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Undifferentiated embryonal sarcoma of the liver (UESL) is a malignancy of mesenchymal origin observed predominantly in the pediatric population and very rarely in adults. We describe the case of a 21-year-old male who presented with acute onset of right upper quadrant pain and distention. Physical examination of the patient revealed right upper quadrant tenderness with the lower border of the liver palpable, 4 cm below the right costal margin. Laboratory tests performed on admission showed that the patient’s liver function tests, urinalysis, complete blood count, and basic metabolic panel were within reference range. The levels of viral hepatitis and tumor serum markers were all within normal limits except for an elevated level of cancer antigen (CA) 19-9. Magnetic resonance imaging (MRI) and a computerized tomography (CT) scan showed two well-circumscribed lesions in the right lobe. The biopsy of the lesion showed UESL. The patient was started on chemotherapy. On his fifth cycle of chemotherapy, the patient was offered orthotopic liver transplantation (OLT). The patient underwent a successful OLT. There were no postoperative complications. Increased survival time and prevention of the recurrence of USEL can be achieved by surgical resection of the tumor combined with adjuvant and neoadjuvant chemotherapy. For unresectable tumors, OLT with chemotherapy can be a potential cure in younger patients.

Unresectable Undifferentiated Embryonal Sarcoma of the Liver in an Adult Male Treated with Chemotherapy and Orthotopic Liver Transplantation