A 12-year-old boy presented with right lower quadrant pain and a 5-lb weight loss. A 4.5-cm ileocecal mass was excised and diagnosed as Epstein-Barr virus–negative Burkitt lymphoma (BL). Numerous pericecal lymph nodes revealed preserved nodal architecture with follicles with intact mantle zones and numerous tingible body macrophages imparting a “starry sky” appearance (panel A: original magnification, ×10; hematoxylin and eosin staining). Closer inspection revealed patchy follicular colonization by BL, which also characteristically imparts a starry sky appearance due to high cellular turnover. This was confirmed by an immunostain for MYC (panel B: original magnification, ×10).

BL is a mature, follicular-derived B-cell neoplasm that harbors a MYC translocation in 100% of cases. Characteristic homing of neoplastic cells to follicular centers may appear deceptively reactive because reactive germinal centers also harbor a starry sky. BL localized to follicles should not be considered an early lesion because it is invariably associated with the presence of aggressive lymphoma elsewhere.