Undifferentiated embryonal sarcoma of the liver

Case contributed by Dr Shimalis Tadasa Fayisa

Presentation

Abdominal swelling over 6 months.

Patient Data

Age: 9 years
Gender: Female

Large solid-appearing mass with variable echogenicity found in the left lobe of the liver.

Quadruple phase CT abdomen

CT

There is a large heterogeneous mass within the left lobe of liver measuring 9.2 x 8.7 cm in axial dimensions. It demonstrates multiloculated hypodense components separated by enhancing internal septations. There is also a gradually enhancing peripheral rim giving the appearance of a pseudocapsule. The mass abuts the superior and medial borders of the spleen. There are no other lesions within the right lobe of the liver and the spleen.

There are no apparent peritoneal or pleural fluid collections.

Case Discussion

Although undifferentiated embryonal sarcoma of the liver is an uncommon type of liver malignancy, some studies place it as the third most prevalent primary liver cancer in the pediatric population after hepatoblastoma and hepatocellular carcinoma. It is most frequently observed in youngsters aged 6 to 10 years.

They are mostly asymptomatic, but when they do cause symptoms, children will typically report stomach pain, swelling, and occasionally constipation, diarrhea, and weight loss.

They appear mostly solid on ultrasound and mostly cystic on CT and MRI, with heterogeneous enhancement on post-contrast studies, notably on the delayed phase with septal and rim enhancement giving the appearance of a pseudocapsule.

This 9-year-old child has a large liver mass within the left lobe that appears solid on ultrasound but is mainly cystic/necrotic on CT with enhancing septa and pseudocapsule. An ultrasound guided core biopsy was performed and histopathology revealed spindle-shaped cells with a sarcomatous appearance.

The diagnosis of undifferentiated embryonal liver sarcoma is almost certain given the patient's age, imaging characteristics, and histopathology findings.

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