Burkitt lymphoma

Name
Burkitt lymphoma
ICD-O-1 Morphology
9750/3
Effective 1978 - 1991
ICD-O-2 Morphology
9687/3
Effective 1992 - 2000
ICD-O-3 Morphology
9687/3
Effective 2001 and later
Reportable
for cases diagnosed 1978 and later
Primary Site(s)
See Abstractor Notes and Module 7

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Grade
6 - B-cell
Module Rule
None
Alternate Names
BL
Burkitt lymphoma, NOS
Burkitt-like lymphoma
Endemic BL
Malignant lymphoma, small non cleaved, Burkitt type
Malignant lymphoma, undifferentiated, Burkitt type
Sporatic BL
Definition
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.
Abstractor Notes
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.
Definitive Diagnostic Methods
FISH
Histological confirmation
Immunohistochemistry
Genetics Data
Clonal IG rearrangements
MYC translocations
Immunophenotyping
BCL6+
CD3-
CD16 weakly positive
CD19+
CD20+
CD22+
CD38+
CD43+
CD56+
CD77+
XS10+
Treatments
Chemotherapy
Immunotherapy
Radiation
Stem cell transplant
Surgery
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Corresponding ICD-9 Codes
200.2 Burkitt's tumor or lymphoma
Corresponding ICD-10 Codes
C83.7 Burkitt's tumor
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C83.7 Burkitt lymphoma
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None