Hepatoblastoma

Case contributed by Dr Yair Glick

Presentation

Severe microcytic hypochromic anemia. Hepatomegaly on palpation.

Patient Data

Age: 4 years
Gender: Female
Ultrasound

Huge mass in the right hepatic lobe measuring approximately 15 cm in diameter. The mass is less vascular than the liver itself and borders on the portal vein. The IVC is displaced and narrowed, demonstrating flow, as well as in the left hepatic vein. Both the intra- and the extrahepatic bile ducts are not dilated. The right kidney is displaced caudally. The collecting system in the left kidney is dilated.

n summary: the features are most compatible with hepatoblastoma.

CT

CT chest-abdomen pelvis:
High position of the right diaphragm due to hepatomegaly (see below), with compressive atelectasis in the right lung base. Small right pleural effusion. No lesion suspicious for metastatic spread to the lungs.

Marked liver enlargement. Large heterogeneous, well-demarcated right-lobe mass in segments VII and VIII measuring 10 cm (AP) x 11 cm (transverse) x 13 cm (CC). The mass enhances less than the adjacent liver parenchyma and shows quite extensive hypodense/necrotic areas and small calcifications, the latter mainly in the periphery of the mass. The right portal vein courses through the mass; no encasement of the main portal vein or its left branch identified. The caudate lobe is most probably involved. The mass exerts pronounced mass effect, including the leftward displacement of the IVC resulting in the latter's narrowing. The hepatic veins are not adequately displayed; however, it appears that a venous tributary - most probably, of the left hepatic vein - is not encased by the mass. No additional hepatic lesions identified. The right kidney is displaced caudally. The inferior segments of the right hepatic lobe are heterogeneous, possibly due to edema or altered perfusion dynamics. The posterior branch of the right portal vein appears very thin, without a filling defect. No unusual dilatation of intra- or extrahepatic bile ducts.

In light of these findings, the supposition is that this is a PRETEXT II tumor with caudate lobe involvement (C1).

Small amount of free abdominal and pelvic fluid. No clear lymphadenopathy.

The osseous structures are preserved. There is a lumbosacral transitional vertebra.

In summary: Large right-lobe hepatic mass, compatible with PRETEXT II C1. The differential diagnosis is of hepatoblastoma and, with a smaller probability, hepatocellular carcinoma and embryonal sarcoma. Small right pleural effusion.  No evidence of metastasis to lungs.

Case Discussion

Pathology report:

Hepatoblastoma, epithelial component, fetal type (clear and dark cell pattern), stained positive by immunostains to hepatocyte, alpha-fetoprotein, glypican3 and INI. 

PlayAdd to Share

Case information

rID: 73479
Published: 13th Jan 2020
Last edited: 15th Jan 2020
Inclusion in quiz mode: Included
Institution: Laniado Hospital

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.