AtlasMalignantBurkitt Lymphoma

Malignant Tumors

Burkitt Lymphoma

aka BL

Highly aggressive B-cell non-Hodgkin lymphoma driven by MYC translocation; jaw involvement is characteristic in the African endemic form.

Genetics
t(8;14) MYC-IGH
Pattern
Starry-sky
Rx
Intensive chemo + rituximab

§ overviewOverview

Aggressive mature B-cell lymphoma defined by the t(8;14) MYC translocation (variants t(2;8), t(8;22)).

§ icdICD Classification

C83.7

§ etiologyEtiology

  • 01Endemic form: EBV + Plasmodium falciparum co-infection
  • 02Sporadic: unknown
  • 03Immunodeficiency: HIV/AIDS-associated

§ epidemiologyEpidemiology

Endemic — equatorial Africa, children 4–7y, jaw mass in 50–70%. Sporadic — worldwide, ileocaecal.

§ pathogenesisPathogenesis

MYC translocation places MYC oncogene under IGH enhancer → constitutive proliferation; near-100% Ki-67 index; doubling time ≈ 24 h.

§ clinicalClinical Features

  • 01Rapidly enlarging, painless jaw swelling (posterior maxilla most common)
  • 02Loose teeth, ill-fitting appliances
  • 03Facial deformity, proptosis
  • 04B symptoms uncommon
  • 05Extranodal: kidneys, ovaries, CNS

§ differentialDifferential Diagnosis

  • 01Osteosarcoma
  • 02Ewing sarcoma
  • 03Odontogenic abscess
  • 04Ameloblastoma
  • 05Rhabdomyosarcoma

§ histopathHistopathology

  • 01Diffuse sheets of medium-sized monotonous lymphoid cells
  • 02Squared-off cytoplasm, multiple nucleoli
  • 03Numerous tingible-body macrophages producing 'starry-sky' pattern
  • 04Ki-67 ≈ 100%

§ radiographicRadiographic Features

  • 01Moth-eaten radiolucency
  • 02Loss of lamina dura
  • 03Floating teeth
  • 04Cortical destruction with soft-tissue mass

§ investigationsInvestigations

  • 01Incisional biopsy with fresh tissue for FISH
  • 02CT/MRI head-neck, chest-abdomen-pelvis
  • 03Bone marrow biopsy
  • 04LDH, uric acid (tumour lysis risk)

§ ihcIHC / Special Stains

  • 01CD20+, CD10+, BCL6+
  • 02BCL2 negative
  • 03MYC rearrangement by FISH

§ treatmentTreatment

  • 01Intensive short-course chemotherapy (CODOX-M/IVAC, DA-EPOCH-R)
  • 02Rituximab
  • 03Intrathecal methotrexate for CNS prophylaxis
  • 04Aggressive tumour lysis prophylaxis (hydration, allopurinol, rasburicase)

§ complicationsComplications

  • 01Tumour lysis syndrome
  • 02Airway obstruction
  • 03CNS relapse

§ prognosisPrognosis

Endemic: 80–90% cure with intensive chemo. Sporadic adult: 70–80%. HIV-associated: worse.

§ examKey Examination Points

  • 01Child with rapidly growing jaw mass — biopsy immediately
  • 02Send fresh tissue for cytogenetics

§ revisionQuick Revision Summary

  • 01MYC t(8;14) · starry-sky · Ki-67 100% · CODOX-M/IVAC

§ vivaBDS Viva Questions

  • 01What is starry-sky appearance?
  • 02Endemic vs sporadic BL?
  • 03What is tumour lysis syndrome?

§ mcqsMCQs — Assessment (3)

Question 1

Characteristic translocation:

Question 2

'Starry-sky' cells are:

Question 3

Ki-67 in BL:

References

  1. WHO Classification of Haematolymphoid Tumours 2022

Draft — pending faculty review. Educational use only; verify against current guidelines and primary sources before clinical application.