A 7-year-old boy presents with a rapidly enlarging mass of the mandible. Biopsy shows sheets of intermediate-sized lymphoid cells with round nuclei, multiple small nucleoli, and abundant apoptosis interspersed with tingible-body macrophages producing a starry-sky pattern. Which genetic alteration defines this tumour?

• A Overexpression of BCL2 from t(14;18)
• B Translocation of MYC to the immunoglobulin heavy chain locus ✓
• C Rearrangement of BCL6 at 3q27
• D Amplification of MDM2 on chromosome 12

Explanation

Endemic Burkitt lymphoma presents as a jaw or facial bone mass in African children and is EBV-associated. Histology shows a starry-sky pattern, and the defining lesion is translocation of the MYC oncogene on chromosome 8 into the immunoglobulin heavy chain locus, t(8;14), causing constitutive MYC-driven proliferation. BCL2 overexpression from t(14;18) is the hallmark of follicular lymphoma, which is an indolent tumour of adults, not a rapidly growing paediatric jaw mass.

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.