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Name
Burkitt lymphoma
ICD-O-1 Morphology
9750/3: Burkitt's tumor
Effective
1978 - 1991
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), bone marrow (for peripheral blood and bone marrow involvement only)
Coding Manual:
Hematopoietic Coding Manual (PDF)
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.
For the Burkitt leukemia phase, where there is only peripheral blood and bone marrow involvement, these were previously coded as histology 9826/3. For 2021+ forward, these cases will be coded with histology 9687/3 and primary site C421.
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.
For the Burkitt leukemia phase, where there is only peripheral blood and bone marrow involvement, these were previously coded as histology 9826/3. For 2021+ forward, these cases will be coded with histology 9687/3 and primary site C421.
Diagnostic Confirmation
This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.
Grade
Not Applicable
Module Rule
None
Alternate Names
Acute lymphocytic leukemia FAB L-3
BL
Burkitt leukemia
Burkitt-like lymphoma with 11q aberration
Burkitt-like lymphoma, NOS
Burkitt lymphoma, NOS
Burkitt tumor [OBS]
Endemic BL
FAB L3 [OBS]
Immunodeficiency-associated BL
Sporadic BL
Definition
Burkitt lymphoma (BL) is a highly aggressive but curable lymphoma that often presents in extranodal sites or as an acute leukemia (see 9826/3). It is composed of monomorphic medium-sized B cells with basophilic cytoplasm and numerous mitotic figures. The frequency of EBV infection varies according to the epidemiological subtype of BL.
Burkitt-like lymphoma with 11q aberration is a subset of lymphomas identified by several recent studies that resemble Burkitt lymphoma (BL) morphologically, but to a large extent phenotypically, and in terms of microRNAs and gene expression profile, but that lack MYC rearrangements. Instead they have a chromosome 11q alteration characterized by proximal gains and telomeric losses.These lymphomas lack the 1q gain frequently seen in BL and have more-complex karyotypes than BL.
Burkitt-like lymphoma with 11q aberration is a subset of lymphomas identified by several recent studies that resemble Burkitt lymphoma (BL) morphologically, but to a large extent phenotypically, and in terms of microRNAs and gene expression profile, but that lack MYC rearrangements. Instead they have a chromosome 11q alteration characterized by proximal gains and telomeric losses.These lymphomas lack the 1q gain frequently seen in BL and have more-complex karyotypes than BL.
Definitive Diagnostic Methods
Bone marrow biopsy
FISH
Genetic testing
Histologic confirmation
Immunophenotyping
Immunophenotyping
BCL2- (no expression/negative)
BCL6+ (expression/positive)
CD3- (no expression/negative)
CD5- (no expression/negative)
CD10+ (expression/positive)
CD19+ (expression/positive)
CD20+ (expression/positive)
CD22+ (expression/positive)
CD23- (no expression/negative)
CD38+ (expression/positive)
CD43+ (expression/positive)
CD56+ (expression/positive)
CD77+ (expression/positive)
CD79a+ (expression/positive)
CD138- (no expression/negative)
Ki67+ (expression/positive)
MYC protein strong expression
TCL1 strongly expressed in pediatric BL
TdT- (no expression/negative)
Treatments
Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Immunotherapy
Radiation therapy
Surgery
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Same Primaries
Corresponding ICD-9 Codes
200.2 Burkitt's tumor or lymphoma
Corresponding ICD-10 Codes
C83.7 Burkitt tumor
Corresponding ICD-10-CM Codes (U.S. only)
C83.7 Burkitt lymphoma (effective October 01, 2015)
Signs and Symptoms
Drenching night sweats
Fatigue
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
Blood chemistry studies
Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunohistochemistry
Immunophenotyping
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
Lymph node biopsy
PET (positron emission tomography) scan
Progression and Transformation
None
Epidemiology and Mortality
Sources
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature B-cell neoplasms
Pages: 330-334
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature B-cell neoplasms
Pages: 330-334
International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
