T-cell-rich large B-cell lymphoma in children and adolescents - A clinicopathologic report of six cases from the Children's Cancer Group Study CCG-5961

BACKGROUND. T-cell-rich large B-cell lymphoma (TCRLBCL) is a morphologic su bset of diffuse large B-cell lymphoma that has been confused with Hodgkin d isease and reactive lymphadenopathies. To the authors' knowledge the majori ty of reports of TCRLBCL are from adults, and it is not widely recognized a s occurring in the pediatric population. The current study reports a cohort of six cases of TCRLBCL from the Children's Cancer Group CCG-5961 study. METHODS. Biopsies from patients entered on CCG-5961 were submitted for cent ral pathology review and immunophenotyping. Six cases of TCRLBCL were ident ified and correlated with clinical characteristics. RESULTS. Of 86 cases centrally reviewed to date on CCG-5961, 20 (23%) were diagnosed as diffuse large B-cell lymphomas. Of these, 6 cases(7% of total cases and 30% of large B-cell cases) were TCRLBCL, based on a diffuse growt h pattern with a minor population of neoplastic large B cells and an associ ated extensive reactive T-cell infiltrate. All patients with TCRLBCL were m ales ages 12-16 years. Three patients with TCRLBCL had advanced stage disea se. No bone marrow or central nervous system involvement was detected in an y case. CONCLUSIONS. TCRLBCL is a morphologic subtype of diffuse large B-cell lymph oma that may be difficult to recognize due to the extensive infiltrate of r eactive T cells. This entity is not well recognized in pediatric patients, but in the current study represented 7% of all cases and 30% of large B-cel l lymphomas received for central review from the ongoing CCG-5961 protocol. Because TCRLBCL may be confused with Hodgkin disease and reactive lymphade nopathies, it is essential that this entity be recognized in the pediatric age group. Cancer 2000;88:2378-86. (C) 2000 American Cancer Society.