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SUMMARY

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SUMMARY

Burkitt lymphoma is one of the highly aggressive B-cell lymphomas. It was the first tumor to be etiologically associated with (1) a virus, specifically Epstein-Barr virus; (2) a specific translocation involving the MYC oncogene; and (3) one of the first cancers shown to be curable by chemotherapy alone. It presents in three clinically distinct forms: endemic, sporadic, and immunodeficiency associated. Burkitt lymphoma is an uncommon form of lymphoma in adults, with an incidence of approximately 1200 patients per year in the United States. Over the last decade, the definition of Burkitt lymphoma has been refined, largely as a consequence of improvements in immunohistochemical, cytogenetic, and molecular diagnostic techniques. Transcriptional profiling has more clearly defined Burkitt lymphoma at the molecular level, while whole-genome sequencing has expanded our understanding of the mutational landscape that underlies this disease. Despite these refinements in diagnostic criteria, the differential diagnosis includes several high-grade lymphomas, including a group of patients with a diagnosis defined by the World Health Organization (WHO) as intermediate between Burkitt lymphoma and diffuse large B-cell lymphoma and a new WHO provisional entity called Burkitt-like lymphoma with 11q aberration. Burkitt lymphoma is a highly curable malignancy in the modern therapeutic era. The majority of younger patients are cured with intensive chemotherapeutic regimens, and increasing efficacy has been demonstrated with reduced-intensity treatments in older patients. Remaining challenges include the optimal management of older patients, the development of therapy for patients with relapsed or refractory disease, and the translation of gains made in treatment to the management of endemic disease.

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DEFINITION AND HISTORY

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Burkitt lymphoma (BL) may present in three distinct forms: endemic (African), sporadic, and immunodeficiency associated.1 The endemic form (eBL) is the most common pediatric tumor in sub-Saharan Africa and other regions of the world where malaria is endemic. It typically presents in the jaw or maxilla and is associated with Epstein-Barr virus (EBV) infection at an early age. Although there are reports dating to as early as 1910, it was Denis Burkitt who is credited with describing this malignancy in 1958 as a common tumor in children of Uganda.2,3 Originally thought by Burkitt to be a sarcoma of the jaw, it was the pathologist George O’Connor who, in 1960, concluded it was a lymphoma.4 In 1964, while studying BL samples by electron microscopy, sent to them from Uganda by Burkitt, Michael Anthony Epstein, Yvonne Barr, and Bert Achong discovered EBV when they recognized viral particles were present in tumor cells,5 thus helping to launch the nascent field of human tumor virology. Further studies of BL over many years led to epidemiologic associations with both EBV and malaria in Africa.6,7 Tumors of a similar histologic appearance were subsequently identified in the United States, Middle East, and elsewhere (i.e., nonendemic regions) and termed sporadic BL. Sporadic cases were found to occur in older individuals, typically presenting in the abdomen ...

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