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WHO Classification of Tumours
Paediatric nodal marginal zone lymphoma
Tumours of haematopoietic and lymphoid tissues


Definition

Nodal MZL in the paediatric age have distinctive clinical and morphological characteristics
Click to access Pubmed
Taddesse-Heath L, Pittaluga S, Sorbara L, Bussey M, Raffeld M, Jaffe ES (2003)
Marginal zone B-cell lymphoma in children and young adults.
Am J Surg Pathol 27: 522-31



. They present predominantly in males (ratio 20:1) with asymptomatic and localized (90% stage I) disease, mainly in the head and neck lymph nodes. Histologically the tumour is similar to that seen in adults except that there are often progressively transformed germinal centres in which the outer border of the follicles is disrupted and infiltrated by neoplastic cells. The immunophenotype is similar to adult NMZL
Click to access Pubmed
Taddesse-Heath L, Pittaluga S, Sorbara L, Bussey M, Raffeld M, Jaffe ES (2003)
Marginal zone B-cell lymphoma in children and young adults.
Am J Surg Pathol 27: 522-31



. The differential diagnosis with atypical marginal zone hyperplasia with monotypic Ig expression may be difficult because the large cells in this condition also express CD43
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Attygalle AD, Liu H, Shirali S, Diss TC, Loddenkemper C, Stein H, Dogan A, Du MQ, Isaacson PG (2004)
Atypical marginal zone hyperplasia of mucosa-associated lymphoid tissue: a reactive condition of childhood showing immunoglobulin lambda light-chain restriction.
Blood 104: 3343-8



. Although this latter process has been reported in extranodal sites, some caution is advised as a similar process might also occur in the lymph nodes. Particularly for these reasons, studies for clonal rearrangements of the IGH@ chain are necessary to help distinguish paediatric NMZL from reactive conditions
Click to access Pubmed
Taddesse-Heath L, Pittaluga S, Sorbara L, Bussey M, Raffeld M, Jaffe ES (2003)
Marginal zone B-cell lymphoma in children and young adults.
Am J Surg Pathol 27: 522-31



. The prognosis of paediatric nodal MZL is excellent with a very low relapse rate and long survival after conservative treatment.

> Related Topics
Nodal marginal zone lymphoma
Introduction: Classification of lymphoid neoplasms