ABSTRACT
This case report describes the diagnostic and therapeutic challenges faced in managing an elderly diabetic man with BCOR-rearranged sarcoma, a rare, aggressive malignancy. The patient presented with neck swelling, initially suspected to be a high-grade lymphoma but later found to be undifferentiated small round cell sarcoma. Further investigations with PET-CT revealed a mass in the lower abdomen, leading us to reconsider the prior diagnosis of non-Hodgkins' lymphoma. Subsequent biopsies from an abdominal deposit indicated a high-grade round cell sarcoma with differentials including BCOR-CCNB3 fusion/BCOR-ITD sarcoma, CIC-DUX4 fusion sarcoma, and EWSR-non-ETS fusion sarcoma. A second opinion from a dedicated oncopathology lab confirmed the diagnosis of BCOR-rearranged sarcoma. The patient underwent exploratory laparotomy and diversion stoma but developed complications post-surgery. Due to the advanced stage and extensive metastases, the patient opted for supportive care due to poor outcomes with treatment. This case underscores the importance of raising awareness and conducting further research to improve the management of rare malignancies like BCOR-rearranged sarcoma.
