Flashcards in Module 2: GI: Liver Tumors and Gallbladder Deck (38):
The next topic for discussion is Liver Tumors. First tumor to discuss is Hemangioma, which is a benign tumor that will be discussed. What are the main features that need to be known.
1. Most common liver tumor
2. More prevalent in females
3. Asymptomatic; incidental finding
4. Vast majority require no treatment (However there are situations that you take out the tumor)
Moving onto the malignant tumors of the liver. The first is hepatic (liver cell) adenoma. What is the etiology of these tumors?
Oral contraceptive/anabolic steroids and glycogen storage diseases
--most common in young women
What investigations should be done for hepatic adenoma?
Normal AFP, AST, ALT
usually solitary and ill -defined (Without capsule)
proliferation of benign hepatocytes (plates greater than 2 cells thick)
No bile duct differentiation --- absence of portal tracts
What treatments are needed for hepatic adenoma?
Stop causative medication and resect tumor
What are complications associated with hepatic adenoma?
Intraparenchyml hemorrhage (which can be fatal in pregnancy)
The next benign tumor of the liver to be discussed his Focal Nodular Hyperplasia. What is the etiology?
What is seen on histology for focal nodular hyperplasia?
---Ill-defined area with a cirrhosis-like appearance and typically has a characteristic central stellate scar
---Proliferation of all 3 elements: benign hepatocytes (greater than 3 cells thick) & fibrous septa with inflammatory cells, bile ductules and prominent (Thick-walled) arteries
What is the non-neoplastic features of focal nodular hyperplasia?
Reactive/reparative process due to localized vascular abnormalities
---mistaken for HCC and adenoma
-no malignant transformation
Next tumor of the liver is a malignant tumor in children called Hepatoblastoma. What are the pre-disposing conditions for hepatoblastoma?
#1 liver tumor in children (around 18 months with M predominance)
---associated with Beckwith-Wiedemann Syndrome, Down syndrome, familial polyposis coli, hemihypertrophy and renal malformation
What is the pathogenesis for hepatoblastoma?
Single/multiple heterogeneous masses with rapid growth and poor prognosis
--spreads to lungs, LN and peritoneum
--patient presents with hepatomegaly or abdominal mass
What are the morphological features of hepatoblastoma?
--fetal (Best prognosis)
---mixed embryonal and fetal
Mixed Epithelial and Mesenchymal Type
--osteoid or cartilaginous differentiation
What investigations are done for hepatoblastoma?
AEP elevated (if negative, more aggressive)
The next malignant tumor to be discussed in the liver is Hepatocellular Carcinoma (HCC) aka Hepatoma. What are associated risk factors
Most common malignant tumor worldwide with M preponderance
---Most commonly due to a background of cirrhosis: Alcohol and Hep C/B and Hemochromatosis
--Exceptions: Aflatoxin from aspergillus (old nuts) goes straight to HCC no cirrhotic intermediate
What is the pathogenesis for HCC?
Solitary/multiple nodules that typically arise in a background of cirrhosis (mimics liver mets)
--trabecular type= nests and cords of hepatocytes
--Fibrolamellar Type: most common in young adults
---no association with viral hepatitis or cirrhosis
--Best prognosis of all types
--single hard tumor with fibrous bands
What is the clinical presentation for HCC?
Symptoms of cirrhosis
Bloody Ascites (because it spreads via blood via portal and hepatic vein)
What is seen on biopsy for a patient with HCC?
Biopsy most accurate
--Dont biopsy if its greater than 2 centimeters
(risk of spread, then just do excision/transplant)
HCC arises from what?
Arises from hepatic stem cells
--secrete bilirubin = jaundice and well differentiated in function
What is the tumor marker used for HCC?
--useful for follow up
Also seen very high in Yolk Sac Tumors (germ cell tumors) and heptoblastoma in kids
What are complications of HCC?
Invades vessels (bloody)
Completely bypasses lymph nodes (so does renal cell, follicular carcinoma of thyroid, gestational/non-gestational choriocarcinoma
Spreads to lungs, veins and bones
How can metastasis to the liver be differentiated from HCC?
In metastasis to the liver there is no background of cirrhosis (has a capsule and decreased liver function due to cirrhosis)
In regards to metastasis to the liver, what is the most common source of metastasis?
Colon is most common source
Metastasis is the most common cause of cancer in what two organs?
Liver and Brain
What is seen on histology for metastasis to the liver?
Shows cells of original tumor
What is the patient presentation with metastasis to the liver?
Asymptomatic for liver, symptomatic of primary tumor
--proceeds to jaundice and impaction of synthetic functions of liver
What is the AFP valve in metastasis?
Normal because liver function is normal
Next topic to be discussed is Cholangiocarcinoma ,which is carcinoma of the bile duct origin (intra or extra hepatic). What are some features of this carcinoma?
Extra-hepatic hilar cholangiocarcinoma = Klatskin Tumor
-seen in older adults with normal AFP
--poor prognosis (death within 6-12 months)
-adenocarcinoma with extensive fibrosis
What is cholangiocarcinoma associated with?
Caroli's Disease (Cystic dilations of biliary system)
Chemicals (thorotrast, benzidene, nitrosamines)
Parasitic Infections (Clonorchis sinensis and Opisthorchis viverini)
What is the presentation for a patient with cholangiocarcinoma?
Patients typically have non-cirrhotic livers and present with obstructive symptoms
--Jaundice, cholangitis (Charcot's Triad: jaundice, fever and chills), malaise and weight loss
--Raynaud Pentad: Charcot's triad + hypotension + change in mental status
Next topic to discuss is gallstones (Cholelithiasis). What is the etiology for gallstones?
Cholesterol Stones (80%)(cholesterol monohydrate):
--6 F's: female, fat (obesity), forty, fertile, fair skin and family history
--common in western/developed countries
Pigment Stones (20%)(calcium bilirubin salts):
--chronic hemolytic anemias (HS, B-thalassemia, and Sickle Cell Disease), Biliary Infections, GI disorders
--common in Asians and Rural communities
What are the signs and symptoms of a patient with gallstones (cholelithiasis)?
70-80% asymptomatic; can experience
--constant or coliky pain after a fatty meal in the RUQ that radiates to the scapula, nausea and vomiting
What is the best investigation for gallstones?
What are complications of gallstones?
1. Acute cholecystitis: inflammation of the gallbladder due to a stone in the cystic duct causing obstruction of flow
--patients will have severe pain RUQ (positive murphy's sign), nausea, vomiting and fevers (Virchows triad: fever, chilis and jaundice)
--can be a surgical emergency due to risk of perforation and peritonitis
2. Chronic Cholecystitis: can be due to repeated bouts of acute cholecystitis and overlaying bacteria
---thickening of the gallbladder wall
--Porcelain gallbladder: dystrophic calcification in the wall of the gallbladder --- this can develop into gallbladder adenocarcinoma
3. Acute Cholangitis (Acute inflammation of the wall of the bile duct due to obstruction of the common bile duct)
4. Acute Pancreatitis: due to obstruction of the common bile duct
5. Empyema of gallbladder
6. Fistula between gallbladder and duodenum, stone then comes out and goes into the ileocecal valve
Finally lets discuss Carcinoma of the gallbladder. Who gets this and what is the etiology?
--cholesterol gallstones most common predisposing factor
-chronic inflammation of the gallbladder -- porcelain gallbladder
--other causes: chronic salmonella typhi and chronic liver fluke infections
Adenocarcinoma is a KRAS mutation
Squamous is a p53 mutation
What is the presentation for carcinoma of the gallbladder?
Present when its already too late
--Jaundice, weight loss, anorexia in late stage
Gallbladder cancer spreads where first?
Invasion of the liver first site of metastasis
--then regional lymph nodes
What tumor marker is used in gallbladder carcinoma?
CA19-9 is tumor marker
--also seen in pancreatic and cholangio
What is seen on histology for gallbladder carcinoma?
Adenocarcinoma with malignant glands most common
--biopsy being the best investigation