Burkitt lymphoma of the jaws in the Amazon region of Brazil

Ha Rebelo-Pontes,Fs Correa-Pontes,Fp Fonseca,Bab De Andrade,Dm Guimaraes,Op De Almeida,Mc De Abreu,Dsp Junior

doi:10.4317/medoral.18936

Abstract

Objectives: To describe the clinicopathologic and immunohistochemical features of Burkitt’s lymphoma of the jaws in 7 patients of Northern Brazil. Study Design: Clinical data concerning gender, age, affected site, clinical presentation, symptomatology and follow-up were collected from the clinical files. Histopathology was complemented with a broad immunohistochemical panel and in situ hybridization for Epstein-Barr virus (EBV). Results: Most of the patients were infants and 5 out of 7 were males. The mandible was affected in 5 cases and all patients also presented abdominal involvement. All cases were positive for CD45, CD20, CD79a, CD10, Bcl-6 and EBV. Ki-67 proliferative index was approximately 100%. Six patients were treated with R-CHOP (Rituximab + Cyclophosphamide, Doxorubicin, Vincristine and Prednisolone) chemotherapy, and 2 of these died of the disease. One young adult patient refused treatment and died 3 months after initial diagnosis. Conclusions: Burkitt’s lymphoma of the jaws diagnosed in the Amazon region of Brazil present similar clinicopathologic features to those described in endemic areas of Africa, including EBV positivity. Key words:Burkitt’s lymphoma, EBV, Brazil, Amazon region.

Highlights

Burkitt’s lymphoma (BL) is a B-cell lineage neoplasia first reported by the British physician and missioner Dr Albert Cook in Uganda in the beginning of the twentieth century, followed by a more complete description by the Irish surgeon Dr Dennis Burkitt
BL is an aggressive B-cell lymphoma with a very high proliferative rate and rapid doubling time that reveals different clinical and biological characteristics mostly similar characteristics to endemic BL seen in Africa
BL in Brazil is considered of an intermediate type, clinically resembling sporadic BL, mostly presenting abdominal involvement and rarely affecting the jaws, with about 60% of the cases revealing positivity for EpsteinBarr virus (EBV) [16,17]

Summary

Introduction

Burkitt’s lymphoma (BL) is a B-cell lineage neoplasia first reported by the British physician and missioner Dr Albert Cook in Uganda in the beginning of the twentieth century, followed by a more complete description by the Irish surgeon Dr Dennis Burkitt. It was originally described as an aggressive tumor that frequently affected the gnathic bones of children from equatorial Africa, whose lymphoid origin was proposed in 1960 by the pathologist George OConor [1,2,3,4,5,6]. The aim of this study is to describe the clinicopathological, immunohistochemical and EpsteinBarr virus (EBV) status of BL affecting the gnathic bones of 7 patients from Northern Brazil and compare the results with other reports from Brazil and endemic areas of Africa

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Conclusion