Definition/General
Introduction:
Burkitt lymphoma is a highly aggressive mature B-cell lymphoma characterized by translocation of the MYC gene
It is one of the fastest-growing human tumors with a doubling time of 24-48 hours.
Origin:
Arises from mature B-cells in germinal center or post-germinal center stage
Characterized by MYC gene rearrangement leading to uncontrolled cell proliferation.
Classification:
WHO Classification recognizes three variants: Endemic (African), Sporadic (non-endemic), and Immunodeficiency-associated
All have similar morphology and genetic features.
Epidemiology:
Endemic form: Children in equatorial Africa, associated with EBV and malaria
Sporadic form: Worldwide, peak 5-15 years
Immunodeficiency-associated: HIV patients.
Clinical Features
Presentation:
Rapidly growing mass
Endemic: Jaw and facial bones (50%)
Sporadic: Abdominal mass (80%)
CNS involvement common
Rapid progression over days to weeks.
Symptoms:
B-symptoms uncommon
Abdominal pain and distension
Bowel obstruction
Cranial nerve palsies (CNS involvement)
Paraplegia (spinal involvement).
Risk Factors:
EBV infection (endemic form)
HIV infection
Other immunodeficiency states
Malaria co-infection (endemic areas)
Young age.
Screening:
No specific screening
Diagnosed by tissue biopsy
Staging by CT/PET scans
Bone marrow biopsy
Lumbar puncture for CNS evaluation
LDH markedly elevated.
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Gross Description
Appearance:
Tan to gray-white mass with fish-flesh appearance
Soft consistency
Areas of necrosis and hemorrhage common due to rapid growth.
Characteristics:
Well-circumscribed masses
May show central necrosis
Friable consistency
Cut surface homogeneous gray-white.
Size Location:
Endemic: Jaw, maxilla, orbit
Sporadic: Abdomen (ileocecal region), ovaries, kidneys
CNS involvement in 15-20%.
Multifocality:
Often multifocal at presentation
Tendency for CNS spread
Bone marrow involvement less common than other lymphomas.
Microscopic Description
Histological Features:
Diffuse proliferation of medium-sized cells
Characteristic starry-sky pattern due to tingible body macrophages
High mitotic rate
Frequent apoptosis.
Cellular Characteristics:
Medium-sized cells (2-3x small lymphocyte)
Round to oval nuclei with coarse chromatin
Multiple nucleoli
Moderate basophilic cytoplasm.
Architectural Patterns:
Diffuse growth pattern
Starry-sky appearance (pathognomonic)
No follicular architecture
Tingible body macrophages scattered throughout.
Grading Criteria:
High-grade lymphoma by definition
Extremely high proliferation rate (Ki-67 >95%)
High mitotic index
Numerous apoptotic bodies.
Immunohistochemistry
Positive Markers:
CD20 positive
CD19 positive
PAX5 positive
CD10 positive (95%)
BCL6 positive
Ki-67 >95% (nearly 100%).
Negative Markers:
BCL2 negative (key distinguishing feature)
TdT negative
CD5 negative
CD23 negative
Cyclin D1 negative.
Diagnostic Utility:
CD10+/BCL6+/BCL2- profile characteristic
Ki-67 >95% virtually diagnostic
MYC protein overexpression
EBV-LMP1 in endemic cases.
Molecular Subtypes:
MYC rearrangement in >95% of cases
Usually t(8;14)(q24;q32) with IGH
Variants: t(2;8) with IGK, t(8;22) with IGL.
Molecular/Genetic
Genetic Mutations:
MYC rearrangement (>95%)
TP53 mutations (70%)
ID3 mutations (35%)
CCND3 mutations
TCF3 mutations
FOXO1 mutations.
Molecular Markers:
MYC-IGH fusion most common
Simple karyotype compared to other lymphomas
EBV episomes in endemic cases.
Prognostic Significance:
CNS involvement worse prognosis
High LDH poor prognostic factor
Overall good prognosis with intensive chemotherapy (>90% cure rate).
Therapeutic Targets:
Intensive multi-agent chemotherapy
CNS prophylaxis mandatory
Rituximab in adults
Investigational: MYC inhibitors, MDM2 antagonists.
Differential Diagnosis
Similar Entities:
Diffuse large B-cell lymphoma
Lymphoblastic lymphoma
Large cell lymphoma with MYC rearrangement
Plasmablastic lymphoma
Atypical Burkitt/Burkitt-like lymphoma.
Distinguishing Features:
Burkitt: Medium-sized cells, Ki-67 >95%, BCL2-, MYC+
DLBCL: Larger cells, Ki-67 <95%, often BCL2+
Lymphoblastic: TdT+, precursor morphology.
Diagnostic Challenges:
Atypical/Burkitt-like lymphoma (morphologic variants)
Double-hit lymphomas
Recognition in immunocompromised patients.
Rare Variants:
Burkitt-like lymphoma with 11q aberration
Plasmacytoid differentiation
EBV-negative endemic cases.
Sample Pathology Report
Template Format
Sample Pathology Report
Complete Report: This is an example of how the final pathology report should be structured for this condition.
Specimen Information
[specimen type], measuring [size] cm
Diagnosis
Burkitt Lymphoma
Variant
[Endemic/Sporadic/Immunodeficiency-associated] type
Histological Features
Diffuse proliferation of medium-sized cells with starry-sky pattern
Cell Morphology
Medium-sized cells with round nuclei and basophilic cytoplasm
Immunohistochemistry
CD20+, CD10+, BCL6+, BCL2-, Ki-67 >95%
Proliferation Index
Ki-67 proliferation index: >95%
MYC Status
MYC rearrangement: [positive/negative] by FISH
EBV Status
EBV: [positive/negative] by EBER in situ hybridization
Cytogenetics
Karyotype: t(8;14)(q24;q32) [if available]
Staging Factors
Stage: [I/II/III/IV], CNS involvement: [present/absent]
Prognostic Factors
LDH: [level], Performance status, CNS involvement
Final Diagnosis
Final diagnosis: Burkitt Lymphoma, [variant], Stage [X]