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Handbook of Genetic Counseling/Pancreatic Cancer

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Pancreatic Cancer

Pancreatic Cancer

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  • Incidence is more than 29,000 new cases/year in US
    • Fifth leading cause of cancer death
    • 4.1% five-year survival rate is lowest of all cancers
    • About 9-10% of pancreatic cancer is familial
  • Pancreas is a gland located in the abdomen between the stomach and the spine
    • Responsible for producing insulin, glucagon, and other hormones
    • Releases digestive juices through series of ducts leading to common bile duct
  • Risk factors for pancreatic cancer
    • Smoking - increases risk 2-3 times
    • Age - most cancers diagnosed between 60 and 80 years
    • Diabetes
    • Chronic pancreatitis
      • Long-term inflammation of the pancreas
      • Especially inherited form of the condition
    • Family history
      • Risk increases by 4.7% if first degree relative has been diagnosed
      • Increases by 7.3% if diagnosis was made before age 60
      • Increases by 12.3% if multiple primary cancers in family
    • Males and African-Americans may be at increased risk
    • Diets high in fat may increase risk

Diagnosis and Surveillance Options

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  • Possible symptoms
    • Pain in upper abdomen or upper back
    • Yellowing of skin and eyes, dark urine due to jaundice
    • Weakness
    • Loss of appetite
    • Nausea and vomiting
    • Weight loss
  • Physical exam
    • Look for signs of jaundice or changes in area near pancreas
    • Check for ascites (build-up of fluid in abdomen)
  • Lab tests
    • Blood, urine, and stool samples
    • Check for bilirubin that may be high if tumor blocking common bile duct prevents its passage
  • CT Scan
    • Produces detailed picture of abdomen
    • Can provide information about nature and location of tumors, resectability
  • Ultrasonography
    • Transabdominal ultrasound
    • Endoscopic ultrasound (EUS)
      • Pass thin, illuminated optic instrument down mouth into stomach
      • Ultrasound device at tip of endoscope takes images of pancreas and surrounding areas
  • Endoscopic Retrograde Cholangiopancreatography (ERCP)
    • Pass endoscope through mouth and stomach into first part of small intestine
    • Inject dye through catheter into ducts
    • Take X-rays to determine if ducts are narrowed or blocked by tumor
  • Percutaneous Transhepatic Cholangiography (PTC)
    • Dye injected through needle inserted through skin into liver
    • Dye moves freely through bile ducts unless blocked
    • X-rays can show if dye has moved through ducts
  • Biopsy
    • Fine-needle aspiration
    • During EUS or ERCP
    • Open abdomen during operation
  • Laparoscopy
    • Uses several small incisions and TV camera to visualize organs in abdomen
    • Usually only used if CT scan shows evidence of metastases

Treatment

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  • Pancreatic cancer is currently difficult to treat so participation in clinical trials is often recommended
  • Surgery
    • May be used alone or in combination with radiation or chemotherapy
    • Whipple procedure
      • If tumor is in head of pancreas
      • Remove head and part of small intestine, bile duct, and stomach
    • Distal pancreatectomy
      • Surgeon removes body and tail of pancreas
      • May also remove the spleen
    • Total pancreatectomy
      • Remove entire pancreas
      • Also remove part of small intestine and stomach, and all of common bile duct, gallbladder, spleen, and nearby lymph nodes
    • Create bypass or insert stent if tumor is blocking common bile duct or duodenum
      • Relives jaundice and pain resulting from blockage
      • Done if cancer cannot be completely removed surgically
    • Side effects of removal of all or part of pancreas
      • Diet control and medication may help with food digestion
      • Diarrhea, pain, cramping, and feelings of fullness are common
  • Radiation therapy and chemotherapy often used to destroy cancer cells

Genetic Conditions Associated with Pancreatic Cancer

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  • Familial Atypical Multiple Mole Melanoma (FAMMM)
    • Due to mutations in p16 tumor suppressor gene
    • TP16 (CKDN2) located at 9p21
    • Affected individuals develop multiple nevi, atypical nevi, melanomas, and pancreatic cancer
  • Peutz-Jeghers Syndrome
    • Due to mutations in LKB1 (STK11) gene at 19p13.3
    • Causes multiple hamartomaous polyps of GI tract and pigmented macules on lips, buccal mucosa, and digits
    • Increases risk to develop cancers
      • In women: breast, pancreas, uterus, and ovary
      • In men: colon, pancreas, lung, or testes
  • Hereditary Breast-Ovarian Cancer Syndrome
    • Due to mutations in BRCA2 tumor suppressor gene at 13q12
    • Most common inherited predisposition to pancreatic cancer identified to date
    • Mutations in BRCA2 have been identified in individuals with no family history of breast or ovarian cancer
    • Estimated that carriers of 6174delT have 10-fold increased risk of developing pancreatic cancer
    • Increases risk for breast, ovarian, prostate, and colon cancers also
  • HNPCC
    • Due to mutations in mismatch repair genes, including MSH2 and MLH1
    • Microsatellite instability has been identified in ~4% of pancreatic cancers
    • Should be considered in patients with family history of colon cancer
    • Increases risk for colon/rectum, endometrium, ovarian, urinary tract, stomach, and small intestine also
  • MEN1
    • Due to mutations in menin tumor suppressor gene at 11q13
    • Associated specifically with insulinomas (10%) or gastrinomas (40%)
    • Can cause tumors on parathyroid and pituitary glands also
  • Hereditary Pancreatitis
    • Due to mutations in cationic trypsinogen gene on 7q35
    • Block inactivation of trypsin, resulting in autodigestion of pancrease
    • Increase in pancreatic cancer may be secondary to chronic injury and repair
    • Clinical testing available through University of Pittsburgh
    • Penetrance of mutations is only 80%

Psychosocial Issues

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  • Worry about the future - caring for themselves, family, etc.
  • Financial concerns
  • Concerns about treatments and side effects
  • Concern about necessity of frequent hospital visits, home care, etc.
  • Feelings of guilt, anxiety, fear surrounding potential diagnosis of hereditary syndrome

Resources

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  • The National Familial Pancreas Tumor Registry
Dr. Ralph H. Hruban, Director
Baltimore, MD 21231-2410
Phone: (410) 955-9132
  • Hereditary Pancreatitis Registry
Dr. Whitcomb or Dr. Lowenfels
The Midwest Multicenter Pancreatic Study Group
Phone: 888-PITT-DNA
  • National Cancer Institute
Phone: 1-800-4-CANCER
http://cancer.gov

References

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  • Hruban RH, et al. "Familial Pancreatic Cancer." Annals of Oncology (1999) 10:S69-S73.
  • "Pancreatic Cancer." Cancer Research UK. www.cancerresearchuk.org/aboutcancer
  • Schenk M, et al. "Familial Risk of Pancreatic Cancer." Journal of the National Cancer Institute (2001) 93:640-644.
  • "What you Need to Know About Cancer of the Pancreas. National Cancer Institute. www.nci.nih.gov/cancer

Notes

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The information in this outline was last updated in June 2002.

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