Endemic Burkitt lymphoma — DTM&H MCQ

Correct answer: C — Endemic Burkitt lymphoma

A) Endemic Burkitt lymphoma is correct. The clinical picture, an African child with a rapidly growing, painless jaw mass, combined with biopsy showing a monomorphic infiltrate of medium-sized B-cells interspersed with tingible-body macrophages (the classic "starry sky" pattern) and positive EBV serology, is pathognomonic for endemic (African) Burkitt lymphoma. This variant is driven by chronic EBV infection acting alongside holoendemic P. falciparum malaria, which impairs T-cell immunosurveillance of EBV-infected B-cells and predisposes to the MYC-driving t(8;14) translocation. Jaw and facial bone involvement is the classic presentation in endemic disease, distinct from sporadic Burkitt lymphoma seen in the UK, which more often presents with abdominal disease and is less consistently EBV-associated. Why the other options are wrong: B. Ameloblastoma: this is a benign, slow-growing odontogenic epithelial tumour; biopsy would show epithelial islands resembling enamel organ, not sheets of monomorphic B-cells or a starry sky pattern, and it is not linked to EBV. E. Osteosarcoma of the mandible: histology shows malignant osteoid-producing spindle cells, not lymphoid sheets, and there is no EBV association; imaging typically shows a sunburst periosteal reaction rather than a purely soft-tissue lymphomatous mass. D. Rhabdomyosarcoma: this presents as a soft-tissue mass with malignant striated muscle differentiation (desmin, myogenin positive), not a B-cell lymphoid proliferation, and lacks the starry sky pattern. A. Dental abscess with osteomyelitis: this would present acutely with pain, fever and inflammatory signs, and biopsy or pus would show acute inflammatory cells and organisms, not a monomorphic malignant lymphoid population. Key point: painless jaw swelling in an African child with starry sky histology and EBV positivity is diagnostic of endemic Burkitt lymphoma, reflecting the EBV-malaria-driven MYC translocation pathway distinct from sporadic Burkitt lymphoma in the UK.

Reference: Cambridge-Africa (University of Cambridge), 'Looking beyond the starry skies of endemic Burkitt lymphoma', on endemic Burkitt lymphoma pathogenesis, EBV/malaria co-infection and jaw presentation in Uganda, https://www.cambridge-africa.cam.ac.uk/cambridge-africa-updates/looking-beyond-the-starry-skies-of-endemic-burkitt-lymphoma/