تشخیص

Hepatomegaly

Increased number of cells

Inflamation

Viral

Bacterial

Toxic

Storage disease

Fat (obesity,Reye syn,malnutrition,CF,DM,TPN,..)

Glycogen

Lipid storage (Neiman Pick,Guuaucher,Wolman)

Micellaneous(wilson ,Alpha 1 anti tripsin def,

Hepatomegaly

Increased number of cells

Infiltration

Primary tumors

Hepatoblastoma

HCC

Rhabdomyosarcoma

Angiosarcoma

Other sarcoma

Angiolipoma

Hamartoma

Adenoma

Teratoma

Biliary cyst

Angiomyolipoma

Hemangioma

Focal nodular hyperplasia

Hepatomegaly

Secondary tumors

Lymphoma

Leukemia

 Histiocytosis

Neuroblastoma

Wilms tumor

Hepatomegaly:

Increased size of vascular space

Intra hepatic obstruction (VOD, hepatic vein thrombosis ,web)

Supra hepatic  (CHF ,pericardial  diseases

Increased size of billiary space

Congenital biliary fibrosis

Caroli disease

Idiopathic

Hepatoblastoma

Mahshid Mehdizadeh MD

Pediatric Hematologist and Oncologist

Shahid Beheshti Medical University

Hepatoblastoma:

1-2% of childhood cancers

1.5/1000000 children<15 years old

2/3 of livers tumor

80% of cases reported before 3 years of age

 Median age 12-16 months

Male/Female 1.4- 2.0/1.0

The incidence is higher in far east countries

 an association between hepatoblastoma & prematurely is suggested

-Von Gierke disease

-Congenital cystathionuria and hemihypertrophy

-Beckwith-Wiedmann syndrom

-Trisomy 18

-Gardner syndrome

-Glycogen storage disease type 1

-Prader –Willi syndrome

-Familial adenomatosis poloposis

 -Metal & petroleum exposure

-Hormone therapy (OCP)

Hepatocellular carcinoma

-Cirrhosis

-Chronic carrier of hepatitis B

-Tyrosinemia

-Glycogen storage disease type 1

-Neurofibromatosis

-Alagille syndrom

-Alpha 1anti tripsin deficiency

=Wilms tumor teated with radiation

-Methotraxate

-Fanconi anemia

-Ataxia-telangiectasia

Geographic Distribution of HBV Carriers and Incidence of Hepatoma

Hepatoblastoma:

-Von Gierke disease

-Hemi hypertrophy

-Beckwith-Wiedmann syndrom

-Umbilical hernia

-Congenital absence of kidney

-Congenital absence of adrenal

-Meckels diverticulum

Epithelial type

Embryonal pattern

Fetal pattern

Macrotrabecular type

Small-cell undifferentiated type or anaplastic

Mixed epithelial and mesenchymal type

Hepatoblastoma:Clinical manifestations:

Abdominal mass                  80%

Abdominal distention           27%

Anorexia                               20%

Weight loss                          19%

Abdominal pain                    15%

Pallor                                    7%

Jaundice (rare)                     5%

Fever                                    4%

Diarrhea                               2%

Costipation

Precocious puberty in male(occ)                          1%

The clinical manifestations of hepatoblastoma and hapatocelluar carcinoma are similar

Hepatoblastoma: Diagnostic  evaluation:

-Extent of intrahepatic disease

Sonogrsphy

CT scan

MRI imaging

-Potential for hepatic resectability

-Presence or absence of extrahepatic disease

Lung

Bone

Bone marrow(very rarely)

CBC

Anemia

Thrombocytosis

Liver enzyme (usually normal or mildly elevated

Hepatitis B

AFP

Beta HCG

CEA

Hepatoblastoma:

AFP

is elevated in 90% of patients with hepatoblastoma and 50% of patient with HCC ( normal<10 ng/ml)

is a marker of monitoring residual and metastatic disease

 serial measurement should be done

Hepatoblastoma:Staging:

POG/CCG staging

1:completely resectable

2:Microscopic residual disease

3:Gross residual disease

4: Distant metastasis

International society of pediatric oncology staging

PRETEXT

Hepatoblastoma :treatment:

Surgury

Chemotherapy(5FU,vincristine, doxorubicin,cisplatin)

Radiation ?

Liver transplantation

Hepatoblastoma: prognosis :

Surgury and complete tumor resection

Degree of mitotic activity (pathology, flow cytometry)

Fetal histology(good)

Reduction of alpha feto protein by chemotherapy (unresectable tumors)

Diploid tumors are better