Sporadic Burkitt Lymphoma Lacking MYC Protein Expression: A Rare Entity With Diagnostic Pitfalls and Dismal Prognosis.

Authors

Xiaoying Lou, Keqi Chen, Mansi Yu, Cai Lei, Qingqing Cai, Xia Yang, Yuhua Huang

Published in

The American journal of surgical pathology. Jul 31, 2026. Epub Jul 31, 2026.

Abstract

The pathognomonic genetic alteration in Burkitt lymphoma (BL) is the immunoglobulin (IG)-MYC translocation, causing intense nuclear MYC expression in almost all cases. Rare cases harbor MYC rearrangements yet lack detectable MYC protein expression. In this study, we report 8 cases of MYC protein-negative sporadic BL (MYC⁻ sBL). These cases accounted for 1.4% (8/574) of all sporadic BL cases. The median age was 10 years (range: 5 to 77 y), with a male-to-female ratio of 3:1. The majority (62.5%, 5/8) presented with advanced-stage disease (III/IV), and 25.0% (2/8) had bone marrow involvement. Morphologically, all cases met the World Health Organization (WHO) diagnostic criteria for BL, characterized by monotonous medium-sized cells with a typical "starry sky" pattern. Immunophenotypically, all cases exhibited a germinal center B-cell phenotype with nearly 100% Ki-67 positivity. Notably, MUM1 was positive in 50.0% (4/8) of cases, while Epstein-Barr virus (EBV)-encoded RNA (EBER) was positive in only 25.0% (2/8). Despite fluorescence in situ hybridization (FISH)-confirmed MYC rearrangement and the absence of BCL2 or BCL6 abnormalities, MYC protein expression was undetectable. Furthermore, patients with MYC⁻ sBL exhibited significantly inferior overall survival (OS) compared with those with MYC protein-positive sporadic BL (MYC+ sBL) (P<0.0001, HR=404.1). These findings confirm that MYC⁻ sBL is a rare but distinct clinicopathological entity associated with poor clinical outcomes, despite lacking unique histologic features. The molecular mechanisms underlying the absence of MYC protein expression remain to be investigated.

PMID:
42533724
Bibliographic data and abstract were imported from PubMed on 31 Jul 2026.

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