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Blood, 15 March 2002, Vol. 99, No. 6, pp. 1959-1964
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
Clinicopathologic analysis of follicular lymphoma occurring
in children
Robert B. Lorsbach,
Dominic Shay-Seymore,
Jennifer Moore,
Peter M. Banks,
Robert P. Hasserjian,
John
T. Sandlund, and
Frederick G. Behm
From the Departments of Pathology and
Hematology/Oncology, St Jude Children's Research Hospital, Memphis,
TN; the Department of Pathology, Carolinas Medical Center, Charlotte,
NC; and the Department of Pathology, Baystate Medical Center,
Springfield, MA.
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.
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