Burkitt lymphoma (BL) is an aggressive form of non-Hodgkin B-cell lymphoma typically presenting in the first and second decades of life. This tumor very commonly is associated with a translocation of chromosome 8 and 14, causing overexpression of the c-Myc proto-oncogene. Tumors may present in the abdomen, kidney, testes, breast, central nervous system (CNS), bone marrow, and head / neck region. There are 3 major variants: endemic, sporadic, and immunodeficiency- (often HIV-) associated BL.

The endemic form is strongly associated with the Epstein-Barr virus (EBV) and is twice as common in males. In addition to the jaw and orbit, endemic BL now includes tumors in the abdomen and several epithelium-containing tissues, including the thyroid, ovaries, kidneys, adrenal glands, and breasts. Coinfection with malaria greatly increases the risk of endemic BL, and, therefore, this variant is commonly seen in areas where malaria is endemic, such as Africa, Brazil, and New Guinea.

The sporadic form is commonly seen in the United States and Western Europe. This variant is approximately 3-4 times more common in males. The typical presentation is an abdominal mass, with approximately one-quarter of cases presenting with a jaw or facial tumor, but this form can affect virtually any organ.

The immunodeficiency-associated form of BL is associated with AIDS or other immunodeficient states (eg, organ transplant recipients). Patients infected with HIV who develop BL typically have a CD4 count greater than 200. The risk of immunodeficiency-associated BL in patients with HIV does not decrease with antiretroviral therapy (ART). Lymph node, bone marrow, gastrointestinal tract, and CNS involvement are common for this BL variant and may lead to life-threatening tumor lysis syndrome.

Cutaneous manifestations of BL are exceedingly rare. The pathogenesis of BL-associated skin lesions is unclear but has been suggested to be the result of direct extension, hematogenous spread, or iatrogenic tumor seeding during procedures or trauma.

Related topic: non-Hodgkin lymphoma