Adult Embryonal Sarcoma of the Liver: Management of a Massive Liver Tumor

Abstract

Undifferentiated embryonal sarcomas of the liver are extremely rare cases in adults. We report the case of a 30-year-old male who presented with early satiety and abdominal pain due to a massive tumor originating from the left liver and occupying the entire epigastrium. The patient underwent bland embolization in an attempt to decrease the size of the tumor. He then underwent a formal left hepatectomy with resection of liver segments 2, 3, and 4. Extrahepatic inflow control of the portal vein and hepatic artery was performed prior to parenchymal transection. No Pringle maneuver was required. Pathology analysis showed a 45 cm tumor consistent with an undifferentiated embryonal sarcoma and negative microscopic margins. The epidemiology, treatment, and prognosis of this unusual cancer presentation are reviewed.

Figure 1

Abdominal mass on physical exam.

Figure 2

CT abdomen and pelvis with intravenous contrast showing large, heterogeneous left hepatic mass. Characterization of mass with regard to hepatic landmarks. (a) Arrow points to umbilical fissure. (b) Arrow identifies portal vein bifurcation. (c) Arrow points to juncture of right hepatic vein with inferior vena cava. (d) Arrow indicates right portal vein.

Figure 3

Tumor occupying entire epigastrium. Large arrow shows the round ligament secured by a suture. Short arrow points to gallbladder.

Figure 4

Small black arrow indicates vessel loop around the left portal vein. Large black arrow indicates loop around common hepatic bile duct. White arrow shows suture on cystic duct stump.

Figure 5

Resection specimen.

Figure 6

PAS positive hyaline globules (high power, H&E).

Figure 7

Vimentin positive stain (high power, H&E).

Figure 8

Beta-catenin showing predominantly a cytoplasmic pattern of staining in the tumor cells, with scattered nuclei showing mild to moderate immunoreactivity (high power, H&E).