Hepato-biliary-pancreatic neoplasia Flashcards
How common is hepatocellular cancer?
third most common cause of cancer worldwide
What is the commonest cause of HCC worldwide?
chronic hepatitis B
What is the most common cause of hepatocellular carcinoma in Europe?
chronic hepatitis C
What is the main risk factor for developing hepatocellular carcinoma?
liver cirrhosis, e.g. secondary to hepatitis B and C, alcohol, haemochoromatosis, and primary biliary cirrhosis
What are 12 causes of liver cirrhosis which can predispose to developing HCC?
- Hepatitis B+C
- Alcohol
- Haemochromatosis
- Primary biliary cholangitis
- Alpha-1 antitrypsin deficiency
- Hereditary tyrosinosis
- Glycogen storage disease
- Aflatoxin
- Drug: oral contraceptive pill, anabolic steroids
- Porphyria cutanea tarda
- Male sex
- Diabetes mellitus/ metabolic syndrome
When in the course of hepatocellular carcinoma does the disease tend to present?
late
What are 7 possible clinical features of a patient with hepatocellular carcinoma?
- Jaundice
- Ascites
- Right upper quadrant pain
- Hepatomegaly
- Pruritus
- Splenomegaly
- Could present as decompensation in a patient with chronic liver disease
What tumour marker may be raised in hepatocellular carcinoma?
AFP (alpha feto protein)
How is screening performed for hepatocellular carcinoma?
ultrasound with/without alpha fetoprotein
What are 2 examples of high risk groups who should be considered for screening for hepatocellular carcinoma?
- Patients with liver cirrhosis secondary to hepatitis B & C or haemochromatosis
- Men with liver cirrhosis secondary to alcohol
What are 5 management options for hepatocellular carcinoma?
- Early disease: surgical resection
- Liver transplantation
- Radiofrequency ablation
- Transarterial chemoembolisation
- Sorafenib: multikinase inhibitor
At what point in the disease course is pancreatic cancer often diagnosed and why?
late, tends to present in a non-specific way
What type of tumour is the vast majority of pancreatic cancers?
adenocarcinomas - 80%
Where in the pancreas does the most common type of pancreatic cancer occur?
head of pancreas (adenocarcinomas)
What are 8 things that pancreatic cancer is associated with?
- Increasing age
- Smoking
- Diabetes
- Chronic pancreatitis (alcohol doesn’t appear an independent risk factor though)
- Hereditary non-polyposis colorectal carcinoma
- Multiple endocrine neoplasia
- BRCA2 gene
- KRAS gene mutation
What is the classic presentation of pancreatic cancer?
painless jaundice; pale stools, dark urine, pruritus
cholestatic LFTs
What does Courvoisier’s law state in regards to pancreatic cancer?
in the presence of painless obstructive jaundice, a palpable gallbladder is unlikely to be due to gallstones
What are 6 less common/atypical features of the presentation of pancreatic cancer?
- Anorexia
- Weight loss
- Epigastric pain
- Loss of exocrine function: steatorrhoea
- Loss of endocrine function: diabetes mellitus
- Atypical back pain
What is a sign seen in pancreatic cancer that is more common than with other cancers?
migratory thrombophlebitis - Trousseau’s sign of malignancy
thrombophlebitis that travels/comes back in different part of body, often from one leg to other
What are 2 types of cancers that Trousseau’s sign (migratory thrombophlebitis) is most commonly seen in?
pancreatic and lung
What are 2 key investigations to perform to diagnose pancreatic cancer?
- Ultrasound
- High reslution CT scanning
What is the investigation of choice if a diagnosis of pancreatic cancer is suspected?
high-resolution CT scanning
What may be seen on imaging in pancreatic cancer?
double duct sign: presence of simultaneous dilatation of the common bile duct and pancreatic ducts
What is the 5 year survival rate like of pancreatic cancer?
extremely poor: <5%
What is Courvoisier’s sign in pancreatic cancer?
painless palpable gallbladder with jaundice
What are 3 sites where pancreatic cancer metastasises to?
lung, liver, bowel
(haemoptysis, jaundice, constipation)
What are LFTs likely to show in pancreatic cancer?
raised bilirubin and raised ALP
What are the limitations of using ultrasound to investigate suspected pancreatic cancer?
good at detecting tumours within head of pancreas but poorer for tumours in body or tail and in determining early disease - so normal US doesn’t rule out pancreatic carcinoma
What are 2 types of imaging that can give further information in pancreatic cancer (in addition to US and HRCT)
MRCP: information about biliary ducts but cannot show extension of tumour
Endoscopic USS: invasive test which can detect small lesions and can be used for biopsies
What is the only potentially curative treatment for pancreatic cancer?
resection of tumour
What proportion of patients will present with resectable pancreatic cancer?
only 15-20%
What are 2 examples of criteria for resection of pancreatic cancer?
- No evidence of involvement of superior mesenteric artery (SMA) or coeliac arteries
- No evidence of disant metastases
What is the most common procedure used to resect the pancreatic tumour if it’s in the head of the pancreas?
pancreaticoduodenectomy (Kausch-Whipple procedure), adjuvant chemotherapy given after if recover from surgery well
What are 2 side effects of pancreaticoduodenectomy for pancreatic cancer (aka Whipple’s resection/ Kausch-Whipple procedure)?
- Dumping syndrome
- Peptic ulcer disease
What adjuvant therapy is usually given following surgery for resectable pancreatic cancer?
chemotherapy
What are 4 options for palliative therapy for pancreatic cancer with locally advanced or metastatic disease?
- Endoscopic stent insertion into bile duct
- Palliative surgery if endoscopic stent insertion fails
- Chemotherapy
- Radiotherapy (only for localised advanced disease)
In addition to palliative therapeutic options what else must be provided for patients with non-resectable pancreatic cancer?
pain management and mental and emotional support - refer to palliative care
Why is the prognosis of gallbladder cancer poor?
non-specific symptoms so commonly diagnosed incidentally and at late stage
What type of cancer are the majority of gallbladder cancers?
adenocarcinomas (90%)
What patients groups is gallbladder cancer most common in?
increases with age - most common over age 75
more common in females
more common in certain ethnic groups: Caucasians, Native Americans, Koreans
What are 2 main groups of risk factors for gallbladder cancer?
- gallbladder and biliary tree pathologies
- environmental/lifestyle factors
What are 5 gallbladder/biliary tree pathology risk factors for gallbladder cancer?
- Gallstones or history of gallstone disease
- Porcelain gallbladder
- Gallbladder adenoma (polyps)
- Abnormal bile duct anatomy
- Primary sclerosing cholangitis
What is meant by the term porcelain gallbladder?
intramural gallbladder wall calcification which occurs as a result of chronic cholecystitis
What is meant by abnormal bile duct anatomy which can increase the risk of gallbladder cancer?
certain people have congenital defect of bile ducts which allows reflux of pancreatic secretions into biliary tree, which can cause damage
What are 3 environmental/lifestyle factors which increase the risk of gallbladder cancer?
- Obesity
- Infection causing chronic cholangitis: salmonella typhi and Helicobacter
- Carcinogen exposure: cigarette smoking and exposure to industrial chemicals
What are the 2 most common infections which can cause chronic cholangitis and predispose to gallbladder cancer?
- Salmonella typhi
- Helicobacter
How is early-stage gallbladder most often detected?
often asymptomatic and found incidentally during surgery
What proportion of gallbladder cancers are found incidentally during surgery?
20%
What proportion of all patients undergoing routine cholecystectomy will have gallbladder cancer?
1%
What are 6 of the symptoms of gallbladder cancer once it becomes symptomatic?
- Right upper quadrant pain
- Anorexia
- Nausea and vomiting
- Malaise
- Weight loss
- Palpable right upper quadrant mass
- Obstructive jaundice (depending on tumour location): yellowing of skin and sclera, pale stools dark urine, pruritus
What may LFTs show in gallbladder cancer?
raised ALP and bilirubin
What are 2 types of tumour markers which may be raised in later-stage gallbladder cancer?
- Ca-19-9
- CEA
How useful are tumour markers for investigating gallbladder cancer?
may be elevated in later-stage disease but this is non-specific and cannot be relied upon for diagnosis
How useful is ultrasound scanning for diagnosing gallbladder cancer?
can appear as mass, polyp or thickening of gallbladder wall on USS, often fails to visualise early-stage disease
What is the most useful imaging modality for diagnosis and staging of GBC, that is the best first-line investigation?
CT scanning
Why is CT the best first-line investigation for gallbladder cancer?
can detect masses and identify lymphadenopathy and tumour invasion into adjacent structures
In addition to CT what other imaging modality can be used for gallbladder cancer pre-operatively and what for?
MRI: to assess extent of disease, good at detecting vascular invasion (MR angiography) and biliary tract involvement (MRCP) for staging
What is the gold standard investigation for gallbladder cancer staging and what does it allow?
endoscopic ultrasound - allows for fine-needle aspiration biopsy for histological analysis of the tumour
What is the staging system used for gallbladder cancer and what are the 5 stages?
TNM staging
- Tis: carcinoma in situ
- T1: tumour invades lamina propria and/or muscular layer of gallbladder
- T2: tumour invades perimuscular connective tissue without involvement of visceral peritoneum or liver
- T3: tumour invades visceral peritoneum and/or liver and/or one adjacent organ (e.g. stomach, pancreas, bile ducts, intestines)
- T4: tumour invades vascular structures (hepatic artery or portal vein) and/or invades two or more adjacent organs
What is the only curative therapy for gallbladder cancer?
surgical resection
What are 3 situations when surgical resection of gallbladder cancer is contraindicated?
- Metastatic disease: liver, peritoneum and intestines most commonly affected
- Malignant ascites
- Vascular invasion: superior mesenteric artery, coeliac artery, hepatic artery, portal vein are common sites
What are 3 common sites of metastatic gallbladder cancer?
- liver
- peritoneum
- intestines
What are 4 common sites of vascular invasion of gallbladder cancer?
- Superior mesenteric artery
- Coeliac artery
- Hepatic artery
- Portal vein
What therapy for gallbladder cancer do patients with surgically unresectable disease often receive?
chemoradiotherapy
What is the limitation of chemoradiotherapy for gallbladder cancer management?
mixed success; mainly useful for palliation
What are 2 types of palliative therapy for gallbladder cancer?
- Chemoradiotherapy
- Endoscopic common bile duct stents - if obstructive jaundice
What is the method of surgical resection for gallbladder of TNM stage Tis/T1?
simple cholecystectomy - removal of gallbladder
What is the method of surgical resection for gallbladder of TNM stage T2?
resection of gallbladder with surrounding 2cm resection of adjacent tissue. also includes removal of surrounding lymph nodes
What is the method of surgical resection for gallbladder of TNM stage T3?
extended cholecystectomy (surrounding 2cm and lymph nodes) plus resection of affected adjacent organ
Which types of gallbladder carcinoma are typically unresectable?
T4, M1
What management can be performed for T4 gallbaldder cancer?
chemoradiotherapy
What management can be performed for N1/2 gallbladder cancer?
local lymph node resection - all surrouding nodes (cystic duct, common bile duct, hepatic artery, portal vein, hepatoduodenal ligaments)
What is the prognosis of gallbladder cancer like?
poor; T1 87% 3 year suvrival but often diagnosed at late stage - T4 0% 3 year survival
What is cholangiocarcinoma?
bile duct cancer
What is the main risk factor for cholangiocarcinoma?
primary sclerosing cholangitis
What are 7 clinical features of cholangiocarcinoma?
- Persistent biliary colic symptoms
- Anorexia
- Jaundice
- Weight loss
- Palpable mass in the right upper quadrant (Courvoisier sign)
- Periumbilical lymphadenopathy (Sister Mary Joseph nodeS)
- Left supraclavicular adenopathy (Virchow node)
What are Sister Mary Joseph nodes?
periumbilical lymphadenopathy
What is Virchow node?
supraclavicular lymphadenopathy
