A unique pattern of extranodal involvement in Korean adults with sporadic Burkitt lymphoma: a single center experience

Abstract

Extranodal involvement is common in patients with Burkitt lymphoma (BL). We evaluated the pattern of extranodal involvement and its impact on clinical outcomes in a single center cohort of adult Korean patients with sporadic BL. We retrospectively identified 64 patients with BL in the registry of non-Hodgkin lymphoma of the Asan Medical Center between 1996 and 2009. We assessed their clinical features and distribution of extranodal sites and analyzed clinical outcomes, including complete response rate after chemotherapy, overall survival, and progression-free survival, relative to baseline characteristics and involved extranodal sites. Extranodal involvement was found in 57 patients (89 %), with 34 (53.1 %) having two or more extranodal sites. The stomach (26.6 %) was the most common site, followed by the small and large intestines (25 %), bone marrow (23.4 %), genitourinary tract (21.9 %), and bones (18.8 %). Two patients (3.1 %) showed central nervous system (CNS) involvement. Complete response rates to chemotherapy were not related to sites of extranodal involvement. Two-year overall survival rates were lower in patients with bone marrow (33.3 vs. 74.6 %, p = 0.010) and CNS (0.0 vs. 66.6 %, p = 0.048) involvement than in patients with involvement at other extranodal sites. The stomach, genitourinary tract, and bones were the most commonly involved extranodal sites in Korean BL patients, but site had no prognostic significance.

Minimally Invasive Treatment of Sporadic Burkitt’s Lymphoma Causing Ileocaecal Invagination

“…BL is a B-cell lymphoma with a short doubling time and usually developing an enormous tumoral mass (bulky disease) [ 2 ]. BL is characterized by a chromosomal translocation that results in deregulation of the c-MYC oncogene.…”

Oculomotor nerve palsy as a preceding symptom of adult sporadic Burkitt lymphoma: A case report and review of the literature

“…The pathology of CNS leukemia affects the entire nervous system, including spinal cords, cranial nerves, cerebral hemispheres and associated nerve roots. The diagnosis of CNS leukemia is based on neurological dysfunction, leukemia cells infiltrating the CSF, and/or abnormal neuroradiography on enhanced CT and MRI scans (12,13). However, it is extremely rare that a patient with neurological deficits does not exhibit any abnormalities in either CSF or neuroradiography, and therefore BL is easily neglected or misdiagnosed.…”

Gastric Burkitt lymphoma

“…[ 2 ] The sporadic forms are very rarely encountered in both Europe and Asia, about 4 to 5 cases being yearly diagnosed in large medical centers. [ 3 ]…”

“…Although BL is considered a nodal lymphoma, the extranodal involvement is present in more than 80% of the cases. [ 3 ] The extranodal BL especially occurs in gastrointestinal tract (2 quarters of the cases) and head and neck areas (1 quarter); bone marrow, genito-urinary tract, bones, central nervous system, and liver were also reported to be involved. [ 3 , 4 ] In more than 60% of the cases, the overall survival is below 5 months; the negative prognostic factors are bone marrow and central nervous system involvement [ 3 ] as well as delayed diagnosis.…”