Living Donor Liver Transplantation for Adult Hepatic Undifferentiated Embryonal Sarcoma: A Case Report

Abstract

Introduction: Undifferentiated embryonal sarcoma of the liver (UESL) is an aggressive tumor. There is no established treatment, and it is an uncommon tumor in adults. Treatment usually involves a combination of surgery, chemotherapy, and occasionally liver transplantation (LT). The role of LT in patients with irresectable UESL merits exploration.

Case description: A 20-year-old boy with a large palpable abdominal mass, shortness of breath, and weight loss presented to our clinic. His computed tomography scan showed showed a large cystic lesion measuring 11.5 × 22.7 × 23 cm, predominantly involving the left lobe and right anterior sector, with a biopsy consistent with UESL. The tumor was abutting to the right hepatic vein, with bland main portal vein thrombosis. Due to an irresectable tumor and deteriorating clinical condition, living donor LT was performed. The patient remains in good health at 16 months of follow-up.

Practical implication: In carefully selected patients with UESL, when other options are not feasible, LT might prolong survival and improve quality of life.

Keywords: Chemotherapy; liver transplantation; survival; undifferentiated embryonal sarcoma.

Figure 1

Large solid-cystic tumor predominantly involving the left lobe and right anterior sector closely applied to the right hepatic vein (white arrow)

Figure 2

Large solid-cystic tumor with bland thrombus in the main portal vein (white arrow)

Figure 3

Intraoperative photo showing large hepatic tumor involving the left lobe and right anterior sector (Segment 8) of the liver

Figure 4

Right lobe graft with Segment 5 and 8 veins reconstructed using DACRON grafts

Figure 5

Explant histopathology showing variably cellular tumor with anaplastic, spindled/oval cells in myxoid stroma (H/E ×200)