Undifferentiated (embryonal) sarcoma of liver

A 7-year-old male was brought to our out-patient department by his father with complaint of pain in the abdomen for 10 days. The parents noticed a swelling in the right upper abdomen a day after the pain started. He denied any past history of fever, jaundice, gastrointestinal blood loss or trauma. Examination revealed a non-tender swelling in the right hypochondrium which moved with respiration. Blood work, including tumor markers, was within normal limits. Contrast-enhanced computed tomographic (CECT) scan of abdomen was carried out which showed a large, hypodense, peripherally enhancing lesion involving the right lobe of liver (Panels A,B). The child underwent right hepatectomy which revealed a 15 cm × 12 cm sized solid exophytic tumor adhered to anterior abdominal wall and diaphragm focally. Grossly, the tumor showed a variegated appearance (Panel C). Histopathological examination revealed malignant nuclear features with cellular polymorphism (Panel D). Intracytoplasmic hyaline globules were also seen (Panel E) which were periodic acid-schiff (PAS) positive and diastase resistant. In addition, positivity for vimentin (Panel F), alpha-1-antitrypsin (Panel G) and Pan-CK (Panel H) was seen consistent with the diagnosis of undifferentiated (embryonal) sarcoma. The child had an uneventful recovery, being discharged 7 days after the surgery.