Clinicopathologic findings in high-grade B-cell lymphomas with typical Burkitt morphologic features but lacking the MYC translocation.
In the World Health Organization classification, cases with classical Burkitt morphologic features and a very high proliferation fraction but without the MYC translocation are not clearly designated as a separate entity and are usually categorized as diffuse large B-cell lymphoma (DLBCL). We identified from our records 33 cases of highly aggressive mature B-cell neoplasms from 8 children and 25 adults with typical Burkitt cytomorphologic, histologic, and immunophenotypic (CD20+/CD10+ and surface immunoglobulin-positive) features. Rearrangement of MYC (MYC+) was present in only 18 of 33 cases, but the proliferation fraction was more than 90% in all MYC-cases (no MYC rearrangement). The immunophenotype of the lymphoma cells in the 2 groups was similar. Although children with MYC+ and MYC- neoplasms were treated with chemotherapy regimens appropriate for Burkitt lymphoma, adults with MYC- lymphomas received less aggressive therapy usually given for DLBCL. Survival analysis showed that adults in the MYC- group had an inferior outcome compared with adults with MYC+ disease. Provisional identification of MYC- lymphomas with typical Burkitt morphologic features as an entity separate from DLBCL will facilitate further studies and possible categorization as a separate entity.
Sevilla
Deborah W
DW
Department of Pathology, Duke University Medical Center, Durham, NC 27710, USA.
Gong
Jerald Z
JZ
Goodman
Barbara K
BK
Buckley
Patrick J
PJ
Rosoff
Philip
P
Gockerman
Jon P
JP
Lagoo
Anand S
AS
D000293:Adolescent; D000328:Adult; D000368:Aged; D018951:Antigens, CD20 / Q000378:metabolism; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000378:metabolism; D002051:Burkitt Lymphoma / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics* / Q000378:metabolism; D049109:Cell Proliferation; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D016259:Genes, myc; D006801:Humans; D016130:Immunophenotyping; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics* / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D015260:Neprilysin / Q000378:metabolism; D014178:Translocation, Genetic; D016896:Treatment Outcome
D018951:Antigens, CD20; D014408:Biomarkers, Tumor; D015260:Neprilysin