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

Summary

Background

Primary cutaneous marginal zone B-cell lymphoma (PCMZL) has rarely been reported in patients younger than 20 years.

Objectives

To report our experience with PCMZL in the paediatric/adolescent age group.

Methods

Medical records of patients diagnosed with PCMZL before age 20 years and managed at two cutaneous lymphoma clinics in the United States and Israel in 1992-2015 were reviewed.

Results

The study group included 11 patients (6 girls) of median age 16 years (range 6–19.5); 10 had generalized/multifocal (T3) and 1 had regional/localized (T2) disease. Lesions were located on the limbs in all patients and the trunk in 6; 2 had facial lesions. Staging in all but one was based on whole-body computed tomography or positron emission tomography. Initial management in most patients included non-radiation modalities: 1 patient with localized disease received intralesional steroids; 6 patients with multifocal disease received the following: 3 - topical/intralesional steroids, 2 - excision, 1 - “watch and wait”. No extracutaneous progression was noted during a median follow-up of 5.5 years (mean 7.5, range 0.5-14). At present, 5/11 patients are in complete remission.

Conclusions

Based on our data (largest series in the literature with the longest follow up); the clinico-pathological presentation and course of PCMZL in the paediatric/adolescent age group are similar to 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.

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