9687/3 Malignant lymphoma, small noncleaved, Burkitt type



Definitions

Breast
ICD-O-3 topography code: C50
ICD10: C83.7
           

Burkitt lymphoma (BL) is a fast growing, highly aggressive lymphoma composed of monomorphic medium-sized B-cells with basophilic cytoplasm and numerous mitotic figures.





Tumours of haematopoietic and lymphoid tissues
ICD-O-3 topography code: C42 and C77
ICD10: C83.7; Burkitt leukaemia C91.8
           

/span>urkitt lymphoma (BL) is a B-cell lymphoma with an extremely short doubling time that often presents in extranodal sites or as an acute leukaemia. It is composed of monomorphic medium-sized transformed cells. Translocation involving MYC is highly characteristic but not specific. No single parameter (such as morphology, genetic analysis or immunophenotyping) can be used as the gold standard for the diagnosis of BL, but a combination of several diagnostic techniques is necessary.

>



Colon and rectum
ICD-O-3 topography code: C18-C20
ICD10: C83.7
           

/span>urkitt 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.

> ic malaria but climatic factors may alo play a role. The jaw and other facial bones are most frequently affected.

Sporadic Burkitt lymphoma> ency 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> .)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



.


Burkitt lymphoma of the colon and rectum
Typical Burkitt lymphoma as primary colorectal lymphoma is rare. The caecum and ascending colon may be involved
4
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 5
Click to access Pubmed
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



6
Click to access Pubmed
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 7
Click to access Pubmed
Carbone A, Gloghini A (2005)
AIDS-related lymphomas: from pathogenesis to pathology.
Br J Haematol 130: 662-70



8
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 9
 
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



.



Lip, Oral Cavity and Oropharynx
ICD-O-3 topography code: C00-C06, C09-C10, C14
ICD10: C83.7
           

/span>urkitt 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.

> ic malaria but climatic factors may alo play a role. The jaw and other facial bones are most frequently affected.

Sporadic Burkitt lymphoma> ency 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> s.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



.



Small intestine
ICD-O-3 topography code: C17
ICD10: C83.7
           

/span>urkitt 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.

> ic malaria but climatic factors may alo play a role. The jaw and other facial bones are most frequently affected.

Sporadic Burkitt lymphoma> ency 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> s.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



.



Stomach
ICD-O-3 topography code: C16
ICD10: C16
           

/span>urkitt 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.

> ic malaria but climatic factors may alo play a role. The jaw and other facial bones are most frequently affected.

Sporadic Burkitt lymphoma> ency 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> s.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



.

In the stomach, classical BLs may be encountered
13
Click to access Pubmed
Andoh A, Takaya H, Bamba M, Sakumoto H, Inoue T, Tujikawa T, Koyama S, Fujiyama Y, Bamba T (1998)
Primary gastric Burkitt's lymphoma presenting with c-myc gene rearrangement.
J Gastroenterol 33: 710-5



14
Click to access Pubmed
Park YH, Kim WS, Kang HJ, Na II, Ryoo BY, Yang SH, Lee SS, Uhm JE, Kim K, Jung CW, Park K, Ko YH (2006)
Gastric Burkitt lymphoma is a distinct subtype that has superior outcomes to other types of Burkitt lymphoma/leukemia.
Ann Hematol 85: 285-90



. The morphology is identical to that of Burkitt lymphoma encountered elsewhere, with diffuse sheets of medium-sized cells with scanty cytoplasm and round/oval nuclei containing small nucleoli. Within the sheets there are numerous macrophages, giving a ?starry sky? appearance. Mitoses are frequent and apoptotic debris is abundant. The cells express CD10, CD20 and BCL6, but not BCL2. Nearly 100% of nuclei are immunoreactive for Ki67. The cases carry t(8;14). The proportion that are EBV positive varies, being relatively low (15?20%) in sporadic Burkitt lymphoma, and higher in cases associated with immunodeficiency (25?40%)15
 
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



.



Skin
ICD-O-3 topography code: C44
ICD10: C83.7
           

/span>urkitt 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.

> ic malaria but climatic factors may alo play a role. The jaw and other facial bones are most frequently affected.

Sporadic Burkitt lymphoma> ency 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> s.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



.