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WHO Classification of Tumours
Burkitt lymphoma
Colon and rectum


Definition

Burkitt lymphoma (BL) is a fast growing, highly aggressive lymphoma composed of monomorphic medium-sized B-cells with basophilic cytoplasm and numerous mitotic figures. It often presents in extranodal sites or as an acute leukaemia. It typically harbours the Epstein-Barr virus (EBV); MYC [t(8;14)] translocation is highly characteristic but not specific.

> Endemic Burkitt lymphoma
This variant is the most common childhood malignancy in equatorial Africa, with an incidence peak at 4 to 7 years and a male:female ratio of 2:1. BL is also endemic in Papua, New Guinea. In endemic BL, the EBV genome is present in the majority of the neoplastic cells in all patients and there is strong epidemiological link with holoendemic malaria but climatic factors may alo play a role. The jaw and other facial bones are most frequently affected.

> Sporadic Burkitt lymphoma
This variant is seen through out the world, mainly in children and young adults. The incidence is low, representing only 1−2% of all lymphomas in Western Europe and in the USA, but accounts for 30−50% of all childhood lymphomas. The median age of adult patients is 30 years and the M:F tatio is 2-3:1. The majority of cases presents with abdominal masses, particulary in the ileo-caecal region.

> Immunodeficiency-associated Burkitt lymphoma
This variant is primarily seen in association with the human immunodeficiency virus (HIV) infection, often occurring as the initial manifestation of the acquired immunodeficiency syndrome (AIDS).

In all variants, the CNS, ovaries, kidneys and breasts are also frequently involved.

> Burkitt leukaemia
A leukaemic phase can be observed in patients with bulky disease. Only rare, mostly male cases present as pure acute leukaemia with peripheral blood (P)B and bone marrow (BM) involvement. Burkitt leukaemia, also termed acute lymphocytic leukaemia-L3, tends to involve the CNS at diagnosis or in early disease stages. Its rapid chemosensitivity easily leads to an acute tumour lysis syndrome

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Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



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Burkitt lymphoma of the colon and rectum
Typical Burkitt lymphoma as primary colorectal lymphoma is rare. The caecum and ascending colon may be involved

Click to access Pubmed
Kohno S, Ohshima K, Yoneda S, Kodama T, Shirakusa T, Kikuchi M (2003)
Clinicopathological analysis of 143 primary malignant lymphomas in the small and large intestines based on the new WHO classification.
Histopathology 43: 135-43



. Sporadic cases of childhood Burkitt lymphoma in the rectum have been described
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Favini F, Massimino M, Esposito V, Maestri L, Fava G, Spreafico F (2008)
Rectal burkitt lymphoma in childhood.
J Pediatr Hematol Oncol 30: 176-8




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Gence A, Sahin C, Celayir AC, Yavuz H (2008)
Primary Burkitt lymphoma presenting as a solitary rectal polyp in a child.
Pediatr Surg Int 24: 1215-7



. Burkitt lymphoma in HIV-infected individuals may still present as a colorectal tumour in the HAART era, as these lymphomas have also been observed in immunocompetent patients in the past
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Carbone A, Gloghini A (2005)
AIDS-related lymphomas: from pathogenesis to pathology.
Br J Haematol 130: 662-70




Click to access Pubmed
Spano JP, Costagliola D, Katlama C, Mounier N, Oksenhendler E, Khayat D (2008)
AIDS-related malignancies: state of the art and therapeutic challenges.
J Clin Oncol 26: 4834-42



. The details of the histology, immunophenotype, cytogenetics and molecular genetics of these tumours are described in detail for the small intestine
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Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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