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Abdomen » Liver & Biliary System
Hepatoblastoma
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Presentation

9-year-old male patient with a tumor in the upper right quadrant of the abdomen. Collateral circulation is visualized in the abdominal wall.


Ultrasound is requested to determine the location of the lesion.
 
 
Caption: Sagittal sonogram of the liver.
Description: Sagittal sonogram shows well-defined isoechogenic mass with thin, thin, regular hypoechoic halo, displacing adjacent structures.
 
 
Caption: Transverse sonogram of the liver.
Description: Transverse sonogram shows well-defined isoechogenic mass with thin, thin, regular hypoechoic halo, displacing adjacent structures.
 
 
Caption: Sagittal sonogram of the liver.
Description: Sagittal sonogram shows well-defined isoechogenic mass with thin, thin, regular hypoechoic halo, displacing adjacent structures. The color Doppler study shows central and peripheral vascularization.
 
 
Caption: Sagittal sonogram of the liver.
Description: Sagittal sonogram shows well-defined isoechogenic mass with thin, thin, regular hypoechoic halo, displacing adjacent structures. The power Doppler study shows central and peripheral vascularization.
 
Differential Diagnosis

- Hepatic mesenchymal hamartoma

- Infantile hemangioendothelioma

- Hepatic metastases, e.g. neuroblastoma

- Hepatocellular carcinoma

- Rhabdomyosarcoma of biliary tract (rare)
 
Final Diagnosis

Hepatoblastoma
 
Discussion

Hepatoblastoma is the most common primary malignant liver tumor in children under four years of age who usually present with painless abdominal mass and raised AFP. It is tumor of embryonic origin1,3.

 

Most cases are seen during the first 18 months of life and diagnosis in adulthood is exceedingly rare. Occasionally the tumor may be diagnosed antenatally2. There is a recognized slight male preponderance with a M/F ratio of up to ≈3:2. There may also be predilection towards the right lobe of the liver.

 

Most children present with abdominal distension or an asymptomatic palpable abdominal mass. However, other presenting symptoms include: abdominal pain, anorexia, vomiting, jaundice, pyrexia, anemia, back pain and pseudo precocious puberty (due to chronic GnRH secretion)1.

 

Most hepatoblastomas are seen sporadically, however, it has been known to be associated with: Beckwith-Wiedemann syndrome1, hemihypertrophy: seen in 2% of patients with hepatoblastoma1, familial adenomatosis polyposis3,5, fetal alcohol syndrome6, prematurity and low fetal birth weight1,6, Gardner syndrome5, glycogen storage disease and biliary atresia1-2.

 

On ultrasound, hepatoblastomas appear as predominantly echogenic soft tissue mass. In larger tumors heterogeneity and variable echogenicity is common. Even when large, they tend to be relatively well defined7. Intralesional calcifications may be visible as areas of shadowing4,7.

 

Serum alpha fetoprotein (AFP) levels are frequently elevated (90% of cases)6.
 
Case References

1. Taeusch HW, Ballard RA, Gleason CA et-al. Avery's diseases of the newborn. W B Saunders Co. (2005) ISBN:0721693474. Read it at Google Books - Find it at Amazon

2. Woodward PJ, Sohaey R, Kennedy A et-al. From the archives of the AFIP: a comprehensive review of fetal tumors with pathologic correlation. Radiographics. 25 (1): 215-42. doi:10.1148/rg.251045156 - Pubmed citation

3. Herzog CE, Andrassy RJ, Eftekhari F. Childhood cancers: hepatoblastoma. Oncologist. 2000;5 (6): 445-53. doi:10.1634/theoncologist.5-6-445 - Pubmed citation

4. Dachman AH, Pakter RL, Ros PR et-al. Hepatoblastoma: radiologic-pathologic correlation in 50 cases. Radiology. 1987;164 (1): 15-9. Radiology (abstract) - Pubmed citation

5. Stoupis C, Ros PR. Imaging findings in hepatoblastoma associated with Gardner's syndrome. AJR Am J Roentgenol. 1993;161 (3): 593-4. AJR Am J Roentgenol (citation) - Pubmed citation

6. Tanimura M, Matsui I, Abe J et-al. Increased risk of hepatoblastoma among immature children with a lower birth weight. Cancer Res. 1998;58 (14): 3032-5. Cancer Res. (link) - Pubmed citation

7. Gubernick JA, Rosenberg HK, Ilaslan H et-al. US approach to jaundice in infants and children. Radiographics. 20 (1): 173-95. Radiographics (full text) - Pubmed citation

8. Russo P, Ruchelli ED, Piccoli DA. Pathology of pediatric gastrointestinal and liver disease. Springer Verlag. (2004) ISBN:0387406549. Read it at Google Books - Find it at Amazon

9. Suchy FJ, Sokol RJ, Balistreri WF. Liver disease in children. Lippincott Williams & Wilkins. (2001) ISBN:0781720982. Read it at Google Books - Find it at Amazon

10. McGahan JP, Goldberg BB. Diagnostic ultrasound. Informa Health Care. (2008) ISBN:1420069780. Read it at Google Books - Find it at Amazon
 
Technical Details

Ultrasound scanner. - Voluson 730 PRO. General Electric.

Transducer.- 2-8MHz convex array.

Scanning in both transverse and longitudinal planes.
 
Follow Up

Surgical resection is the treatment of choice although preoperative chemotherapy may be used to reduce tumor bulk. Chemotherapy is also employed in following surgical resections. If the tumor is resectable then liver transplantation provides a cure, as long as no metastatic disease is present. The lungs are a relatively frequent site of metastases.

 

Overall there is 65-70% long-term survival9, however, prognosis depends on staging:

 

- stage I: can expect very good long-term survival, of up to 100% with combined chemotherapy and surgery8,10.

- stage II: 75-80%

- stage III: 65%

- stage IV: 0-27%9.

 

The patient was referred to the child's hospital (high complexity), where he underwent laboratory tests (AFP) and imaging (CT and MRI) confirming the diagnosis. No metastases or adenopathies were found, nor vascular compromise. He is receiving chemotherapy and preparing for liver transplantation.
 
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