Search Database ICD-O-3 Code Lists

Name

Burkitt lymphoma (BL), (NOS)

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: lymph nodes, head and neck, GI tract, abdomen, thyroid, ovary, testis, breast.

See abstractor notes

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Burkitt lymphoma (BL), NOS is part of the Mature B-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B15)

For Burkitt lymphoma, extranodal sites are commonly involved. In endemic BL, the mandible and maxilla, and other facial bones are frequently involved. In sporadic BL, the abdomen, soft tissues of the orbit, Waldeyer ring, gingiva, thyroid gland, testis, and breast are commonly involved. BL arising in immune deficiency/dysregulation settings more commonly have nodal involvement. Extranodal involvement is frequent.

For High grade B-cell lymphoma with 11q aberration (HGBCL-11q), most patients present as localized nodal or extranodal disease in the head and neck or gastrointestinal tract.

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.

Module Rule

None

Alternate Names

Acute leukemia, Burkitt type [OBS] (see 9826/3 prior to 2021)
Acute lymphoblastic leukemia, mature B-cell type (see 9826/3 prior to 2021)
Acute lymphocytic leukemia mature FAB L3 [OBS] (see 9826/3 prior to 2021)
Burkitt, acute lymphoblastic leukemia (B-ALL)
Burkitt cell leukemia [OBS] (see 9826/3 prior to 2021)
Burkitt-like lymphoma, NOS
Burkitt-like lymphoma with 11q aberration
Burkitt tumor [OBS]
EBV-associated Burkitt lymphoma
EBV-negative Burkitt lymphoma
High grade B-cell lymphoma with 11q aberration (HGBCL-11q)
Malignant lymphoma, small non cleaved, Burkitt type [OBS]
Malignant lymphoma, undifferentiated, Burkitt type [OBS]

Definition

Burkitt lymphoma (BL) is a mature aggressive B-cell neoplasm composed of monomorphic, medium-sized cells with basophilic cytoplasm, multiple small nucleoli, a germinal-centre B-cell phenotype, a high proliferation index, and an IG::MYC rearrangement. (WHO 5th ed)

High-grade B-cell lymphoma with 11q aberration (HGBCL-11q) is an aggressive mature B-cell lymphoma with a morphology similar to that of Burkitt lymphoma or showing an intermediate to blastoid appearance in most cases and a characteristic chromosome 11q-gain/loss pattern. Cases with concomitant MYC rearrangements are excluded. (WHO 5th ed)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

t(8;14)(q24;q32) translocation
t(8;22)(q24;q11) and t(2;8)(p12;q24) translocations
11q aberration (11q23.3)

Immunophenotyping

BCL2- (no expression/negative)
BCL6+ (expression/positive)
CD10+ (expression/positive)
CD19+ (expression/positive)
CD20+ (expression/positive)
CD79a+ (expression/positive)
EBV+ (expression/positive)
Ki67+ (expression/positive)
MYC protein strong expression
PAX5+ (expression/positive)

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-10 Codes (Cause of Death codes only)

C83.7 Burkitt tumor

Corresponding ICD-10-CM Codes (U.S. only)

C83.7_ Burkitt lymphoma (effective October 01, 2015)
C83.7A Burkitt lymphoma, in remission (effective October 01, 2024)

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

Age: common in children
Survival: 90% cure for low stage disease, 60-80% cure for high stage disease

Sources

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Large B-cell lymphomas/Burkitt Lymphoma
Pages: Part B: 486-488, 532-538

International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 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

PDQ® Adult Treatment Editorial Board. PDQ Aggressive B-Cell Non-Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <02/24/2025>. Available at: https://www.cancer.gov/types/lymphoma/hp/aggressive-b-cell-lymphoma-treatment-pdq. Accessed <03/20/2025>.
Section: Aggressive B-Cell Non-Hodgkin Lymphoma Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/lymphoma/hp/aggressive-b-cell-lymphoma-treatment-pdq
Glossary