Florid granulomatous reaction in Epstein-Barr virus-positive nonendemic Burkitt lymphomas: report of four cases.
Epithelioid cell granulomas have been reported in association with a wide range of neoplasms including malignant lymphomas. In lymphoma, this refers mainly to Hodgkin disease and T-cell-derived non-Hodgkin lymphomas where a granulomatous reaction is probably evoked by aberrant cytokine production in the tumor cells or other cells composing the tumor background. Here we report on four cases of sporadic Burkitt lymphoma with unusual florid granulomatous reaction. In all samples, the tumor cells were admixed with numerous epithelioid cells that formed clusters and granulomatous lesions. No microorganisms could be detected at the tissue level, and there were no clinical or laboratory indications of an underlying immunodeficiency. The lymphomas harbored a specific morphology and immunophenotype of Burkitt lymphoma, and the presence of translocation breakpoint in MYC gene was confirmed by interphase fluorescence in situ hybridization. In all four patients, the lymphoma was associated with Epstein-Barr virus infection, detected by EBER in situ hybridization and the latency I phenotype as defined by lack of expression of LMP1. All four patients were treated with polychemotherapy, achieved a complete remission, and are alive without evidence of disease. We draw attention to this unusual phenomenon as it caused difficulties in interpretation and delayed diagnosis and hypothesize on the possible role of Epstein-Barr virus products.
Haralambieva
Eugenia
E
Department of Pathology & Laboratory Medicine, University Hospital Groningen, Groningen, The Netherlands.
Rosati
Stefano
S
van Noesel
Carel
C
Boers
Evan
E
van Marwijk Kooy
Marinus
M
Schuuring
Ed
E
Kluin
Philip
P
D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis; D002051:Burkitt Lymphoma / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D019353:Endemic Diseases; D005260:Female; D006099:Granuloma / Q000188:drug therapy / Q000473:pathology* / Q000821:virology; D004854:Herpesvirus 4, Human / Q000235:genetics / Q000302:isolation & purification*; D006801:Humans; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D012333:RNA, Messenger / Q000032:analysis; D012367:RNA, Viral / Q000032:analysis; D016896:Treatment Outcome
D014408:Biomarkers, Tumor; C099537:Epstein-Barr virus encoded RNA 1; C106732:Epstein-Barr virus encoded RNA 2; D012333:RNA, Messenger; D012367:RNA, Viral