• Highly aggressive B-cell lymphoma

Etiology

• Translocation from chromosome 8 to chromosome 14
  • Chromosome 8 – locus of c-myc oncogene
  • Chromosome 14 – locus of Ig heavy chain
• Can also be t(8:22) or t(2:8) translocation
• Agents: EBV and malaria

Epidemiology

• Children and adolescents (50% childhood malignancy in Africa)
• 3-8 year olds
• M:F = 2:1

Clinical

• Doubling time is 25 hours and 100% growth fraction, therefore be quick to diagnose
• Rapidly expanding intraoral mass
• Pain and paresthesia

3 main clinical forms:

1. Endemic:

• Equatorial Africa
• Malaria as a co-factor
• 95% associated with EBV infection
• 50% have jaw involvement
• Involves mandible, abdominal viscera, kidney, adrenal glands, ovaries

2. Sporadic:

• Outside Africa
• Affects young adults
• Involves ileocecum and peritoneum
• Abdominal mass due to ileocecal involvement

3. Immunodeficiency associated:

• HIV
• Organ transplant patients (Immunosuppressant drugs)

Diagnosis

• Incisional biopsy
• Immunochemistry – Monoclonality of lymphocytes
• Ki67 staining (proliferation marker) – all cells in various stages of cell cycle
• Immunofluorescence (EBV and CD21)

Histology

• Monomorphic sheets of densely packed neoplastic lymphocytes
• High mitotic index: 100% cells in active division
• “Starry sky” appearance:
  • B lymphocytes – Sky
  • Macrophages – Stars – pale foamy cytoplasm

Radiology

• Floating teeth appearance (due to osteolysis)

Management

1st line drugs: (CHOP)

• Cyclophosphamide
• Adriamycin
• Vincristine
• Prednisolone

2nd line drugs:

• CHOP regimen + Procarbazine/cytarabine

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