ICD-O-1 Morphology

9750/3: Burkitt's tumor
Effective 1978 - 1991

ICD-O-2 Morphology

9687/3: Burkitt lymphoma, NOS
Effective 1992 - 2000

ICD-O-3 Morphology

9687/3: Burkitt lymphoma, NOS
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)

Grade

Not Applicable

Module Rule

None

Alternate Names

BL
Burkitt leukemia variant
Burkitt-like lymphoma with 11q aberration
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

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.

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
MYC translocation at band 8q24 to the IGH region on chromosome 14q32
IGK locus on 2p12 [t(2;8]
IGL locus on 22q11 [t(8;22]

Immunophenotyping

BCL2-
BCL6+
CD3-
CD5-
CD10+
CD19+
CD20+
CD22+
CD23-
CD38+
CD43+
CD56+
CD77+
CD79a+
CD138-
Ki67+
MYC protein strong expression
TCL1 strongly expressed in pediatric BL
TdT-

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

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 (U.S. only)

C83.7 Burkitt lymphoma (effective October 01, 2015)

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

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

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

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Glossary