Unusual Presentation of Ewing Sarcoma in the Adrenal Gland: A Secondary Malignancy from a Survivor of Burkitt Lymphoma

The occurrence of Ewing sarcoma as a secondary malignancy is an extremely rare event in long-term cancer survivors. In addition, the occurrence of Ewing sarcoma in the adrenal gland is highly unusual. In this case report, we treated a 20-year-old male patient with cyclophosphamide, doxorubicin, vincristine, dexamethasone, and methotrexate and cytarabine chemotherapy following a diagnosis of Stage IV Burkitt lymphoma. Following complete remission, he had been maintained for 2 years without evidence of disease. However, a regular follow-up computed tomography scan found a left adrenal gland mass and a biopsy revealed positive membrane-localized mic-2 expression (CD99) and the presence of the translocation of the EWSR1 gene. To our knowledge, this is the first case report of Ewing sarcoma occurring in the adrenal gland of a patient who was treated with cyclophosphamide, doxorubicin, vincristine, dexamethasone/methotrexate and cytarabine chemotherapy for Burkitt lymphoma.