Burkitt lymphoma is one type of non-Hodgkin lymphomas that is highly malignant and dificult to cure. Atypical Burkitt lymphoma, one subtype of this tumor according to the WHO classification, is a rare disease. We describe the case of a 78-year-old man who was given a diagnosis of atypical Burkitt lymphoma. Paresthesia of the right mental region arose 4 days before presentation. Three mass lesions (in the right pterygomandibular space, the left mandibular ramus, and the duodenum) were found on clinical and radiologic examinations. Histopathologically, a sheet of predominantly medium-sized proliferating lymphoid cells with nuclear pleomorphism, a starrysky pattern, and a very high growth fraction were revealed. We therefore diagnosed atypical Burkitt lymphoma. The disease was Stage IV according to the Ann Arbor classification. He was transferred to a hematology unit and received COP therapy. Although transient improvement was noted, the patient soon showed signs of central nervous system involvement and died 2 months later.
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