Clinicopathologic analysis of follicular lymphoma occurring in children.
Follicular lymphoma is a rare lymphoid malignancy in pediatric patients and consequently remains poorly characterized, particularly with respect to its immunophenotype and molecular pathogenesis. A total of 23 pediatric patients with follicular lymphoma were identified, with a median age of 11 years and a male-to-female ratio of 2.3:1. Of the 19 patients for whom presenting clinical features were available, 15 patients had stage I, 1 had stage II, and 3 had stage III or IV disease. All tumors had a follicular architecture, and 74% of cases had grade 2 or 3 histologic features. All patients expressed CD20 and bcl-6, and 80% were positive for CD10. Bcl-2 expression was detected in only 5 of 16 cases. Consistent with this finding, bcl-2 gene rearrangements were detected in only 2 of 16 cases by polymerase chain reaction. These patients were treated primarily with cyclophosphamide, doxorubicin, vincristine, and prednisone-based chemotherapy; 4 patients also received involved-field irradiation. Of the 13 patients with available clinical follow-up, all but 2 achieved durable clinical remission. Importantly, all 4 patients with tumors diffusely positive for bcl-2 either presented with stage III/IV disease or had disease refractory to therapy, whereas patients with bcl-2-negative tumors uniformly had stage I disease, achieved complete remission, and experienced no relapses. These findings indicate that, in contrast to adult follicular lymphomas, dysregulated bcl-2 expression does not play a significant pathogenetic role in most pediatric follicular lymphomas. However, bcl-2 expression in pediatric follicular lymphoma identifies a subset of patients in whom disease is often disseminated at clinical presentation and is more refractory to combination chemotherapy.
Lorsbach
Robert B
RB
Department of Pathology, St Jude Children's Research Hospital, 332 N Lauderdale Street, Memphis, TN 38105, USA. robert.lorsbach@stjude.org
Shay-Seymore
Dominic
D
Moore
Jennifer
J
Banks
Peter M
PM
Hasserjian
Robert P
RP
Sandlund
John T
JT
Behm
Frederick G
FG
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D020732:Cytogenetic Analysis; D005260:Female; D015321:Gene Rearrangement; D019254:Genes, bcl-2 / Q000235:genetics; D006801:Humans; D016130:Immunophenotyping; D008224:Lymphoma, Follicular / Q000209:etiology / Q000473:pathology* / Q000628:therapy; D008297:Male; D009367:Neoplasm Staging; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000235:genetics / Q000378:metabolism; D012074:Remission Induction
D019253:Proto-Oncogene Proteins c-bcl-2