Hepatocellular carcinoma (HCC) is the most frequent primary liver cancer with a high mortality rate. Other name: hepatoma HCC often develops in patients with chronic liver disease

1. childhood hepatocellular
carcinoma

2. Liver
tumors
in
children
Hepatocellular carcinoma-HCC
HCC

3. Childhood Hepatocellular
Carcinoma-HCC

4. Hepatocellular carcinoma-HCC -info
• Hepatocellular carcinoma (HCC) is the most frequent primary
liver cancer with a high mortality rate.
• Other name: hepatoma
• HCC often develops in patients with chronic liver disease
❖ This type of liver cancer develops from the main liver cells called
hepatocytes

5. HCC- Childhood Hepatocellular Carcinoma
• HCC in childhood is a rare type of cancer that forms in liver
cells called hepatocytes
• 87% are older than 5 years of age
• Male more effected than female
pediatric liver cancers incidence
• 80% Hepatoblastoma
• 20%-33% HCC ,

6. subsets of HCC in the pediatric age group
HCC subset
1-The first group related to With underlying
risk factor such as
❖ cirrhosis
❖ or underlying metabolic, infectious or
vascular liver disease
2- Sporadic HCC without clear risk factor

7. Risk factors of HCC in the pediatric age group
➢ Cirrhosis is absent in 26%-62% of childhood HCC
two major risk factors
❖ perinatally acquired HBV infection
❖ and tyrosinemia
Tyrosinemia is a genetic disorder characterized by problems breaking
down the amino acid tyrosine, which is a building block of most proteins.
With sever health problems.
❖ Untreated cases predispose HCC developing
❖ The incidence of HCC in tyrosinemia is reported to be 14%-75%

8. ADULT VS PEDIATRIC HCC - ARE THEY DIFFERENT DISEASES?
Adult HCC usually develops in the setting of
❖ chronic necro-inflammation going on for several years to
decades secondary to alcohol,
❖ viral hepatitis-B or C
❖ or non-alcoholic fatty liver disease NAFLD
pediatric HCC develops either in
❖ a cirrhotic or non-cirrhotic background
❖ or result of tyrosinemia or HBV effect.

9. ❖ Chronic hepatitis B virus infection is the leading cause of HCC worldwide
❖ HCC risk factors
aflatoxins are a family of toxins produced by certain fungi that are
found on agricultural crops such as maize (corn), peanuts,

10. Mutations in the TERT promoter are the most frequent genetic alterations,
accounting for approximately 60% of case
HCC-Genetic Alterations
• TERT promoter (~60%)
• TP53 (~30%)
• CTNNB1 (~30%)
• ARID1A (~10%)
• ARID2 (~5%)
• CCND1/FGF19* (5–10%)
• VEGFA* (5–10%)
• AXIN1 (~10%)
• The TP53 gene
provides instructions for
making a protein called
tumor protein p53 (or
p53).
• This protein acts as a
tumor suppressor

11. Diagnosis of childhood
hepatocellular carcinoma

12. Signs and symptoms of childhood hepatocellular carcinoma
• large abdominal mass or a swollen abdomen.
•pain on the right side that may extend to the back and shoulder.
•back pain from compression of the tumor.
•decreased appetite and weight loss. vomiting. jaundice. fever.
itching skin
Physical exam and health history:

13. Blood tests investigation in HCC
▪ CBC, LFT, Hepatitis assay-viral profile study
• Serum tumor marker test:
❖Alpha-Fetoprotein (AFP)
❖AFP-L3 -
❖AFP level > 400 ng/mL and AFP-L3 presence
❖PIVKA-II as a Potential New Biomarker for Hepatocellular
Carcinoma IN 80% of all cases
protein induced by vitamin K absence (PIVKA-II)

14. Alpha-Fetoprotein (AFP) and AFP-L3
❖ AFP can be elevated not only in the setting of HCC, but also in
chronic hepatitis, liver cirrhosis,
❖ AFP-L3 is a subfraction of AFP that is produced by malignant
hepatocytes
❖ The ratio of AFP-L3 to total AFP is reported as a percentage,
❖ and over 10% AFP-L3 is consistent with a diagnosis of HCC
❖ and HCC patients with positive AFP-L3 would have worse liver
function, poorer tumor histology, and larger tumor
❖ AFP used for detection and for monitoring the HCC disease course.

15. ❖ Has been described in relation to HCC
❖ It is released in association with vitamin K deficiency and in the
presence of HCC
❖ PIVKA-II is a useful tumor marker for HCC, complementary to
AFP.
❖ The diagnostic value of PIVKA-II in HCC was better than that of
AFP,
❖ and combined detection improved the diagnostic sensitivity and
specificity.
Protein induced by vitamin K absence (PIVKA-II)

16. HCC Route of metastasis
1-Lymphatic spread → to other lymph
nodes
2-Blood spread → lung –bones-adrenal
3-Direct infiltration →Through the body
wall into the abdominal and chest cavities
(transcoelomic → across the peritoneal
cavity

17. Metastasis is a multi-step process encompassing the local infiltration of tumor
cells into the adjacent tissue
5 major steps in metastasis
1-Invasion and infiltration of surrounding normal host tissue
2-Release of neoplastic cells
3-Survival in the circulation
4-Arrest in the capillary beds of distant organs
5-Penetration of lymphatic or blood vessel walls followed by growth of the
disseminated tumor cells

19. Staging of HCC- Barcelona Clinic Liver Cancer (BCLC) Staging System
a classification system should incorporate tumor size and number, presence of vascular
invasion and extrahepatic spread, liver function
The Child- Pugh classification is a means of assessing the severity of liver cirrhosis
ECOG it describes a patient's level of functioning in terms of their ability to care for themself,
daily activity, and physical ability (walking, working, etc.)

20. HCC – Barcelona Clinic Liver Cancer (BCLC) Staging
ECOG it describes a patient's level of functioning in terms of their ability to care for
themself, daily activity, and physical ability (walking, working, etc.)

21. Dysplastic nodules are defined as regenerative nodules containing atypical cells with
nuclear crowding and architectural derangement and a variable number of unpaired
arterioles or capillaries without definite histologic signs of malignancy

22. Staging of childhood hepatocellular carcinoma -PRETEXT

23. Staging of childhood hepatocellular carcinoma -COG

24. Imaging Features of Hepatocellular Carcinoma in Children
❖Ultrasound
❖Computed tomography –CT
give specific information about the
size, shape, and location of any tumors
in the liver or elsewhere in the
abdomen, as well as nearby blood
vessels
❖ Magnetic resonance imaging-MRI
• is most commonly used to detect hepatocellular carcinoma (HCC)
better detect lesions less than 1 cm in diameter

25. (CT) image -shows multiple arterially enhancing lesions within the hepatic parenchyma.
(HCC)

26. MRI –STUDY A 6-year-old male diagnosed with extensive multifocal HCC and a large left lobe mass as
demonstrated

27. ❖ metastases of HCC tend to spread through the intrahepatic vessels,
the lymphatic system, or by direct invasion.
❖ lung, peritoneum, bone, spleen, adrenal gland, brain, pleura and
kidneys
Metastasis of childhood hepatocellular carcinoma

28. Histopathology of hepatocellular carcinoma
Well-differentiated HCC moderately differentiated HCC poorly differentiated HCC

29. varies by the stage of disease, a person's likelihood to
tolerate surgery, and availability of liver transplant
❖Surgical resection
❖Liver transplantation
❖Ablation
❖Transcatheter arterial chemoembolization (TACE)
❖Radiotherapy in the treatment
Treatment of hepatocellular carcinoma

30. Prognosis
❖ The usual outcome is poor because only 10–20% of
hepatocellular carcinomas can be removed completely using
surgery
❖ If the cancer cannot be completely removed, the disease is
usually deadly within 3 to 6 months