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Malignant lymphoma, small non cleaved, Burkitt type
Malignant lymphoma, undifferentiated, Burkitt type
Highly aggressive NHL often presenting in extranodal sites such as the GI tract; the hallmark is over-expression of oncogene c-Myc. Three clinical variants: endemic, most often in African children involving extranodal sites, especially facial bones; sporadic, in children, adolescents; immunodeficiency, frequently in setting of HIV with CD4 counts > 200 cells/microliter.
There are four variants of Burkitt lymphoma (BL): Endemic BL, Sporadic BL, Immunodeficiency-associated BL and Burkitt leukemia variant. All of these variants are coded 9687/3. Each of these variants manifests differences in clinical presentation, morphology, and biology. In each of the clinical variants, patients are at high risk for central nervous system involvement.
Endemic BL occurs in equatorial Africa and is the most common childhood malignancy in this area. Endemic BL is also endemic in Papua, New Guinea. The jaws and other facial bones (orbit) are the site of presentation in about 50% of cases. The distal ileum, cecum, anterior and/or omentum, gonads, kidneys, long bones, thyroid, salivary glands, and tonsils may be affected with or without jaw involvement. Localization may be found in BM, but manifestation of leukemia in peripheral blood is not present.
Sporadic BL is seen throughout the world, mainly in children. This variant is primarily seen in association with HIV infection often occurring as the initial manifestation of the acquired immunodeficiency syndrome (AIDS). Jaw tumors are very rare. Majority of cases present with abdominal masses. The ileocecal region represents the most frequent site of involvement. Ovaries, kidneys, and breast are frequently involved. Retroperitoneal masses may result in spinal cord compression. LN presentation is seen more commonly in adults. Waldeyer ring and mediastinal involvement are rare.
Immunodeficiency-associated-BL is seen in association with AIDS. It is often the first manifestation of AIDS. Nodal localization is frequent as well as bone marrow involvement.
Burkitt leukemia variant is a leukemic phase observed in patients with bulky disease but only rare cases present purely as acute leukemia with peripheral blood and bone marrow involvement.