PEDBASE.org - The Pediatric Database - Detailed information of HEPATOCELLULAR CARCINOMA

HEPATOCELLULAR CARCINOMA

DEFINITION:

A malignant tumor of the liver characterized by a mass in the right upper quadrant.

EPIDEMIOLOGY:

incidence: ?
age of onset:
- median: 9-10 years; two peaks: <4 years and between 12-15 years
risk factors:
- see below
- M > F (1.3:1)

PATHOGENESIS:

1. Background

also called hepatoma
in childhood, hepatocellular carcinomas are associated with essentially all types of cirrhosis, (i.e., cirrhosis due to malnutrition, biliary atresia, giant cell hepatitis, etc.)
HBsAg acquried through perinatal transmission may result in the development of a hepatocellular carcinoma by 10 years of age

2. Conditions Predisposing to Hepatoma

1. Gastrointestinal

Hepatitis B & C
Neonatal Hepatitis
Biliary Atresia
Intrahepatic Cholestasis

2. Metabolic

Tyrosinemia-I
Von Gierke Disease
Alpha-1-Antitrypsin Deficiency

3. Drugs

Methotrexate
Androgenic Anabolic Steroids (Fanconi Anemia)

4. Others

Ataxia Telangiectasia
Neurofibromatosis

CLINICAL FEATURES:

1. Gastrointestinal Manifestations

abdominal mass +/- distension
abdominal pain
hepatomegaly/splenomegaly
anorexia and weight loss
jaundice or fever (in 15-20%)
hemoperitoneum due to tumor rupture (in 5%)

2. Metastases

lung
bone marrow
regional lymph nodes

INVESTIGATIONS:

1. Diagnostic

1. Liver Biopsy

tumor invasive, usually multicentric and bile stained
large well differentiated tumor cells, broad trabeculae, tumor giant cells, nucleolar prominence
a fibrolamellar subgroup

2. Imaging Studies

1. Abdominal Ultrasound/CT/MRI

solid tumor

2. Chest X-Ray/CT/MRI

for pulmonary metastases

3. Serum

CBC - mild anemia, thrombocytosis (in 10%)
LFT - elevated AST, ALT, LDH, alkaline phosphatase
serum alpha-fetoprotein level may be elevated (but not to the same degree as in HEPATOCELLULAR CARCINOMA)

4. Bone Marrow

for metastases

MANAGEMEMT:

1. Surgery

1. Lobectomy

right and left partial and complete lobectomy and trisegmentectomy

2. Liver Transplantation

experimental

2. Chemotherapy

1. Adjuvent Therapy

used pre and post resection
can reduce the size of the primary tumor and metastases
doxorubicin and cisplatin

3. Prognosis

5 year survival 13% (only with tumors which have been totally resected)

Pediatric Database - HEPATOCELLULAR CARCINOMA

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