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30 Cards in this Set
- Front
- Back
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What hormones does the pancreas secrete?
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Pancreatic Hormones:
Insulin Glucagon Somatostatin Gastrin |
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What kinds of tumors occur in the pancreas?
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Pancreatic Tumors?
95% are adenocarcinomas of the pancreas. Gastrinomas (Zollinger-Ellison Syndrome seen with MEN 2) Insulinomas |
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What is the average age of onset of pancreatic cancer?
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average age of onset of pancreatic cancer
72 years |
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What is the average 5-year survival rate for pancreatic cancer?
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5-year survival rate for pancreatic cancer
5.6% |
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Name the High Risk Factors for Pancreatic Cancer (3)
(> 10 fold increase for pancreatic cancer) |
High Risk Factors for Pancreatic Cancer (3)
Hereditary Pancreatitis (Not a genetic risk factor - it is d/t pancreatic damage) 3 or more relatives with pancreatic cancer Having mutations in CDKN2A or STK11 |
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Name the Moderate Risk Factors for Pancreatic Cancer (5-10 fold increase)
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Moderate Risk Factors for Pancreatic Cancer (5-10 fold increase)
Cystic Fibrosis Chronic Pancreatitis 2 Relatives BRCA2 |
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Name the slightly Increased Risk Factorrs (>5 fold increase) for Pancreatic Cancer.
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the slightly Increased Risk Factorrs (>5 fold increase) for Pancreatic Cancer.
Male Ancestry (African or Ashkenazi Jewish) Obesity Smoking Diabetes H. Pylori 1 Relative APC/ HNPCC/ BRCA1 |
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For those in the "high risk" category of pancreatic cancer what is the lifetime risk of developing Pancreatic Cancer?
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30-40%
Hereditary Pancreatitis 3 or more relatives CDKN2A, STK11 |
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Pancreatic Cancer. What do the following mean?
Localized Regional Metastatic |
Pancreatic Cancer.
Localized = no nodes Regional = local nodes Metastatic = distant nodes or further spread |
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Currently what percent of pancreatic cancer is diagnosed:
Localized Regional Metastatic |
Currently percent of pancreatic cancer is diagnosed:
Localized 8% Regional 26% Metastatic 53% |
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What is the tumor marker for pancreatic cancer?
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tumor marker for pancreatic cancer
Ca 19-9 |
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How can you screen those at high risk for pancreatic cancer?
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Pancreatic Cancer Screening
Annual CA 19-9 & Every 1-2 years Imaging: CT (radiation risk) MRCP (Magnetic REsonance Choliangopancreatography) EUS (endoscopic Ultrasound |
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What is the only gene that we know at this time that predisposes to ancreatic cancer?
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Pancreatitis Gene
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How much of pancreatic cancer is hereditary?
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5% - 10% of Pancreatic cancer is hereditary, rest is sporadic
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BRCA and Pancreatic Cancer:
Who can you screen for BRCA when Pancreatic Cancer is involved? |
Someone with personal history of breast and/or ovarian cancer at any age with two or more close relatives with pancreatic cancer.
Personal history of pancreatic cancer at any age with 2 or more close relatives with breast and/or ovarian and/or pancreatic cancer at any age |
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Only one gene has a clear, prognositic association with hereditary pancreatitis.
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Hereditary Pancreatitis
PRSS1 gene |
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Hereditary Pancreatitis
With a PRSS1 Mutation what is the average age of onset of pancreatitis? |
Hereditary Pancreatitis
PRSS1 Mutation average age of onset of pancreatitis 10-14 |
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Hereditary Pancreatitis
With a PRSS1 Mutation what is the pattern of inheritance? |
Hereditary Pancreatitis
PRSS1 Mutation Autosomal Dominant |
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Hereditary Pancreatitis
With a PRSS1 Mutation what is the lifetime risk for pancreatic cancer? |
Hereditary Pancreatitis
With a PRSS1 Mutation lifetime risk for pancreatic cancer 40% |
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Hereditary Pancreatitis
Clinical Diagnostic Criteria |
2 1st degree relatives or
3 2nd degree relatives over 2 generations with recurrent acute pancreatitis or chronic pancreatitis without another identifiable cause. |
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There are other genes associated with pancreatitis (CFTR, SPINK1, CTRC) why don't we test those in patients with chronic recurring pancreatitis?
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These other genes are not very prognositc for pancreatic cancer because of very low penetrance.
These other genes are considered modifying factors for pancreatitis rather than causative factors. |
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The CFTR Gene (Cystic Fibrosis Transmenbrane Regulator) increases risk for pancreatitis. What is the increase in risk for:
*carriers of a single CFTR mutation *Compound heterozygotes |
The CFTR Gene increases risk for pancreatitis.
*carriers of a single CFTR mutation = 3-4 fold increase *Compound heterozygotes - 40 - 80 fold increase |
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The SPINK1 gene (Serine Protease Inhibitor of the Kazal type) increaes chronic pancreatitis by how much?
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SPINK 1 gene increases chronic pancreatitis risk
by 12 fold |
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What other Hereditary Syndromes increase the risk for Pancreatic Cancer?
(give lifetime risk) |
Increased risk for Pancreatic Cancer (& lifetime risk)
Peutz-Jeghers (36%) FAMM Familial Atypical Melanoma (p16) (11-17%) BRCA 2 (3-8%) Familial Pancreatic Cancer (PALLD) (unclear) |
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If a woman has a BRCA 2 mutation should we do pancreatic cancer screenings?
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We have not been offering that.
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What cancers are seen in Peutz-Jeghers? (include prevalence)
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Peutz-Jeghers
Colon (39%) Pancreatic (36%) Stomach (29%) Breast (54%) |
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Do we offer pancreatic cancer screening in Peutz-Jeghers?
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Yes - Peutz-Jeghers offer Pancreatic screening every 1-2 years starting at age 30
Colonoscopy every 1-3 years starting at age 25 Upper endoscopy and small bowel exam every 2 years starting between age 8-10 Mammograms every 2 years between age 20-40; annually thereafter (women only) Annual uterine cancer screening starting at 20 |
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What cancers are associated with Ataxia Telangiectasia (ATM gene)?
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Ataxia Telangiectasia (ATM gene)
lymphoma leukemia breast pancreas colon stomach |
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What non-cancer features are associated with Ataxia Telangiectasia (ATM gene)?
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Ataxia Telangiectasia (ATM gene)
neurodegeneration (ataxia) occular/ facial telangiectasia dysarthria |
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What is the PALLD gene?
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mutation in PALLD is associated with pancreatic cancer.
Appears to be a proto oncogene - |
