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Paediatric primary cutaneous marginal zone B‐cell lymphoma: does it differ from its adult counterpart?
Author(s) -
AmitayLaish I.,
Tavallaee M.,
Kim J.,
Hoppe R.T.,
Million L.,
Feinmesser M.,
Fenig E.,
Wolfe M.E.L.,
Hodak E.,
Kim Y.H.
Publication year - 2017
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/bjd.14932
Subject(s) - medicine , lymphoma , cutaneous lymphoma , young adult , trunk , localized disease , marginal zone , radiation therapy , surgery , pediatrics , mycosis fungoides , cancer , b cell , ecology , prostate cancer , biology , antibody , immunology
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 U.S.A. and Israel from 1992 to 2015 were reviewed. Results The study group included 11 patients (six girls; median age 16 years, range 6–19·5); 10 had generalized/multifocal (T3) and one had regional/localized (T2) disease. Lesions were located on the limbs in all patients and the trunk in six; two 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 nonradiation modalities: one patient with localized disease received intralesional steroids; six patients with multifocal disease received the following: topical/intralesional steroids ( n = 3); excision ( n = 2); ‘watch and wait’ ( n = 1). No extracutaneous progression was noted during a median follow‐up of 5·5 years (mean 7·5, range 0·5–14). At present, five patients are in complete remission. Conclusions 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.