Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + • Angiosarcoma• Hepatoblastoma• Hepatic adenocarcinoma• Intrahepatic cholangiocarcinoma +++ Epidemiology + • Hepatoblastoma mainly in children• Frequency increased in Beckwith-Wiedemann syndrome hemihypertrophy, fetal alcohol syndrome, and parenteral nutrition• Angiosarcoma and sarcoma risk factors-Vinyl chloride-Arsenic-Androgenic steroids +++ Symptoms and Signs + • Right upper quadrant pain• Weight loss• Hepatomegaly• Abdominal mass in childhood• Hepatic bruit• Ascites• Distant metastases• Asymptomatic• Intraperitoneal hemorrhage• Decompensated cirrhosis +++ Laboratory Findings + • Hyperbilirubinemia• Elevated transaminases• Elevated alkaline phosphatase +++ Imaging Findings + • CT scan-Hypervascular tumor (for angiosarcoma)-Multi-septate cyst for adenocarcinoma + • Biopsy of limited usefulness given high degree of sampling error and risk of tumor seeding• Overall and planned reserve liver function +++ Rule Out + • Metastatic carcinoma• Benign neoplasms if possible• Other abdominal malignancy + • CT scan• US• Angiography• Biopsy if unresectable• Resection for diagnosis and treatment +++ When to Admit + • Intrahepatic hemorrhage• Decompensated cirrhosis +++ Surgery + • Resection +++ Indications + • Localized tumor +++ Contraindications + • Poor liver function• Extrahepatic disease• Diffuse hepatic disease +++ Medications + • Chemotherapy for unresectable lesions +++ Treatment Monitoring + • CT surveillance +++ Complications + • Hemorrhage• Biliary leak• Abdominal infection• Liver failure +++ Prognosis + • Resectable cholangiocarcinoma: 5-year survival, 15-20%• Angiosarcoma: Very poor• Hepatoblastoma: Overall survival 50% +++ References ++Endo I et al: Intrahepatic cholangiocarcinoma: rising frequency, improved survival, and determinants of survival after resection. Ann Surg 2008; 247:994. ++Patel T: Increasing incidence and mortality of primary intrahepatic cholangiocarcinoma in the United States. Hepatology. 2001;33:1353. [PubMed: 11391522] +++ Practice Guidelines + • The National Comprehensive Cancer Networkhttp://www.nccn.org/ Your Access profile is currently affiliated with [InstitutionA] and is in the process of switching affiliations to [InstitutionB]. Please select how you would like to proceed. Keep the current affiliation with [InstitutionA] and continue with the Access profile sign in process Switch affiliation to [InstitutionB] and continue with the Access profile sign in process Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Error: Incorrect UserName or Password Username Error: Please enter User Name Password Error: Please enter Password Sign in Forgot Password? Forgot Username? Sign in via OpenAthens Sign in via Shibboleth You already have access! Please proceed to your institution's subscription. Create a free profile for additional features.