A 40-year-old White man presented after having 3 months of dull epigastric pain and a weight loss of 13 kg. A computed tomography scan of the abdomen showed multiple hepatosplenic tumors (panel A) and peripancreatic lymphadenopathy. A liver biopsy showed a diffuse infiltrate of lymphoma cells with focal starry-sky appearance (panel B; original magnification ×40). The cells were medium size and possessed a thin rim of cytoplasm and round or oval nuclei with coarse chromatin and inconspicuous nucleoli (panel C; original magnification ×100). Notable were prominent (5-9) phagocytosed apoptotic bodies and coarse apoptotic debris in macrophages. The neoplasm had a Burkitt-like immunophenotype (CD20⁺/CD10⁺/BCL6⁺/MUM1^–/BCL2^–/Ki-67^high). Fluorescence in situ hybridization analysis showed gains of ARHGAP42 on 11q22.1 (panel D; green signals; original magnification ×40; inset, ×100) and loss of FLI1 on 11q24.3 (panel D; red signals; yellow arrows), There was no evidence of MYC translocations. The patient was treated with 4 courses of a rituximab plus cyclophosphamide, vincristine, doxorubicin, and dexamethasone (R-Hyper-CVAD) regimen, and an imaging study revealed interval regression.

These morphologic, immunophenotypic, and molecular findings are characteristic of Burkitt-like lymphoma with 11q aberrations. This entity is a preliminary entity in the 2016 World Health Organization classification scheme and shares similar pathologic features with Burkitt lymphoma, but it bears different genetic alterations. The observation of prominent apoptotic bodies and coarse apoptotic debris in macrophages in a neoplasm with Burkitt lymphoma-like features warrants the search for 11q aberrations.