Abstract
A 31-year-old female complained of upper abdominal and back pain. Laboratory tests showed elevated levels of aspartate aminotransferase, alanine aminotransferase and α-fetoprotein. Computed tomography revealed that the tumor, measuring 14.5 cm × 10.4 cm, occupied the anterior and medial segments of the liver and consisted of multicystic and solid lesions. The preoperative diagnosis was a hepatic cystadenocarcinoma. The operation was performed urgently because of tumor rupture. Histopathologically, spindle and asteroid cells were found to have proliferated diffusely. There were no neoplastic epithelial tumor cells. Tumor cells had periodic acid-Schiff-positive hyalin globules. At the periphery, trapped normal bile duct cells were observed. The final diagnosis was embryonal sarcoma of the liver (ESL). Interestingly, irregular islands of chondrosarcoma-like lesions were found in the tumor and the tumor-associated vascular endothelium showed immunoreactivity for KIT. Two months after the operation, the tumor recurred. At 6 mo follow-up, the patient is alive with the disease and undergoing chemotherapy. This is the first report of ESL with chondroid differentiation.