Prof.

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PERIAMPULLARY TUMORS

• Prof. ÖZCAN GÖKÇE, MD
• Director of the Department of General Surgery
• Yeditepe University Hospital

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Neoplasms of the Endocrine Pancreas

• INSULINOMA
• GASTRINOMA
• VIPoma
• GLUCAGONOMA
• SOMATOSTATINOMA
• NONFUNCTIONING ISLET CELL TUMORS

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Passaro's triangle (The typical location of a
gastrinoma)
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Radiolabeled octreotide scan
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Insulinoma

• Whipples triad
• 1)Hypoglisemic symptoms by fasting
• 2)Episodic low blood sugar(below 50 mg/dl)
• 3)Relief of symptoms by glucose

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Treatment

• Surgery
• Diazoxide
• Streptozotocin

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GASTRINOMA

• (Zollinger-Ellison Syndrome)
• Symptoms
• Ulcers and diarrhea
• Treatment
• Surgery
• Acid blocking agents
• Streptozotocin-5FU

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VIPoma

• Verner-Morrison SyndromeWDHA
• Watery diarrhea
• Hypokalemia
• Achlorhydria
• Pancreatic Cholera

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Treatment

• Surgery
• Streptozotocin
• 5FU
• Steroids
• Trifluoperazine
• Somatostatin

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MULTIPL ENDOCRINE NEOPLASIA

• MEN I.3-P(Pituiatary,parathyroid,pancreas)
• Autosomal dominant(11q13 coded gene)
• MEN IIA...(Pheo-Medullary tc-parathyroid tm)
• MEN IIB..(Pheo-Medullary tc-ganglioneuromas)
• Mutation in ret proto-oncogene

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Neoplasms of the Exocrine Pancreas
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PERIAMPULLARY EPITHELIAL TUMORS

• 1)PANCREATIC CANCER
• 2)CANCER OF COMMON BILE DUCT
• 3)CANCER OF DUODENUM
• 4)TUMOR OF AMPULLA OF WATER
• 5)BENIGN TUMORS OF THE REGION(EXTREMELY
  RARE)cystadenomas

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• Pancreatic cancer is one of the most serious
  types of cancer occurring. It is a disease which
  forms malignant or cancerous cells in the tissues
  of the pancreas.

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• The digestive juices are produced by exocrine
  pancreas cells and the hormones are produced by
  endocrine pancreas cells. About 95 of pancreatic
  cancers begin in exocrine cells.

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• Pancreatic Cancer has been called a "silent"
  disease because early Pancreatic Cancer usually
  does not cause symptoms.

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• If the tumor blocks the common bile duct and bile
  cannot pass into the digestive system, the skin
  and the sclera may become yellow (jaundiced), and
  the urine darker as a result of accumulated bile
  pigment called bilirubin.

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• Pancreatic Cancer Causes
• The exact as to what damages DNA in the vast
  majority of cases of pancreatic cancer is not
  clear. In other words the exact pancreatic cancer
  causes are not clear. But it is known that a
  small percentage of people develop the disease as
  a result of a genetic predisposition. These
  people who have a close relative, such as a
  parent or sibling, with pancreatic cancer have a
  higher risk of developing pancreatic cancer
  themselves.

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• A number of genetic diseases have been associated
  with an increased risk of pancreatic cancer,
  including familial adenomatous polyposis,
  nonpolyposis colon cancer, familial breast cancer
  associated with the BRCA2 gene, hereditary
  pancreatitis, and familial atypical multiple
  mole-melanoma syndrome which is a serious type of
  skin cancer. This means that people who have a
  hereditary predisposition to develop these
  cancers are also more likely to develop
  pancreatic cancer.

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• Although the above causes are evident only 10
  percent of pancreatic cancers result from an
  inherited tendency. A greater number are caused
  by environmental or lifestyle factors, such as
  smoking, diet and chemical exposure.

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• Age is also a factor to be considered which
  increases the incidence of the disease. As age
  increases the probability of pancreatic cancer
  also increases. The incidence of Pancreatic
  Cancer is relatively low in individuals up to age
  50, after which it increases significantly. The
  age group 65 - 79 has the highest incidence of
  Pancreatic Cancer.

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• Smoking increases the chances of developing
  Pancreatic Cancer. Study reveals that smokers
  develop Pancreatic Cancer more than twice as
  often as nonsmokers.

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• Non vegetarians have a high risk of getting
  Pancreatic Cancer, if the intakes of meat fat
  are high.

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• Medical factors such as cirrhosis (a chronic
  liver disease), chronic pancreatitis, diabetes
  and a history of surgery to the upper digestive
  tract also contribute to the risk

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• Environmental factors such as long-term exposure
  to certain chemicals, like gasoline and related
  compounds, as well as certain insecticides, may
  increase the risk of developing Pancreatic
  Cancer.

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• Genetic predisposition should also be considered
  as the possible reason to increase the risk.
  Possibly 10 of cases of Pancreatic Cancer are
  related to genetic disorders.

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• Pancreatic Cancer Stage
• To apt for any treatment option the doctor has to
  know the exact stage of the pancreatic cancer in
  the patient. Tests and procedures to stage
  pancreatic cancer are usually done at the same
  time as diagnosis.
• The following are pancreatic cancer stage
  descriptions

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• Stage 0 In this stage the cancer is found only
  in the lining of the pancreas. Stage 0 is also
  called carcinoma in situ.

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• Stage I Cancer is found only in the pancreas in
  this stage. This stage further divided into stage
  IA and stage IB, based on the size of the tumor.
• Stage IA The tumor is 2 centimeters or smaller.
• Stage IB The tumor is larger than 2 centimeters.

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• Stage II Cancer may have spread to nearby tissue
  and organs, and may have spread to lymph nodes
  near the pancreas in this stage. This stage is
  further divided into stage IIA and stage IIB,
  based on where the cancer has spread.
• Stage IIA Cancer has spread to nearby tissue and
  organs but has not spread to nearby lymph nodes.
• Stage IIB Cancer has spread to nearby lymph
  nodes and may have spread to nearby tissue and
  organs.

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• Stage III Cancer has spread to the major blood
  vessels near the pancreas and may have spread to
  nearby lymph nodes in this stage. (Beyond this
  point the patient is surgically incurable)

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• Stage IV Cancer may be of any size and has
  spread to distant organs, such as the liver,
  lung, and peritoneal cavity. It may have also
  spread to organs and tissues near the pancreas or
  to lymph nodes.

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Pancreatic Cancer Symptoms

• Discomfort in abdomen or Upper abdominal pain
  that may radiate to the middle or upper back
• Yellowing of the skin and the sclera (jaundice)
• Itching
• Nausea and vomiting
• Digestive problems
• Weight loss
• Fatigue
• Loss of appetite
• Glucose intolerance
• Malnutrition.

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• In addition, the pancreas may produce too much
  insulin, causing such symptoms as dizziness,
  weakness, diarrhea, chills, or muscle spasms. 

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• The patient may not even notice the gradual onset
  of these relatively nonspecific symptoms. The
  doctor may interpret them as being caused by
  something else. Therefore one should be very
  careful if any of the above symptoms are
  experienced.

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Pancreatic Cancer Treatment  

• The choice of Pancreatic Cancer treatment depends
  largely on the stage the pancreatic cancer in the
  patient.
• Possible treatments include surgery,
  chemotherapy, radiation, and biological therapy.
  Biological therapy is also known as
  immunotherapy.

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Surgery

• During diagnosis, only about 20 of pancreatic
  tumors can be removed by surgery. This option is
  usually reserved for Stage 1 patients whose
  cancer is resectable. The surgeon may perform a
  resection, which is the removal of a small part
  of the pancreas.
• The standard procedure is called a
  pancreaticoduodenectomy (Whipple procedure).This
  surgery for Pancreatic Cancer should be done at
  centers that perform the procedure frequently. It
  is the more extensive surgery which is the
  removal of the pancreas head and nearby
  structures such as the duodenum. It might be
  necessary to remove the entire pancreas as well
  as the spleen, gallbladder, and bile duct.

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• When the tumor is confined to the pancreas but
  cannot be removed, a combination of radiation
  therapy and chemotherapy may be recommended.

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Radiation therapy

• Certain substances emit invisible, high-energy
  rays, or radiation. These substances are called
  radioactive. The energy of the rays can kill
  cells. Special machines aim the rays at the parts
  of the body with cancerous tumors, so as to kill
  the cancer cells while minimizing damage to
  healthy tissue.
• Unlike chemotherapy, radiation is not necessarily
  carried throughout the body. As such, it is known
  as a local therapy, rather than as a systemic
  therapy. However, there are still potential side
  effects of radiation including fatigue, localized
  hair loss, changes to appearance of skin, and
  digestive problems. Medicines and other
  treatments can reduce the intensity of the side
  effects. As with other cancer treatments, the
  incidence of side effects varies with patient
  health and the exact nature of the treatment.

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Chemotherapy

• When the tumor has spread to other organs
  (metastasized) such as the liver, chemotherapy
  alone is usually used.
• The standard chemotherapy agent is gemcitabine,
  but other drugs may be used. Gemcitabine provides
  clinical improvement in approximately 25 of
  patients.For patients who have biliary
  obstruction (blockage of the vessels that
  transport bile) and the tumor cannot be totally
  removed, the obstruction must be relieved. There
  are generally two approaches to this, surgery and
  placement of a biliary stent (similar to stents
  placed in the arteries of the heart to relieve
  blockages) during ERCP.

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Immunotherapy

• This form of therapy involves using drugs to
  boost the body's natural immune response (its
  ability to fight disease). Examples of these
  drugs are interferon and monoclonal antibodies.
  They work with the body's immune system to block
  the growth of cancer cells. Biological therapy
  can be used on its own or in conjunction with
  other therapies. Immunotherapy can result in
  side effects that are reminiscent of the flu
  fever, aches, weakness, fatigue, and chills.
  Patients may also experience skin problems such
  as easy bruising or rashes, as well as diarrhea
  and nausea.

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Pancreatic Cancer Survival Rate

• Pancreatic Cancer is rarely curable. The overall
  pancreatic cancer survival rate is less than 4.
  The cure rates are highest (although still
  usually under 25) if the tumor is small (less
  than 2 cm in diameter) and is truly localized to
  the pancreas but, unfortunately, such cases
  account for fewer than 20 of all cases of
  Pancreatic Cancer. For patients with advanced
  cancers, the overall survival rate of all stages
  is less than 1 at 5 years with most patients
  dying within 1 year.

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Prognosis of periampullary tumors

• 5-year survival rate
• Pancreas...............10
• Ampulla of Vater... 35
• Duodenum. 30
• Bile duct. 15
• After Whipple operation(without lymph node
  metastases).. 35

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CYSTIC NEOPLASMS OF THE PANCREAS
MUCINOUS CYSTADENOMA AND CYSTADENOCARCINOMA
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CYSTADENOMA