Hepatoblastoma

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD medical weight loss NYC and sleep center NYC

Hepatoblastoma
Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Abdominal mass, abdominal pain, weight loss, nausea, vomiting
Complications	N/A
Onset	Typically in children under 3 years old
Duration	Variable
Types	N/A
Causes	Unknown
Risks	Premature birth, low birth weight, Beckwith-Wiedemann syndrome, familial adenomatous polyposis
Diagnosis	Medical imaging, biopsy
Differential diagnosis	Hepatocellular carcinoma, mesenchymal hamartoma, infantile hemangioendothelioma
Prevention	N/A
Treatment	Surgery, chemotherapy, liver transplantation
Medication	N/A
Prognosis	Generally good with treatment
Frequency	Rare, most common liver cancer in children
Deaths	N/A

Hepatoblastoma is an infrequent malignant liver tumor, predominantly occurring in pediatric patients. Most frequently diagnosed within the initial three years of life, hepatoblastoma represents a significant health concern due to its malignant nature.

Symptoms

The clinical presentation of hepatoblastoma can be elusive in its early stages, often presenting no overt signs or symptoms. However, as the tumor advances in size, notable manifestations include:

• A palpable lump in the abdomen, which may be painful.
• Abdominal distension or swelling.
• Unanticipated weight loss.
• Diminished appetite.
• Episodes of nausea and vomiting.

Etiology

The precise causative factors behind hepatoblastoma remain ambiguous and are yet to be definitively understood.

Risk Factors

Certain conditions and genetic syndromes elevate the susceptibility to hepatoblastoma:

• Premature birth accompanied by extremely low birth weight.
• Early exposure to the hepatitis B virus.
• Biliary atresia.
• Genetic conditions, including:
• Beckwith-Wiedemann syndrome
• Familial adenomatous polyposis
• Aicardi syndrome
• Glycogen storage diseases
• Simpson-Golabi-Behmel syndrome

Diagnostic Approach

To ascertain a definitive diagnosis of hepatoblastoma, a biopsy of the suspicious pediatric liver tumor is imperative.

Tumor Markers

In the context of hepatoblastoma, tumor markers play a pivotal role. Specifically, the Alpha Feto Protein (AFP) and beta-hCG markers offer invaluable insights during the diagnostic and management phases of liver tumors. However, it's vital to note that an elevated AFP isn't solely indicative of malignancy. Its levels can be raised in both benign and malignant tumors. In neonates, AFP levels are naturally high and gradually decrease post-birth. With a half-life spanning 5 to 7 days, AFP levels are expected to be below 10 ng/mL by the age of one.

Therapeutic Interventions

The therapeutic strategy for hepatoblastoma is multidimensional and tailored based on the tumor's severity and progression. Potential interventions include:

• Surgery: To remove the tumor.
• Watchful Waiting: Monitoring the patient without immediate intervention, especially for smaller tumors.
• Chemotherapy: Utilizing drugs to target and eliminate cancer cells.
• Radiation Therapy: Harnessing high-energy rays to shrink or eliminate the tumor.

Prognostic Outlook

With current medical advancements, the 5-year overall survival (OS) rate for pediatric patients diagnosed with hepatoblastoma is approximately 70%. This underscores the significance of early detection and comprehensive therapeutic intervention for optimizing patient outcomes.

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