ORIGINAL ARTICLESMarginal Zone B-Cell Lymphoma in Children and Young AdultsTaddesse-Heath, Lekidelu M.D.; Pittaluga, Stefania M.D.; Sorbara, Lynn Ph.D.; Bussey, Mary; Raffeld, Mark M.D.; Jaffe, Elaine S. M.D.Author Information From the Hematopathology Section, Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, Bethesda, Maryland, U.S.A. Presented in part at the 90th Annual Meeting of the U.S. and Canadian Academy of Pathology, March 2000, Atlanta, Georgia. Address correspondence and reprint requests to Elaine S. Jaffe, MD, Laboratory of Pathology, National Cancer Institute/National Institutes of Health, Building 10, Room 2N202, 10 Center Drive, MSC 1500, Bethesda, MD 20892, U.S.A.; e-mail: [email protected] The American Journal of Surgical Pathology: April 2003 - Volume 27 - Issue 4 - p 522-531 Buy Abstract We describe the clinicopathologic findings of 48 cases of marginal zone B-cell lymphoma (MZL) in children and young adults, a disease that has been recognized previously only rarely in this age group. Patients ranged in age from 2 to 29 years, with pediatric patients (≤18 years) comprising 52% of the cases. As in adults, both primary nodal (N) and extranodal (E) MZL were observed. However, primary NMZL comprised the majority of the cases (67%) and demonstrated distinctive clinical and histologic features. NMZL occurred most commonly in young males (median 16 years, male/female ratio 5.4:1), with no underlying disease, presenting as localized adenopathy (90% stage I), with excellent prognosis and low rate of recurrence. In contrast, EMZL were much less common, and patients were older (median 24.5 years), with only a slight male predominance (male/female ratio 1.2:1). Most patients had localized disease (73% stage I) with excellent prognosis and infrequent recurrences. In addition, an association with autoimmune disease was observed in 19% of the EMZL. Both primary NMZL and EMZL in young patients shared similar morphologic and immunophenotypic findings to those described in adults and were monoclonal B-cell proliferations with monoclonality demonstrated in 94% of the cases. A common morphologic feature in NMZL was disruption of residual follicles resembling progressive transformation of germinal centers (PTGC), observed in 66% of the cases. Although the precise relationship of primary NMZL and the PTGC-like changes is unclear, it is possible that NMZL arises in a background of PTGC, as florid PTGC often occurs in young males. We conclude that EMZL in children and young adults are similar to EMZL of mucosa-associated lymphoma tissue occurring in older patients. However, pediatric NMZL appear to have distinctive clinical and histologic features. © 2003 Lippincott Williams & Wilkins, Inc.