HepatoblastomaBy mahyarHistory: 4 Y/ M with swollen abdomen and Decreased appetite Findings:
:: CT 1 ::First CECT
:: CT 2 ::First CECT CECT emonstrates mass that appears to be multinodular, with heterogeneous enhancement less than that of the surrounding liver
:: CT 3 ::CT without IV contrast after 2 months Precontrast CT scan pr shows a large mass encompassing both lobes of the liver, which is of lower density than normal liver.
:: CT 4 ::CECT after 2 months
:: Fluoroscopy 1 ::celiac artery DSA
:: Fluoroscopy 2 ::SMA DSA
:: Fluoroscopy 3 ::portal-venous phase or delayed phase DSA of the SMA Discussion: Hepatoblastoma is a rare tumor (an abnormal tissue growth) that originates in cells in the liver. It is the most common malignant liver tumor in early childhood. Most hepatoblastoma tumors begin in the right lobe of the liver. Hepatoblastoma cancer cells also can spread to other areas of the body. The most common site of metastasis is the lungs. Hepatoblastoma primarily affects children from infancy to about 5 years of age. Most cases appear during the first 18 months of life. Hepatoblastoma affects white children more frequently than black children, and is more common in boys than girls up to about age 5, when the gender difference disappears. It occurs more frequently in children who were born very prematurely (early) with very low birth weights. Although the exact cause of liver cancer is unknown, there are a number of genetic conditions that are associated with an increased risk for developing hepatoblastoma. They include: 1. Beckwith-Wiedemann syndrome: this syndrome is characterized by a combination of Wilms tumor, kidney failure, genitourinary malformations and gonad (ovaries or testes) abnormalities. 2. Familial adenomatous polyposis and Gardner syndrome : this is a group of rare inherited diseases of the gastrointestinal tract. 3. Hemihypertrophy. This condition is the faster growth of one limb on one side of the body in comparison with the other side. 4. foetal alcohol syndrome 5. prematurity and low foetal birth weight 6. glycogen storage disease Children who are exposed to hepatitis B infection at an early age, or those who have biliary atresia, are also at increased risk for developing liver cancer. signs and symptoms: The signs and symptoms of hepatoblastoma often depend on the size of the tumor and whether it has spread to other parts of the body. Symptoms may include: 1. A large mass in the abdomen 2. Swollen abdomen 3. Weight loss 4. Decreased appetite 5. Vomiting 6. Jaundice (yellowing of eyes and skin) 7. Itchy skin 8. Anemia 9. Back pain Pathology Macroscopically hepatoblastomas are usually relatively well circumscribed masses, usually single, with heterogeneous cut surface. Histological classification of hepatoblastoma is complicated with multiple subtypes identified. The notable subtypes include: 1. epithelial : most common 2. small cell undifferentiated : worst prognosis 3. mixed type : calcifications more common in mixed type. It is important to note however, that with the possible exception of small cell undifferentiated subtype, prognosis is independent of histology when adjusted for stage gender and age. Radiographic features Plain film: Abdominal x rays are non-specific, typically demonstrating a right upper quadrant mass. Calcifications are visible in 10% of cases. Ultrasound: On ultrasound, hepatoblastomas appear as predominantly echogenic soft tissue mass. In larger tumours heterogeneity and variable echogenecity is common. Even when large, they tend to be relatively well defined . Intra-lesional calcifications may be visible as areas of shadowing. CT: Usually seen as a well defined heterogeneous mass, which is usually hypoattenuating compared to surrounding liver. Frequently there are with areas of necrosis and haemorrhage. Chunky, dense calcifications may be seen in approximately 40% of cases. CT is also able to evaluate the lungs for metastases and for nodal enlargement. MRI: MRI is superior to CT in defining tumour margins, vessel involvement and adenopathy. 1. T1 : generally hypo intense 2. C+ (GAD) : can show heterogeneous enhancement 3. T2 : I. generally hyper intense compared to liver II. areas of necrosis and haemorrhage are common. Angiography: -Tend to be hypervascular. Treatment and prognosis Surgical resection is the treatment of choice although preoperative chemotherapy may be used to reduce tumour bulk. Chemotherapy is also employed in following surgical resections. If the tumour is reselectable then liver transplantation provides a cure, as long as no metastatic disease is present. The lungs are a relatively frequent site of metastases. Differential diagnoses: mesenchymal hamartoma infantile haemangioendothelioma hepatic metastases : e.g. neuroblatoma hepatocellular carcinoma (HCC) rhambdomyosarcoma of biliary tract (rare) Differential diagnosis: mesenchymal hamartoma infantile haemangioendothelioma hepatic metastases : e.g. neuroblatoma hepatocellular carcinoma (HCC) rhambdomyosarcoma of biliary tract (rare) Diagnosis confirmation: Surgery / Histo Category: Pediatric Region / Organ: Abdomen-Liver Etiology: neoplastic References: 1. Herzog CE, Andrassy RJ, Eftekhari F. Childhood cancers: hepatoblastoma. Oncologist. 2000;5 (6): 445-53. 2. Dachman AH, Pakter RL, Ros PR et-al. Hepatoblastoma: radiologic-pathologic correlation in 50 cases. Radiology. 1987;164 (1): 15-9 3. Stoupis C, Ros PR. Imaging findings in hepatoblastoma associated with Gardner s syndrome. AJR Am J Roentgenol. 1993;161 (3): 593-4 Get more for Hepatoblastoma Peer-reviewed resources Literature  More publications about HepatoblastomaChallenge yourself with the case quiz! |