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 Module 7
Most common sites of involvement: ileocecal region, ovaries, kidneys, breast, lymph nodes (adults)

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Grade
6 - B-cell
Module Rule
None
Alternate Names
BL
Burkitt leukemia variant
Burkitt-like lymphoma (see 9680 for 2010+)
Burkitt tumor [OBS]
Endemic BL
Immunodeficiency-associated BL
Malignant lymphoma, small non cleaved, Burkitt type [OBS]
Malignant lymphoma, undifferentiated, Burkitt type [OBS]
Sporadic BL
Definition
Highly aggressive NHL often presenting in extranodal sites such as the GI tract; the hallmark is over-expression of oncogene c-Myc.
Abstractor Notes
There are three variants of Burkitt lymphoma (BL): Endemic BL, Sporadic BL and Immunodeficiency-associated BL. 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.

Three clinical variants:
1. 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.

2. 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. Retroperitoneal masses may result in spinal cord compression.

3. 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 is the leukemic phase observed in patients with bulky disease, but only rare cases present purely as acute leukemia with peripheral blood and bone marrow involvement. (See 9826/3 for Burkitt leukemia)
Definitive Diagnostic Methods
FISH
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
Clonal IG rearrangements
Immunophenotyping
BCL6+
CD3-
CD10+
CD19+
CD20+
CD22+
CD38+
CD43+
CD56+
CD77+
Ki67+
TdT-
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
Drenching night sweats
Fever (for no known reason)
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph node
Skin rash or itchy skin
Weight loss (for no known reason)
Diagnostic Exams
CT (CAT) scan
Complete blood count (CBC)
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan
Progression and Transformation
None
Epidemiology and Mortality
Age: common in children
Survival: 90% cure for low stage disease, 60-80% cure for high stage disease