These cysts are benign and commonly occur in middle-aged women. They usually are located in the body or tail of the pancreas. Typically, they are small and cause no symptoms although rarely they may cause abdominal pain.

Some of these cysts contain cancer, and those that do not contain cancer are considered precancerous. They also commonly occur in middle-aged women and are usually located in the body or tail of the pancreas

These cysts have a high likelihood of being or becoming cancerous. At the time of diagnosis, there is a 45% to 65% chance of the cyst already being cancerous. These cysts are more common in middle-aged men and are more commonly located in the head of the pancreas. These cysts can cause abdominal pain, jaundice, and pancreatitis. Increased risk for cancer occurs with older age of the patient, presence of symptoms, involvement of the main pancreatic duct, dilation of the main pancreatic duct over 10 mm, the presence of nodules in the wall of the duodenum, and size over 3 cm for side-branch IPMN.

Pancreatic cancer is the fourth-leading cause of cancer-related mortality in the United States. Most patients have incurable disease at the time of diagnosis, and the overall 5-year survival is approximately 5%. The median age at diagnosis is 65 years. An increased risk of pancreatic cancer has been associated with smoking, alcoholism, family history, hereditary disorders [hereditary nonpolyposis colon cancer (HNPCC), von Hippel–Lindau disease (VHL), Peutz–Jeghers syndrome, familial breast cancer (BRCA2), familial atypical multiple mole melanoma (FAMMM)], and chronic pancreatitis. Inherited pancreatic cancers represent approximately 5% to 10% of all diagnoses of the disease.

90% of pancreatic carcinoma is what we call ductal adenocarcinoma. Seventy percent of pancreatic cancers occur at the head, 20% in the body, and 10% in the tail. The symptoms associated with pancreas cancer are almost always gradual in onset and are nonspecific, being the most common one mid epigastric pain, malaise, nausea, fatigue, and weight loss. Classically, the report of new-onset “painless jaundice” is believed to be pancreas cancer until proven otherwise. Pruritus (itchiness) may accompany obstructive jaundice. If obstructive jaundice is present, patients will also note darkening of the urine and “light-coloured” stools. New-onset diabetes within the year prior to diagnosis is found in 15% of patients with pancreatic cancer.

CA19-9 is a useful marker to follow in patients with elevated levels prior to initiation of therapy; however, it is often low in patients with resectable disease and can be elevated in non-malignant biliary obstructive disease. CA19-9 levels pre-treatment may also have some role in prognosis and follow up.

CT and MRI are the best image exams to diagnose pancreas cancer.

CT imaging should be a fine-cut, “pancreatic protocol CT” including 3-phases (arterial, venous, and portal venous – in which the scan is done during different times when the contrast goes through the organs) and thin slices to allow for assessment of the relationship of the mass to vascular structures as this is crucial to determine the probability of surgical resection. The use of CT to determine resectable versus borderline resectable disease is imperative as some advocate borderline resectable disease is a marker of worse biology and that these patients should undergo neoadjuvant therapy. SO essentially patients are classified in three categories that will dictate the management as follow:

Local resectable disease: no distant metastases or major invasion of vessels – patient to proceed to surgical resection

Borderline resectable: no distant metastases; vascular involvement to some extension that the tumour could be resected if chemotherapy can reduce the tumour

Unresectable: Distant metastases are present or major vascular occlusion is seen.

Positron emission tomography (PET) is limited to investigating for occult metastatic disease and has limited utility in primary diagnosis. A relatively high false-positive rate has been reported in pancreatitis or other inflammatory conditions when evaluating the pancreas for the diagnosis of cancer.

Treatment

Pancreaticoduodenectomy or Whipple procedure consists of en bloc resection of the head of the pancreas, distal common bile duct, duodenum, jejunum, and gastric antrum. A modification of this operation, preserving the gastric antrum, is called pylorus-preserving pancreaticoduodenectomy (Willian-Traverso operation) has been advocated by some, but there are no data demonstrating improved survival or lower morbidity. There has been a sharp decline in morbidity and mortality in specialized centres, with a 30-day mortality of less than 3%.

Distal pancreatectomy. The procedure of choice for lesions of the body and tail of the pancreas is distal pancreatectomy. Distal pancreatectomy consists of resection of the pancreas and the spleen. It can be done as an open procedure or as a keyhole procedure (laparoscopic).

Postoperative considerations. Delayed gastric emptying, pancreatic fistula, and wound infection are the three most common complications of the Whipple’s operation. Up to 10% of patients require a nasogastric tube for longer than 10 days, but delayed gastric emptying almost always subsides with conservative treatment. The rate of pancreatic fistula has been demonstrated to be around 10-30% depending on expertise and the pancreas conditions (duct size, pancreas texture, previous pancreatic abnormality). Distal pancreatectomy has a higher morbidity and leak rates than Whipple with an approximately 20 to 40% pancreatic leak rate in most series.

There is a clear benefit to have chemotherapy after the pancreatic surgery; however, the choice between chemoradiation (chemotherapy and radiation) and chemotherapy alone is less clear. In the USA, the standard practise of most centres is chemoradiation. In Europe, is chemotherapy alone. Here in Australia, most oncologists would suggest chemotherapy only.

Some groups have shown an improvement in survival with neoadjuvant chemoradiation (chemotherapy and radiation before the pancreatic resection) therapy however it is only used in certain specific condition at the moment. The radiation given can turn the surgery more difficult and also can delay the access to operation in case there is a major complication related to the chemotherapy.

Prognosis. Surgical resection increases survival over patients with similar stage disease that do not undergo resection. Overall 5-year survival rates are approximately 20% for patients after resection. In patients with small tumours, negative resection margins, and no evidence of nodal metastases, the 5-year survival rate is as high as 40%. Median survival for unresectable locally advanced disease is 9 to 12 months, and for hepatic metastatic disease it is 3 to 6 months.

Pancreatic neuroendocrine tumours (PNETs) develop from cells in the endocrine gland of the pancreas, which secretes the hormones insulin and glucagon into the bloodstream to regulate blood sugar. Also known as endocrine or islet cell tumours, neuroendocrine cancers are rare, making up less than 5 percent of all pancreatic cancer cases. PNETs are classified according to its grade of differentiation, function (they secrete hormones or not).

Tumour grade describes how quickly the cancer is likely to grow and spread. Grade 1 (also called low-grade or well-differentiated) neuroendocrine tumours have cells that look more like normal cells and are not multiplying quickly. Grade 2 (also called intermediate-grade or moderately differentiated) tumours have features in between those of low- and high-grade (see below) tumours. Grade 3 (also called high-grade or poorly differentiated) neuroendocrine tumours have cells that look very abnormal and are multiplying faster.

Cancers that are grades 1 or 2 are called pancreatic neuroendocrine tumours. These cancers tend to grow slowly and can possibly spread to other parts of the body.

Cancers that are grade 3 are called pancreatic neuroendocrine carcinomas (NECs). These cancers tend to grow and spread quickly and can spread to other parts of the body. Another important part of grading is measuring how many of the cells are in the process of dividing into new cells.

PNETs can also so named based on whether they are functioning (making hormones that cause symptoms) or non-functioning (not making hormones).

Functioning NETs

About half of pancreatic NETs make hormones that are released into the blood and cause symptoms. These are called functioning NETs. Each one is named for the type of hormone the tumour cells make.

Here is a list of them:

Insulinomas come from cells that make insulin. Glucagonomas come from cells that make glucagon. Gastrinomas come from cells that make gastrin. Somatostatinomas come from cells that make somatostatin. VIPomas come from cells that make vasoactive intestinal peptide (VIP). ACTH-secreting tumours come from cells that make adrenocorticotropic hormone (ACTH).

Most (up to 70%) functioning NETs are insulinomas. The other types are much less common.

Non-functioning NETs:

These tumours don’t make enough excess hormones to cause symptoms. Because they don’t make excess hormones that cause symptoms, they can often grow quite large before they're found. Symptoms that may occur when they grow to a large size include abdominal pain, lack of appetite, and weight loss.

Carcinoid tumours: These NETs are much more common in other parts of the digestive system, although rarely they can start in the pancreas. These tumours often make serotonin.

The treatment for pancreatic NETs depends on the specific tumour type and the stage (extent) of the tumour, but the general outcome is generally better than for pancreatic exocrine cancers.

Solid pseudopapillary neoplasia (SPN) of the pancreas is an extremely rare epithelial tumor of low malignant potential that usually affects young woman. It accounts for less than 1% to 2% of exocrine pancreatic tumors. SPN can occur in every part of the pancreas but they are slightly more common in the tail. Depending on the tumor position (head, body or tail of the pancreas), the differential diagnosis includes adrenal mass, pancreatic endocrine tumor, liver cyst or tumor, or a pseudocyst.

In approximately 85% of the patients, SPN is limited to the pancreas, while about 10% to 15% of tumors have already metastasized at the time of presentation.

Malignant SPN, designated as a solid-pseudopapillary carcinoma, occurs in 15% of adult patients.

Surgery is the only curative treatment for SPN and will depend on location (head, body or tail) of the lesion.

IPMN and Mucinous serous cystadenoma are the most common pancreatic cystic lesions that can become a cancer. Certain characteristics seen on CT or MRI scan can define which one is more likely to become a cancer. If not possible, endoscopic ultrasound with FNA sampling can be done.