30+ve lymphoma and do not require systemic chemotherapy. Systemic anaplastic large cell lymphoma must be excluded by detailed work up, careful clinicopathological correlation and discussion at supraregional skin lymphoma multi-disciplinary team.
• Primary cutaneous marginal zone B-cell lymphoma and follicle cell lymphoma are indolent forms of cutaneous B-cell lymphoma with a good prognosis. They can be managed by expectant therapy, surgical excision, radiotherapy, intralesional steroids or rituximab.
• Primary cutaneous diffuse large B-cell lymphoma requires chemotherapy due to its aggressive clinical course but is exceedingly rare in children.