Embryonal sarcoma of the liver with chondroid differentiation

Area of Science:

• Hepatology
• Pediatric Oncology
• Surgical Pathology

Background:

• Embryonal sarcoma of the liver (ESL) is a rare, aggressive malignant liver tumor.
• ESL typically affects children and young adults, presenting with nonspecific symptoms.
• Preoperative diagnosis can be challenging due to varied imaging and histological findings.

Purpose of the Study:

• To report a rare case of embryonal sarcoma of the liver (ESL) in an adult female.
• To describe the unique presentation of ESL with chondroid differentiation.
• To discuss the diagnostic and management implications of this rare entity.

Main Methods:

• Case presentation of a 31-year-old female with liver tumor.
• Diagnostic workup including laboratory tests, computed tomography (CT), and histopathological examination.
• Surgical intervention and postoperative follow-up.

Main Results:

• A large liver tumor (14.5 cm × 10.4 cm) with mixed cystic and solid components was identified.
• Histopathology revealed diffuse proliferation of spindle and asteroid cells, lacking neoplastic epithelial cells, with periodic acid-Schiff-positive hyalin globules.
• The final diagnosis was embryonal sarcoma of the liver (ESL) with unusual chondroid differentiation and KIT-positive vascular endothelium.
• Tumor recurrence occurred two months post-operation, with the patient alive with disease at 6-month follow-up on chemotherapy.

Conclusions:

• This is the first reported case of embryonal sarcoma of the liver (ESL) exhibiting chondroid differentiation.
• ESL, even in adults, requires high suspicion and comprehensive histopathological analysis for accurate diagnosis.
• The aggressive nature of ESL necessitates vigilant follow-up and consideration of multimodal treatment strategies.