2015年11月26日 星期四

胰臟癌病患該問醫生哪些問題? - 約翰霍普金斯醫學院的建議

Questions

What questions should I ask my doctor?

If you are in the midst of dealing with pancreatic cancer, you have a lot on your mind and you may have difficulty knowing where to start. Since every patient has a unique case, your doctors are your best source of information and you have every right to ask them questions.
Heather Sentkoski, a clinical social worker at Johns Hopkins, has compiled the following list of questions as a quideline. Here are her suggestions:

If you are meeting with a surgeon or oncologist for the first time, do not be afraid to ask:

  • Have you ever treated a PC patient before?
  • If this is a surgeon, how many surgeries have you performed on PC patients?
  • What has the general outcome of those patients been?
  • Where were you trained? (medical school, residency)
  • Which surgeons did you study under?

At any point in the relationship with your physican, you have the right to ask:

  • What is the diagnosis?
  • What treatments are recommended?
  • Are there other treatment options available that you do not provide? i.e. protocol treatments, herbal therapy, touch therapy, other alternative therapies)
  • What are the benefits of each treatment?
  • What are the side effects of each treatment?
  • What are the medications being prescribed? What are they for? What are their side effects?
  • Are there any clinical drug trials I can participate in?
  • How should I expect to feel during the treatment(s)?
  • What are the risks of the treatment(s)?
  • Will my diet need to be changed or modified?
  • Will I need to take enzymes, vitamins, etc?

Do not forget to ask about the things that are most important to you:

  • How will this affect my ability to work?
  • Can this treatment be done as an outpatient so that I can spend more time at home with family?
  • Will I have any physical limitations?
  • How will my current lifestyle be changed?

Finally - and most importantly - ask these questions of YOURSELF:

  • Does my doctor appear interested in answering my questions?
  • Or, does my doctor look annoyed when I ask questions, like I'm doubting their expertise or I am holding them up?
  • Do I feel that my doctor cares about my medical outcome?
If you are uncomfortable with the results of some of these questions, you may want to re-evaluate your choice of physician or get a second opinion.
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胰臟癌會遺傳嗎?

Is pancreatic cancer hereditary?

Cancer of the pancreas is a genetic disease which means that it is caused by changes (mutations) in DNA. These changes can be inherited (we are born with them) or they can be acquired (they develop after we are born). The inherited changes explain why cancer of the pancreas runs in some families, and the acquired changes can be the result of either bad luck during cell replication or by exposure to carcinogens (cancer causing chemicals) such as those found in cigarette smoke. The genetics of hereditary pancreatic cancer is a focus of research at Johns Hopkins.
It has been estimated that ten percent of pancreatic cancers are hereditary. Many of these occur as part of rare medical syndromes. These include:
  1. Familial breast cancer gene(BRCA2)
    BRCA2 was the second familial breast cancer gene identified. It was discovered in 1995 because of a remarkable advance made by the Hopkins team studying pancreatic cancer. The team at Hopkins had discovered a "homozygous deletion" (a missing piece of DNA) in a pancreatic cancer and postulated that this missing piece of DNA is where the BRCA2 gene could be found (Schutte et al, Proceedings of the National Academy of Sciences, 1995). Subsequently, Dr. Goggins at Hopkins demonstrated that as many as 10% of pancreas cancers are caused by inherited defects in the BRCA2 gene. One particular defect in BRCA2 (a mutation called 6174 del T) is found in about 1% of individuals of Ashkenazi Jewish descent. This mutation may explain the higher rate of pancreatic cancer observed in Jews as compared to Catholics and Protestants. Testing is now available for BRCA2 gene mutations. We advise that people speak with a trained cancer genetics counselor before undergoing genetic testing.
  2. More recently, the team at Johns Hopkins discovered that inherited mutations in PALB2 (partner and localizer of BRCA2), a gene that functions together with BRCA2, cause about 3% of familial pancreatic cancer, and other groups have found that inherited mutations in the first breast cancer gene, BRCA1, may also increase the risk of pancreatic cancer. Testing is also available for BRCA1 and PALB2 gene mutations. We advise that people speak with a trained cancer genetics counselor before undergoing genetic testing.
  3. Peutz-Jeghers syndrome
    This is a very rare hereditary syndrome in which affected family members develop polyps in their small intestines and pigmented spots on their lips and inside of their mouth. These polyps are masses of tissue that protrude from the normal surface of the intestine. Peutz-Jeghers is caused by inherited mutations in the STK11gene. Patients with Peutz-Jeghers syndrome have an increased risk of developing pancreas cancer.
    Peutz-Jeghers syndrome
  4. Familial melanoma
    The Familial Atypical Multiple Mole Melanoma (FAMMM) syndrome is a rare hereditary syndrome in which affected family members develop skin moles and melanomas (an aggressive form of skin cancer). These patients also have an increased risk of developing pancreas cancer. FAMMM is caused by inherited mutations in the p16/CDKN2A gene.
  5. Hereditary colon cancer
    The Hereditary Non-polyposis Colorectal Cancer (HNPCC) syndrome, also known as Lynch syndrome, strikes as many as 1 in 200 individuals and it is characterized by the inherited predisposition to develop colon cancer, endometrial (uterine) cancer, stomach cancer and ovarian cancer. Patients with HNPCC may also have an increased risk of developing pancreas cancer. Indeed, the DNA finding typical of HNPCC, called microsatellite instability has recently been reported in a small (about 4%) fraction of pancreas cancers. Gene testing for HNPCC is now available.
  6. Hereditary pancreatitis
    This rare disease is characterized by the development of recurrent episodes of severe chronic pancreatitis (inflammation of the pancreas) starting at an early age (often in patients in their teens). The gene responsible for hereditary pancreatitis, called the trypsinogen gene, was discovered by Dr. Whitcomb of the University of Pittsburgh. Clinical gene testing for hereditary pancreatitis is now available.
  7. Ataxia telangiectasia
    The team at Johns Hopkins discovered that inherited mutations in the ATM gene also increase the risk of pancreatic cancer. Inherited mutations in ATM are known to cause the clinical syndrome of "ataxia telangiectasia," and 2-3% of people with familial pancreatic cancer inherited a defective (mutant) copy of theATM gene.
  8. Familial pancreatic cancer
    While the above genetic syndromes account for ~20% of familial pancreatic cancer, it is clear that there are other, yet undiscovered familial pancreatic cancer genes. For example, relatives of patients with pancreas cancer have an increased risk for developing pancreas cancer themselves, and the National Familial Pancreas Cancer Registry now contains over 1500 families in which two or more family members have had pancreas cancer.
    Scientists at Johns Hopkins are working diligently to discover the reasons why pancreas cancer runs in these families. Anyone wishing to join this registry or wishing to learn more about familial pancreas cancer may contact Alison Klein at aklein1@jhmi.edu.

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胰臟內分泌瘤 (胰島細胞瘤) 療法

Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ®)

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General Information About Pancreatic Neuroendocrine Tumors (Islet Cell Tumors)

KEY POINTS

  • •Pancreatic neuroendocrine tumors form in hormone-making cells (islet cells) of the pancreas.
  • •Pancreatic NETs may or may not cause signs or symptoms.
  • •There are different kinds of functional pancreatic NETs.
  • •Having certain syndromes can increase the risk of pancreatic NETs.
  • •Different types of pancreatic NETs have different signs and symptoms.
  • •Lab tests and imaging tests are used to detect (find) and diagnose pancreatic NETs.
  • •Other kinds of lab tests are used to check for the specific type of pancreatic NETs.
  • •Certain factors affect prognosis (chance of recovery) and treatment options.

Pancreatic neuroendocrine tumors form in hormone-making cells (islet cells) of the pancreas.

The pancreas is a gland about 6 inches long that is shaped like a thin pear lying on its side. The wider end of the pancreas is called the head, the middle section is called the body, and the narrow end is called the tail. The pancreas lies behind the stomach and in front of the spine.
ENLARGEAnatomy of the pancreas; drawing shows the pancreas, stomach, spleen, liver, gallbladder, bile ducts, colon, and small intestine. An inset shows the head, body, and tail of the pancreas. The bile duct and pancreatic duct are also shown.
Anatomy of the pancreas. The pancreas has three areas: head, body, and tail. It is found in the abdomen near the stomach, intestines, and other organs.
There are two kinds of cells in the pancreas:
This summary discusses islet cell tumors of the endocrine pancreas. See the PDQ summary on Pancreatic Cancer Treatment for information on exocrine pancreatic cancer.
Pancreatic neuroendocrine tumors (NETs) may be benign (not cancer) or malignant (cancer). When pancreatic NETs are malignant, they are called pancreatic endocrine cancer or islet cell carcinoma.
Pancreatic NETs are much less common than pancreatic exocrine tumors and have a better prognosis.

Pancreatic NETs may or may not cause signs or symptoms.

Pancreatic NETs may be functional or nonfunctional:
  • •Functional tumors make extra amounts of hormones, such as gastrin, insulin, and glucagon, that cause signs and symptoms.
  • •Nonfunctional tumors do not make extra amounts of hormones. Signs and symptoms are caused by the tumor as it spreads and grows. Most nonfunctional tumors are malignant (cancer).
Most pancreatic NETs are functional tumors.

There are different kinds of functional pancreatic NETs.

Pancreatic NETs make different kinds of hormones such as gastrin, insulin, and glucagon. Functional pancreatic NETs include the following:
  • •Gastrinoma: A tumor that forms in cells that make gastrin. Gastrin is a hormone that causes the stomach to release an acid that helps digest food. Both gastrin and stomach acid are increased by gastrinomas. When increased stomach acid, stomach ulcers, and diarrhea are caused by a tumor that makes gastrin, it is called Zollinger-Ellison syndrome. A gastrinoma usually forms in the head of the pancreas and sometimes forms in the small intestine. Most gastrinomas are malignant (cancer).
  • •Insulinoma: A tumor that forms in cells that make insulin. Insulin is a hormone that controls the amount of glucose (sugar) in the blood. It moves glucose into the cells, where it can be used by the body for energy. Insulinomas are usually slow-growing tumors that rarely spread. An insulinoma forms in the head, body, or tail of the pancreas. Insulinomas are usually benign (not cancer).
  • •Glucagonoma: A tumor that forms in cells that make glucagon. Glucagon is a hormone that increases the amount of glucose in the blood. It causes the liver to break down glycogen. Too much glucagon causes hyperglycemia (high blood sugar). A glucagonoma usually forms in the tail of the pancreas. Most glucagonomas are malignant (cancer).
  • •Other types of tumors: There are other rare types of functional pancreatic NETs that make hormones, including hormones that control the balance of sugar, salt, and water in the body. These tumors include:
    • •VIPomas, which make vasoactive intestinal peptide. VIPoma may also be called Verner-Morrison syndrome.
    • •Somatostatinomas, which make somatostatin.
    These other types of tumors are grouped together because they are treated in much the same way.

Having certain syndromes can increase the risk of pancreatic NETs.

Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk.
Multiple endocrine neoplasia type 1 (MEN1) syndrome is a risk factor for pancreatic NETs.

Different types of pancreatic NETs have different signs and symptoms.

Signs or symptoms can be caused by the growth of the tumor and/or by hormones the tumor makes or by other conditions. Some tumors may not cause signs or symptoms. Check with your doctor if you have any of these problems.
Signs and symptoms of a non-functional pancreatic NET
A non-functional pancreatic NET may grow for a long time without causing signs or symptoms. It may grow large or spread to other parts of the body before it causes signs or symptoms, such as:
  • •Diarrhea.
  • •Indigestion.
  • •A lump in the abdomen.
  • •Pain in the abdomen or back.
  • •Yellowing of the skin and whites of the eyes.
Signs and symptoms of a functional pancreatic NET
The signs and symptoms of a functional pancreatic NET depend on the type of hormone being made.
Too much gastrin may cause:
  • •Stomach ulcers that keep coming back.
  • •Pain in the abdomen, which may spread to the back. The pain may come and go and it may go away after taking an antacid.
  • •The flow of stomach contents back into the esophagus (gastroesophageal reflux).
  • •Diarrhea.
Too much insulin may cause:
  • •Low blood sugar. This can cause blurred vision, headache, and feeling lightheaded, tired, weak, shaky, nervous, irritable, sweaty, confused, or hungry.
  • •Fast heartbeat.
Too much glucagon may cause:
  • •Skin rash on the face, stomach, or legs.
  • •High blood sugar. This can cause headaches, frequent urination, dry skin and mouth, or feeling hungry, thirsty, tired, or weak.
  • •Blood clots. Blood clots in the lung can cause shortness of breath, cough, or pain in the chest. Blood clots in the arm or leg can cause pain, swelling, warmth, or redness of the arm or leg.
  • •Diarrhea.
  • •Weight loss for no known reason.
  • •Sore tongue or sores at the corners of the mouth.
Too much vasoactive intestinal peptide (VIP) may cause:
  • •Very large amounts of watery diarrhea.
  • •Dehydration. This can cause feeling thirsty, making less urine, dry skin and mouth, headaches, dizziness, or feeling tired.
  • •Low potassium level in the blood. This can cause muscle weakness, aching, or cramps, numbness and tingling, frequent urination, fast heartbeat, and feeling confused or thirsty.
  • •Cramps or pain in the abdomen.
  • •Weight loss for no known reason.
Too much somatostatin may cause:
  • •High blood sugar. This can cause headaches, frequent urination, dry skin and mouth, or feeling hungry, thirsty, tired, or weak.
  • •Diarrhea.
  • •Steatorrhea (very foul-smelling stool that floats).
  • •Gallstones.
  • •Yellowing of the skin and whites of the eyes.
  • •Weight loss for no known reason.

Lab tests and imaging tests are used to detect (find) and diagnose pancreatic NETs.

The following tests and procedures may be used:
  • •Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
  • •Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances, such as glucose (sugar), released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.
  • •Chromogranin A test: A test in which a blood sample is checked to measure the amount of chromogranin A in the blood. A higher than normal amount of chromogranin A and normal amounts of hormones such as gastrin, insulin, and glucagon can be a sign of a non-functional pancreatic NET.
  • •Abdominal CT scan (CAT scan): A procedure that makes a series of detailed pictures of the abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
  • •MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
  • •Somatostatin receptor scintigraphy : A type of radionuclide scan that may be used to find small pancreatic NETs. A small amount of radioactive octreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is also called octreotide scan and SRS.
  • •Endoscopic ultrasound (EUS): A procedure in which an endoscope is inserted into the body, usually through the mouth or rectum. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. A probe at the end of the endoscope is used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. This procedure is also called endosonography.
  • •Endoscopic retrograde cholangiopancreatography (ERCP): A procedure used to x-ray the ducts (tubes) that carry bile from the liver to the gallbladder and from the gallbladder to the small intestine. Sometimes pancreatic cancer causes these ducts to narrow and block or slow the flow of bile, causing jaundice. An endoscope is passed through the mouth, esophagus, and stomach into the first part of the small intestine. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. A catheter (a smaller tube) is then inserted through the endoscope into the pancreatic ducts. A dye is injected through the catheter into the ducts and an x-ray is taken. If the ducts are blocked by a tumor, a fine tube may be inserted into the duct to unblock it. This tube (or stent) may be left in place to keep the duct open. Tissue samples may also be taken and checked under a microscope for signs of cancer.
  • •Angiogram : A procedure to look at blood vessels and the flow of blood. A contrast dye is injected into the blood vessel. As the contrast dye moves through the blood vessel, x-rays are taken to see if there are any blockages.
  • •Laparotomy : A surgical procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs are removed or tissue samples are taken and checked under a microscope for signs of disease.
  • •Intraoperative ultrasound : A procedure that uses high-energy sound waves (ultrasound) to create images of internal organs or tissues during surgery. A transducer placed directly on the organ or tissue is used to make the sound waves, which create echoes. The transducer receives the echoes and sends them to a computer, which uses the echoes to make pictures called sonograms.
  • •Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. There are several ways to do a biopsy for pancreatic NETs. Cells may be removed using a fine or wide needle inserted into the pancreas during an x-ray or ultrasound. Tissue may also be removed during a laparoscopy (a surgical incision made in the wall of the abdomen).
  • •Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the blood. The radioactive material collects in bones where cancer cells have spread and is detected by a scanner.

Other kinds of lab tests are used to check for the specific type of pancreatic NETs.

The following tests and procedures may be used:
Gastrinoma
  • •Fasting serum gastrin test: A test in which a blood sample is checked to measure the amount of gastrin in the blood. This test is done after the patient has had nothing to eat or drink for at least 8 hours. Conditions other than gastrinoma can cause an increase in the amount of gastrin in the blood.
  • •Basal acid output test: A test to measure the amount of acid made by the stomach. The test is done after the patient has had nothing to eat or drink for at least 8 hours. A tube is inserted through the nose or throat, into the stomach. The stomach contents are removed and four samples of gastric acid are removed through the tube. These samples are used to find out the amount of gastric acid made during the test and the pH level of the gastric secretions.
  • •Secretin stimulation test : If the basal acid output test result is not normal, a secretin stimulation test may be done. The tube is moved into the small intestine and samples are taken from the small intestine after a drug called secretin is injected. Secretin causes the small intestine to make acid. When there is a gastrinoma, the secretin causes an increase in how much gastric acid is made and the level of gastrin in the blood.
  • •Somatostatin receptor scintigraphy: A type of radionuclide scan that may be used to find small pancreatic NETs. A small amount of radioactive octreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is also called octreotide scan and SRS.
Insulinoma
  • •Fasting serum glucose and insulin test: A test in which a blood sample is checked to measure the amounts of glucose (sugar) and insulin in the blood. The test is done after the patient has had nothing to eat or drink for at least 24 hours.
Glucagonoma
  • •Fasting serum glucagon test: A test in which a blood sample is checked to measure the amount of glucagon in the blood. The test is done after the patient has had nothing to eat or drink for at least 8 hours.
Other tumor types
  • •VIPoma
    • •Serum VIP (vasoactive intestinal peptide) test: A test in which a blood sample is checked to measure the amount of VIP.
    • •Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. In VIPoma, there is a lower than normal amount of potassium.
    • •Stool analysis : A stool sample is checked for a higher than normal sodium (salt) and potassium levels.
  • •Somatostatinoma
    • •Fasting serum somatostatin test: A test in which a blood sample is checked to measure the amount of somatostatin in the blood. The test is done after the patient has had nothing to eat or drink for at least 8 hours.
    • •Somatostatin receptor scintigraphy: A type of radionuclide scan that may be used to find small pancreatic NETs. A small amount of radioactive octreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is also called octreotide scan and SRS.

Certain factors affect prognosis (chance of recovery) and treatment options.

Pancreatic NETs can often be cured. The prognosis (chance of recovery) and treatment options depend on the following:
  • •The type of cancer cell.
  • •Where the tumor is found in the pancreas.
  • •Whether the tumor has spread to more than one place in the pancreas or to other parts of the body.
  • •Whether the patient has MEN1 syndrome.
  • •The patient's age and general health.
  • •Whether the cancer has just been diagnosed or has recurred (come back).
Next section >
Stages of Pancreatic Neuroendocrine Tumors
  • Updated: July 30, 2015

  • 資料來源:美國國家癌症研究中心 (National Cancer Institute, NCI)
  • http://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq
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