Undifferentiated embryonal sarcoma of the liver in adult patient: A report of two cases

Abstract

Primary sarcomas of the liver are unusual neoplasms developing in adults. They constitute a heterogeneous group of neoplasms including undifferentiated embryonal sarcoma. Patients usually present with an abdominal mass and abdominal pain. Case 1: A 53-year-old woman presented with abdominal pain. Computed tomography showed an occupying mass in the right lobule and an intra-auricular multi-lobulated mass suggestive of a secondary deposit. Biopsy of the hepatic lesion revealed undifferentiated embryonal sarcoma. Despite radiotherapy and supportive measures, her overall status progressively worsened until cardiac arrest. Case 2: A 41-year-old woman presented with hepatomegaly. Abdominal imaging showed cystic lesions in the right hepatic lobule with multiple septae. The patient underwent extended right hepatectomy and a histopathological study reported high-grade undifferentiated embryonal sarcoma. Two years after surgery, a new cystic lesion in the surgical site was recorded and chemotherapy was scheduled. The lesion remained stable for three years when disease progression was observed and second-line chemotherapy was initiated. Although undifferentiated embryonal sarcoma of the liver has poor prognosis, early diagnosis is essential to increase the chances of survival. Currently, surgical resection and chemotherapy are the primary treatment modalities.

Keywords: Liver neoplasia; Sarcoma; Undifferentiated embryonal sarcoma.

Fig. 1. Computed tomography with intravenous contrast showing a (A) right liver lobule with a 20 cm-sized hypodense lesion (arrow) with irregular borders, a few septa, and centripetal reinforcement without any intra or extrahepatic biliary tract dilation. (B) The heart was augmented in size and the left auricle showed a 39 mm-sized hypodense mass with multi-lobulated borders leading to filling defect (black arrow). In addition, a lytic lesion in the fourth right costal arch was documented (white arrow), both of which suggesting secondary deposits.

Fig. 2. Computed tomography with intravenous contrast that showed a (A) liver enlargement due to the presence of a 26 cm-sized cystic lesion with multiple thick septa (arrow). The lesion showed reinforcement with contrast material without evidence of solid tissue, in addition to compression and displacement of structures in the midline towards the left. Abdominal ultrasound (B) showed a previously described lesion with inner echoes and multiple septa, and compressed intrahepatic vascular structures including portal vein (arrow).

Fig. 3. Computed tomography with intravenous contrast (A) after extended right hepatectomy with post-surgical changes, the left lobule showed homogeneous density and regular form as well as an intrahepatic biliary tract with preserved caliber. Two years later (B) there was a 10 mm-sized hypodense heterogeneous lesion in the anterior portion of the left portal vein without occasional filling defects. The remainder of the liver parenchyma showed no changes. Five years after the initial diagnosis (C) the lesion documented previously showed an increase in size to 30 mm, suggesting disease progression.