Juvenile onset of primary low-grade cutaneous B-cell lymphoma

Juvenile onset of primary low-grade cutaneous B-cell lymphoma
Br J Dermatol. 2009 Mar 26

Amitay-Laish I, Feinmesser M, Ben-Amitai D, David M, Manor Y, Kidron D, Barzilai A, Hodak E.
Department of Dermatology, Rabin Medical Center, Beilinson Hospital, Petah Tikva 49100, Israel.

Summary Background: Cutaneous lymphomas rarely occur in children and adolescents, and are mostly of the T-cell lineage. Low-grade primary cutaneous B-cell lymphoma (CBCL) is extremely rare in individuals under 18 years old. Only 11 patients under 20 years old have been reported in the literature.

Objectives: To evaluate the number of patients younger than 18 years with primary CBCL diagnosed at our centre and to investigate its clinicopathological features, treatment and course in this age group.

Methods: We reviewed the files of all 90 patients with primary CBCL who attended the Department of Dermatology of our tertiary care university-affiliated centre from 1992 to 2007.

Results: Four patients who met study criteria were identified: three girls and one boy. Mean age at diagnosis was 16.6 years (range 16-17). Three patients had cutaneous marginal zone lymphoma (CMZL), and one had a spindle-cell (sarcomatoid) lymphoma, most probably follicular centre cell type. All were treated with the standard regimen used in adults. The mean duration of follow up was 45 months. No extracutaneous progression was noted. At present two of the four patients are in complete clinical remission.

Conclusions: In Israel, primary CBCL apparently occurs more often in young patients than reported in the literature. CMZL is the most frequent type. Long follow up is mandatory to assess the biological behaviour of CBCL in the paediatric/adolescent age group.

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