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Joseph Junewick, MD FACR
over 6 years ago
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Hepatoblastoma

Case Detail

Anatomy: Gastrointestinal
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Neoplasia Malignant
Created: over 6 years ago
Updated: over 6 years ago
Tags: PEDS
Modality/Study Types: CT US
Activities:
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History

2 year old male with hepatomegaly but otherwise healthy.


Case Images


Diagnosis

Hepatoblastoma

Findings

US – Heterogeneous predominantly hyperechoic infiltrative hepatic mass.

CT – Axial and coronal reformated images demonstrate macrolobulated heterogeneous mass nearly replacing the entire liver.

Discussion

Hepatoblastoma is the most common primary hepatic neoplasm in childhood. Patients usually present with abdominal mass; less commonly with anorexia, weight loss, anemia, hemihypertrophy, or isosexual precosity. There is a higher incidence of hepatoblastoma in patients with Beckwith-Wiedemann syndrome and familial polyposis. The median age of onset is one year with most cases occurring before 5 years. Hepatoblastoma occurs more frequently in Caucasians and males.

The malignant component is comprised of either fetal or embryonal epithelial cells, but can also demonstrate a malignant mesenchymal component. Pure fetal histology is associated with a better prognosis. Lesions are usually large, homogeneous, well-circumscribed and unifocal. Serum alpha-fetoprotein is elevated in about 80% of patients; patients with low or normal alpha-fetoprotein levels are associated with a poor prognosis. Alpha-fetoprotein is used to assess response to treatment and often heralds recurrent or metastatic disease.

Approximately 40% of patients have advanced disease at the time of presentation. Metastatic disease most often affects the lungs. Regional lymph node metastasis to the porta hepatis and invasion of the portal vein also occur. Osseous metastasis is unusual.

Reference

Junewick JJ and Shreve P. PET-CT in Pediatric Malignancies in Clinical PET-CT in Radiology, Springer (2011).



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