A 7-year-old boy presents with a rapidly enlarging right mandibular mass. Biopsy shows sheets of intermediate-sized lymphoid cells with round nuclei, multiple nucleoli, and scant basophilic cytoplasm containing lipid vacuoles, interspersed with tingible body macrophages. Ki-67 proliferation index approaches 100%. Which genetic alteration and therapeutic principle define this tumour?
- A BCL2 rearrangement; treat with observation if completely resected
- B Cyclin D1 rearrangement; treat with lenalidomide maintenance
- C MYC translocation; treat with short-duration intensive multi-agent chemotherapy such as dose-adjusted R-EPOCH ✓
- D PAX5 mutation; treat with single-agent rituximab
Correct answer: C. MYC translocation; treat with short-duration intensive multi-agent chemotherapy such as dose-adjusted R-EPOCH
Explanation
Endemic Burkitt lymphoma presents as a jaw or facial bone tumour in African children and is associated with Epstein-Barr virus. Histology shows a starry-sky pattern from tingible body macrophages, and near-universal MYC activation occurs through t(8;14) or variant translocations. Because the tumour doubling time is extremely fast, treatment requires brief, very intensive multi-agent chemotherapy with CNS-directed therapy, such as dose-adjusted R-EPOCH or CODOX-M/IVAC. Local excision or single-agent rituximab is inadequate for a curable tumour.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.