neoplasms of liver, biliary tree and pancreas

any chronic liver condition will increase your risk for HCC- mostly after HCV tho HBV is most prevalent world wide. Fatty liver is another etiology.

Hepatitis is more common- Africa and Asia has hi prevalence of HCC

>95% of primary malignant neoplasms in liver
Global incidence and distribution strongly correlated with prevalence of HBV infection
- Endemic regions (Asia/Africa): HBV carriers from infancy; HCC often occurs in persons ages 20-40
- Western societies: cirrhosis precedes HCC in 80-90%; risk factors include ethanol, HCV, HBV, any chronic liver disease such as hemochromatosis

Often masked by cirrhosis; weight loss, malaise, abdominal pain/fullness, hepatomegaly
Serum alpha-fetoprotein: elevated in 50-70% cases (also elevated in chronic hepatitis, liver necrosis, cirrhosis, gonadal germ cell tumors)
Imaging: ultrasound, CT, MRI, angiography if surgical resection is considered
Treatment: complete surgical excision (before lung and nodal metastases) is only hope of cure
5 year survival all HCC: 5% (dismal)

Imaging studies
Ultrasound*
Computed tomography
No significant differences between spiral CT and MRI Stoker J, Gut 2002
Blood tests
Alpha-fetoprotein*

US q 6-12 months and AFP q 6 months is the most commonly used strategy in Asia and U.S.
Rationale for 6-month screening interval
Doubling time: median = 6 mo

JAUNDICE!

Female sex
Age > 50 years
Gallstones > 40 years (mostly cholesterol). High risk population such as Native Indians, Chile
Chronic cholecystitis
Calcified GB (porcelain GB)
-same as for benign gallbladder disease

Surgery:
Only chance for cure. Prophylactic cholecystectomy for asymptomatic gallstones not recommended, even if > 40 yrs
Chemotherapy and Radiation: Poor response
Palliation: ERCP for jaundice / pruritus

Overall mean survival of 6 months
5%, 5-year survival
Invasion usually local (contiguous spread)
Palliative management
If detected incidentally at time of cholecystectomy, then may survive long-term

risk factor for cancer

PSC-primary sclerosing cholangitis (assoc bw IBD and PSC) narrowing of ducts.
Congenital Hepatic fibrosis

klastskin tumor (hilum)-it is cholangiocarcinoma- name refers to location

Cholestasis, jaundice, pruritus, weight loss
Hepatomegaly; ascites, RUQ mass (late)
Cholangitis rare

Labs:
Cholestatic obstructive LFTs
Elevated serum CA19-9 (>100 U/ml) in 55-65%
US: dilated intrahepatic or extrahepatic duct; no mass detected
CT: dilated intra or extrahepatic ducts; usually difficult to identify mass lesion
ERCP: (can itself cause pancreatitis- MRCP is better)
Detect level of stricture
Brush cytology (sensitivity 50-55%)
Stenting for palliation or bridge to surgery

Familial Polyposis
Ampullary adenoma

Cholestatic jaundice
Pruritus
Intermittent bleeding
Cholangitis
Pancreatitis
Obstructive LFTs

CT or US: Dilated intra- & extra- hepatic ducts and no mass lesion or ?duodenal mass
ERCP: Usually diagnostic
Biopsies
Brushings for cytology
EUS: Staging

Important to distinguish from pancreatic cancer or cholangiocarcinoma
Surgical resection possible in 75%
Pancreatoduodenectomy (Whipple’s)

40% 5-year survival
Otherwise palliation:
Stenting
Ampullectomy

5-year survival rate is 2-5%
At presentation, most pts have advanced disease; jaundice if in the head (70%-80%), pruritus. Back pain (25%), recent onset of diabetes, anorexia. Trousseau’s (migratory thrombophlebitis), recent pancreatitis
CT scan detects tumors >3 cms. Obtain a pancreatic protocol CT
EUS is effective and most sensitive for small lesions< 2-3 cms
Allows EUS-guided FNA for tissue dx

Smoking
Chronic pancreatitis (sporadic < familial)
Juvenile onset DM
Recent onset of diabetes

Mostly arise from ductular cells
AdenoCa (>90%); islet cell (5%); cystadenocarcinomas
Head (76%); body (20%); tail (10%)

Pain. Nausea/vomiting. Anorexia, weight loss, jaundice
Mass; Palpable distended GB (Courvoisier’s sign)

Abdominal Ultrasound
Abdominal CT scan
Best study if suspicion is high
Abdominal MRI
ERCP
Best study to palliate with stenting
Endoscopic Ultrasound
Best study to find small lesions and to stage disease

Surgical: < ¼ are resectable; 1/10 are potentially curable
Chemo +/- radiotherapy
Palliation: stenting

No extrapancreatic disease

Unobstructed SMV-PV confluence

No tumor extension into Celiac or SMA
Clear fat plane between around Celiac and SMA
CT has 80% predictability using these criteria
Despite this, 5 year survival <20% and median
survival 15-17 months

Serous Cystadenoma, Intraductal papillary Mucinous Neoplasm, Mucinous Cystadenoma/carcinoma

Benign
Elderly women (>60); asymptomatic
Large lesions; multiloculated , multicystic(honeycomb)
Malignant transformation is rare

Premalignant, seen in elderly men, pancreatic duct obstruction

40-60 years of age
Unilobular or multilobular cyst containing mucin
Should be resected
Excellent prognosis if resected prior to carcinoma

Functional vs Non-functional
All endocrine tumors appear similar histologically. Immunos/peroxidase to distinguish subtype and biochemical data.
Malignancy based on the presence of local invasion, spread to regional LN, or liver/distant mets
Inuslinoma, VIPoma,gastrinoma, glucagonoma, somatistatinoma

Appreciate that HBV, HCV are implicated in HCC worldwide. US and AFP levels are recommended for screening.
Understand the risk factors for gallbadder cancer, and cholangiocarcinomas. Think of associated diseases
Distinguish ampullary from pancreatic cancer;similar presentations, but markedly different survival rates
Recognize that cysts in the pancreas are not necessarily pseudocysts. Beware of the mucinous cyst
Recognize that neuoreondocrine neoplasms of the pancreas are characterized by the hormone produced.