Hepatocellular carcinoma

Key points

Older children - most common hepatic malignancy in older than 10 years

2nd most common hepatic malignancy in children

Risk factors

• Hepatitis B + C

• Pre-existing liver disease

• Familial Adenomatous Polyposis: APC

• Neurofibromatosis: NF-1

• Fanconi Anaemia

• Glycogen Storage Disease: Glucose-6-phosphatase

• Alagille Syndrome: JAGGED-1, NOTCH2

Good prognostic indicators:

• Age <4

• Male

• Black

aFP not elevated >400 in most cases

Investigations:

PRETEXT staging

Can use TNM to compare to adults

No need for biopsy unless doubt of diagnosis

Histology: Epithelial tumour - giant tumour cells forming broad trabecula

Management:

PLADO chemotherapy (cisplatin and doxorubicin) with sorafenib (tyrosine kinase inhibitor)

Resection

Overall 5y survival 25%

References

Skube, Mariya, et al. "Hepatocellular Carcinoma." Pediatric Surgery NaT, American Pediatric Surgical Association, 2022. Pediatric Surgery Library, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829480/all/Hepatocellular_Carcinoma.

Tomlinson GE. Hepatoblastoma and other liver tumors in children. In: Nathan, ed. Hematology and Oncology of Infancy and Childhood. 8th ed. Philadelphia, PA: Elsevier, Inc; 2015:1886-1905