What is Burkitt Lymphoma?

Burkitt lymphoma is a highly aggressive non-hodgkin B cell lymphoma. They may occur in 3 categories: 1) African (endemic) Burkitt lymphoma, 2) sporadic (nonendemic) Burkitt lymphoma, and 3) a subset of aggressive lymphomas occurring in individuals infected with HIV.

What is the Pathology of Burkitt Lymphoma?

The pathology of Burkitt lymphoma is:

-Etiology: The cause of Burkitt lymphoma is primarily involving c-MYC rearrangement arising in germinal center derived B cells.

-Genes involved: MYC gene.

-Pathogenesis: The sequence of events that lead to Burkitt lymphoma are associated with translocations of the MYC gene on chromosome 8 that lead to increased MYC protein levels. It was also seen that these tumors have an increased activity of the transcription factor TCF3 which leads to rapid growth.

-Histology: The histology associated with Burkitt lymphoma shows tissues that are effaced by a diffuse infiltrate of intermediate-sized lymphoid cells 10 to 25 um in diameter with round or oval nuclei, coarse chromatin, several nucleoli and moderate amount of cytoplasm. The tumor exhibits a high mitotic index and contains numerous apoptotic cells, the nuclear remnants of which are phagocytosed by interspersed benign macrophages. These phagocytes have abundant clear cytoplasm creating a characteristic ‘starry sky’ pattern.

Burkitt Lymphoma. Biopsy of preaortic lymph node showing monomorphic moulded neoplastic lymphoid cells with large pale macrophage "stars". Burkitt lymphoma masquerading as cardiac tamponade. Kaul P, Javangula K - Journal of cardiothoracic surgery (2007). Not altered. CC.
Burkitt Lymphoma. Burkitt’s lymphoma of the adult - Histopathology and immunohistochemistry. a, Medium sized lymphocytes with high mitotic index and macrophages with tingible bodies (Hematoxylin-Eosin, 40x); b, CD79a expression; c, bcl6 expression; d, CD 20 expression; e, CD 10 expression; f, Ki- 67 expression (LSAB-HRP, nuclear counterstaining with type II Gill’s Hematoxylin). The role of EBV in the pathogenesis of Burkitt's Lymphoma: an Italian hospital based survey. Pannone G, Zamparese R, Pace M, Pedicillo MC, Cagiano S, Somma P, Errico ME, Donofrio V, Franco R, De Chiara A, Aquino G, Bucci P, Bucci E, Santoro A, Bufo P - Infectious agents and cancer (2014). Not altered. CC.
Burkitt Lymphoma. Burkitt’s lymphoma of the child - Histopathology and immunohistochemistry. a, Medium sized lymphocytes with high mitotic index and macrophages with tingible bodies (Hematoxylin-Eosin, 60x); b, CD79a expression; c, bcl6 expression; d, CD 20 expression; e, CD 10 expression; f, Ki 67 expression (LSAB-HRP, nuclear counterstaining with type II Gill’s Haematoxylin). The role of EBV in the pathogenesis of Burkitt's Lymphoma: an Italian hospital based survey. Pannone G, Zamparese R, Pace M, Pedicillo MC, Cagiano S, Somma P, Errico ME, Donofrio V, Franco R, De Chiara A, Aquino G, Bucci P, Bucci E, Santoro A, Bufo P - Infectious agents and cancer (2014). Not altered. CC.
Burkitt Lymphoma. Immunohistochemical expression of LMP-1 in Burkitt’s lymphoma. Note the strong nuclear staining of EBV-infected cells. (LSAB-HRP, x400; nuclear counterstaining with type II Gill’s Haematoxylin). The role of EBV in the pathogenesis of Burkitt's Lymphoma: an Italian hospital based survey. Pannone G, Zamparese R, Pace M, Pedicillo MC, Cagiano S, Somma P, Errico ME, Donofrio V, Franco R, De Chiara A, Aquino G, Bucci P, Bucci E, Santoro A, Bufo P - Infectious agents and cancer (2014). Not altered. CC.
Burkitt Lymphoma. In situ hybridization for EBER in Burkitt’s lymphoma. Note diffuse and strong staining showing early RNA transcripts of EBV accumulated in the nucleus of EBV-infected cells (Ventana® EBER ISH iView Blue Plus Kit; original magnification x200). The role of EBV in the pathogenesis of Burkitt's Lymphoma: an Italian hospital based survey. Pannone G, Zamparese R, Pace M, Pedicillo MC, Cagiano S, Somma P, Errico ME, Donofrio V, Franco R, De Chiara A, Aquino G, Bucci P, Bucci E, Santoro A, Bufo P - Infectious agents and cancer (2014). Not altered. CC.
Burkitt Lymphoma. The macroscopic photograph of Burkitt lymphoma. (a) A 12-years boy presented with abdominal lesions. There was a huge mass within the mesentery and multiple polyploid lesions were present in the cecum; (b) a 6-years boy had a jejunum mass, with thickening of intestinal wall and hemorrhage; (c) an 8-years boy presented with a big facial bone mass. Sporadic Burkitt lymphomas of children and adolescents in Chinese: a clinicopathological study of 43 cases. Bi CF, Tang Y, Zhang WY, Zhao S, Wang XQ, Yang QP, Li GD, Liu WP - Diagnostic pathology (2012). Not altered. CC.
Burkitt Lymphoma. Dr. Denis Burkitt, a child with Burkitt lymphoma and the“starry-sky pattern”. Analogies in medicine: starry-sky appearance. Andrade-Filho Jde S - Revista do Instituto de Medicina Tropical de São Paulo (2014 Nov-Dec). Not altered. CC.
Burkitt Lymphoma. Starry Night over the Rhone. Van Gogh. 1888. Analogies in medicine: starry-sky appearance. Andrade-Filho Jde S - Revista do Instituto de Medicina Tropical de São Paulo (2014 Nov-Dec). Not altered. CC.

How does Burkitt Lymphoma Present?

Both endemic and sporadic Burkitt lymphomas are found mainly in children or young adults with a male predominance. Overall Burkitt lymphoma accounts for about 30% of childhood non-Hodgkin lymphomas in the United States. The symptoms, features, and clinical findings associated with Burkitt lymphoma include: Endemic Burkitt lymphoma often presents as a mass involving the mandible and shows an unusual predilection for involvement of abdominal viscera (kidney, ovaries and adrenal glands). Sporadic Burkitt lymphoma most often appears as a mass involving the ileocecum and peritoneum.

How is Burkitt Lymphoma Diagnosed?

Burkitt lymphoma is diagnosed mainly by karyotyping or FISH studies detecting C-MYC translocation. Elevated serum lactate dehydrogenase may raise its suspicion.

How is Burkitt Lymphoma Treated?

Burkitt lymphoma is treated with intensive chemotherapy (R-CODOX-M / R-IVAC, (rituximab, cyclophosphamide, vincristine, doxorubicin, methotrexate, ifosfamide, etoposide, cytarabine ; or R-hyper-CVAD/ R-MA (rituximab, cyclophosphamide, vincristine, doxorubicin, dexamethasone, methotrexate, cytarabine).

What is the Prognosis of Burkitt Lymphoma?

The prognosis of Burkitt lymphoma is fair with an event free survival in children of around 90% at 5 years and around 85% at 4 years in adults. Most children and young adults can be cured. The outcome is more guarded in older adults.