Paediatric primary cutaneous marginal zone B-cell lymphoma: does it differ from its adult counterpart?

Abstract

Based on our data (largest series in the literature with the longest follow-up), the clinicopathological presentation and course of PCMZL in the paediatric/adolescent age group are similar to those in adults. Given the indolent course and the long life expectancy of these young patients, the cumulative risk of imaging studies and the age-related potential toxicity of treatment, especially radiation, should be taken into consideration.

Cutaneous lymphomas in childhood and adolescence: much needed new data

“…The study by Amitay‐Laish et al . in this issue of the BJD contributes significant new data to increase our knowledge on primary cutaneous marginal zone lymphoma (PCMZL) in children . In accordance with previous data on smaller case series of PCMZL in children and adolescents, their data corroborate that PCMZL affects mostly teenagers of both sexes and preferentially manifests with multifocal tumour lesions and shows identical histological features to those in adults .…”

“…In accordance with previous data on smaller case series of PCMZL in children and adolescents, their data corroborate that PCMZL affects mostly teenagers of both sexes and preferentially manifests with multifocal tumour lesions and shows identical histological features to those in adults . Of utmost importance is the observation that no extracutaneous progression occurred during a median follow‐up of 5·5 years in this series as well as in other reports . Thus, the course of PCMZL in children seems to be indolent, but characterized by disease persistence; only half of the patients experienced complete remission.…”

“…Finally, a ‘watch and wait’ strategy may be justified in some patients, as some lesions can undergo spontaneous regression. In conclusion, although this study by Amitay‐Laish and co‐workers is timely and most welcome, more international multicentre studies on cutaneous lymphoma and other rare skin neoplasms in children are needed to clarify their course and to optimize their management.…”

“…It is especially true in children that treatment modalities such as radiation or chemotherapy may result in long‐term sequelae; these include an increased risk for second neoplasms due to chemotherapy‐linked immunodeficiency and radiation‐induced skin tumours. Given the excellent prognosis of PCMZL also in children, tumour excision, topical or intralesional steroids or interferon alpha represent safe and effective therapeutic options . PCMZL in children and adults can be associated with Borrelia burgdorferi sp.…”

Diagnosis and Treatment of Primary Cutaneous B-Cell Lymphomas: State of the Art and Perspectives

“…The five-year survival rate is around 99% [ 2 ]. It typically affects medium-aged adults although pediatric cases have also been reported [ 7 ].…”