Liver and Biliary Tract-Tumors

Liver and Biliary Tract-Tumors
Presentation lecture by:Inga Gurevich, MD, PhD

Liver - Benign Tumors and Tumor Like Conditions

* Focal nodular hyperplasia
* Liver cell adenoma
* Hemangioma (most common benign lesion)
* Mesenchymal hamartoma

Focal nodular hyperplasia (FNH)
* Common (#2 liver tumor after hemangioma)
* Mass lesion of young (median age 38 years); some studies show female predominance
* Represents 2-10% of pediatric hepatic tumors
* May be associated with oral contraceptives (66-95% of cases), hepatic cavernous hemangioma (20%), glycogen storage disease type Ia, portal hypertension
* Tumors associated with oral contraceptives often have hemorrhage, necrosis, infarction
* Usually an incidental finding; present in 1% of autopsies
* Xray: mass with central scar, centrifugal hypervascularity by angiography; CT and MRI are important,
* Treatment: excellent prognosis; discontinue oral contraceptives, if applicable; surgery if symptomatic, complications, compression of adjacent organs or lesion progression
* Well-demarcated, subcapsular, light brown to yellow ; bulging nodule, 70-80% solitary, up to 5 -10cm; has central gray-white stellate scar (unless < 1 cm) from which fibrous septa radiate to periphery and create multiple smaller nodules; hemorrhage, necrosis, infarction, bile staining often seen; larger tumors may have multiple scars; adjacent liver is normal
* Micro: Most tumors (80%) have the 3 classic features of abnormal architecture, bile ductular proliferation and malformed vessels.
* Non-classic forms lack either abnormal architecture or malformed vessels, and are divided into three types - (a) telangiectatic, (b) mixed hyperplastic and adenomatous or (c) atypia of large cell
* Positive stains: alpha-1-antitrypsin
* Negative stains: p53, CD143 (angiotensin I-converting enzyme: reduced expression
* DD: Osler-Weber-Rendu disease, Budd-Chiari syndrome or cirrhosis (adjacent liver is not normal), fibrolamellar hepatocellular carcinoma (marked atypia of hepatocytes), hepatocellular adenoma (encapsulated, monoclonal)

Liver Cell Adenoma
Hemangioma
Mesenchymal Hamartoma
Liver Cell Tumors - Malignant
* Hepatocellular carcinoma
* Hepatoblastoma
* Epithelioid hemangioendothelioma
* Angiosarcoma
* Undifferentiated (embryonal) sarcoma
* Squamous Cell Carcinoma
* Carcinoid Tumor
* Lymphoma/leukemia

Hepatocellular carcinoma
Clear cell variant of hepatocellular carcinoma
Fibrolamellar Variant of Hepatocellular Carcinoma
Hepatoblastoma
Epithelioid Hemangioendothelioma
Angiosarcoma
Undifferentiated Sarcoma
Bile Duct Tumors
Bile Duct Adenoma
Biliary Cystadenoma
Biliary Cystadenocarcinoma
Cholangiocarcinoma (intrahepatic)
case studies ..

Liver and Biliary Tract-Tumors.ppt