Hepatoblastoma Definition:  
A malignant tumor of the liver usually presenting as a right upper quadrant (RUQ) mass.

Epidemiology:
Incident Rate (Rate at which the general population would get this type of cancer) : 0.9/million or 0.9 people get the cancer out of every 1 million people.

Age of Onset: 
Median age: 12 months; majority detected by 18 months of age

Risk Factors:
Males > Females (1.5-1.7:1)

Pathogenesis:
1.     Background
                Hepatoblastomas have been associated    
                with:
                    Beckwith-Wiedemann Syndrome
                    Wilm's Tumor
                    Rhabdomyosarcoma
                    Aicardi Syndrome
                    Familial Adenomatous Polyposis
        Hepatoblastomas and the first three disorders        
        may share a common 
            chromosomal  anomaly->abnormal gene 
            product->tumor

Clinical Features

1.     Gastrointestinal Manifestations
                -RUQ mass
                -+/- abdominal pain (15-20% of patients)
                - +/- abdominal distention
                - hepatomegaly/splenomegaly
                - digital clubbing
                - jaundice (in < 5% of cases)
                - tumor rupture with acute abdominal 
                  signs/symptoms
2.     Others
                A. Systemic
                    - anorexia, nausea/vomiting, weight loss
                    - hemihypertrophy (in 2-3% of cases)
                    - congenital hemangiomas
B.     Endocrine
                - isosexual precosity (virilization)
                    - in 10% of male patients
                    - genital enlargement, pubic hair

Investigations

1.     Diagnosis
                A. Liver Biopsy
                    - 4 cell subtyptes
                        - fetal 
                        - embroyonal
                        - macrotrabecular
                        - small - cell undifferentiated
                    - Highly pedunculated and vascular with      
                      areas 
                      of focal necrosis and vascular invasion
                    - multicentric>right lobe>left lobe

2.     Imaging Studies
                A. Abdominal Ultrasound/CT/MRI
                    - solid tumor with calcifications in 30%
                B. Chest X-Ray/CR/MRI
                    - 10% will have pulmonary metastases
                C. Skeletal X-Rays
                    - osteopenia, multiple pathological fractures, 
                       vertebral compression (hepatoblastomas 
                       may be associated with an abnormal calcium 
                       metabolism)

3.     Serum
                - elevated alpha-fetoprotein (AFP) in 90-95% of 
                  cases
                - elevated beta-hCG (in virilizing males)
                - elevated transaminases and bilirubin in 20% of 
                  cases

Management

1.     Surgery
                A. Lobectomy
                        - right and left partial and complete 
                          lobectomy and trisegmentectomy

                B. Liver Transplantation
                        - experimental

2.     Chemotherapy
                A. Adjuvent Therapy
                    - used pre and post resection
                    - can reduce size of the primary tumor by 90 % 
                      and pulmonary metastases
                    - doxorubicin, cisplatin

3.     Prognosis
                - 5 year survival rate is 35% 

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