Medicine · Hematological Malignancies (Leukemias, Lymphoma, Myeloma, Myeloproliferative)

A 9-year-old boy presents with a rapidly enlarging jaw mass. Biopsy shows sheets of intermediate-sized lymphoid cells with round nuclei, multiple small nucleoli, and a starry-sky pattern created by interspersed macrophages. Which molecular event defines this tumor?

  • A Activation of MYC due to translocation to the IgH locus, t(8;14)
  • B Overexpression of BCL2 due to t(14;18)
  • C Cyclin D1 overexpression due to t(11;14)
  • D ALK fusion gene expression
Correct answer: A. Activation of MYC due to translocation to the IgH locus, t(8;14)

Explanation

The jaw mass, starry-sky pattern, and childhood age are classic for endemic Burkitt lymphoma. All forms of Burkitt lymphoma carry a translocation placing the MYC oncogene under immunoglobulin heavy chain promoter control, most commonly t(8;14)(q24;q32). BCL2 overexpression via t(14;18) defines follicular lymphoma, cyclin D1 via t(11;14) defines mantle cell lymphoma, and ALK rearrangements define anaplastic large cell lymphoma.

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.

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