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Types of Pancreatic Cancer |
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The cause of pancreatic cancer is largely not known, although in the case of ductal-type cancer there is an association with smoking tobacco. There is also an increased risk in patients with chronic pancreatitis and Hereditary Pancreatitis. Pancreatic cancer also occurs in certain familial cancer syndromes such as Peutz-Jeghers Syndrome, Breast and Ovarian cancer Syndromes, Familial Atypical Mole and Melanoma Syndrome and Familial Adenomatous Polyposis. Rarely there are families with Familial Pancreatic Cancer.
You can get cancer in the head, body or tail of the pancreas. Between 7 and 8 out of 10 pancreatic cancers (70 - 80%) are in the head of the pancreas. The symptoms vary depending on where in the pancreas the tumour is and the type of pancreatic cancer. Different types of surgery are used depending on the site of the cancer.
There are many types of cancer of the pancreas. The vast majority are exocrine type but there are also rarer endocrine type cancers which produce hormones and the very rare lymphoma of the pancreas. The majority of tumours are malignant but some types are benign. More than 9 out of 10 of exocrine pancreatic cancers are adenocarcinomas. Nearly all of these are ductal adenocarcinomas.
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The commonest type of pancreatic cancer is that arising from the small ducts of the pancreas (ductal-type adenocarcinoma or PDAC). (The ducts are the tubes that carry the pancreatic digestive juices to the main pancreatic duct and the duodenum). This tumour accounts for almost 90% of pancreatic cancer cases. About 70-80% of these occur in the head of the pancreas.
It affects both sexes almost equally, has its peak incidence in the sixth decade of life and is extremely rare before the age of 40.
Resection of the cancer followed by adjuvant chemotherapy is the standard treatment. If this is not possible it may be possible to obtain good palliative results with chemotherapy. Patients are strongly advised to join a clinical trial, which will normally always be available at their regional centre.
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Adenosquamous carcinoma, undifferentiated (sarcomatoid) carcinomas and mixed ductal-endocrine carcinomas are closely related to PDAC.
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Cystic tumours are rarer exocrine tumours that cause a cyst or fluid filled sac in the pancreas. Most pancreatic cysts are benign, but some are cancerous. Cystic cancers can have a better outlook (prognosis) than other types of exocrine pancreatic cancer.
In non-specialist centres they may be confused with pancreatic pseudocysts that occur because of inflammation of the pancreas called pancreatitis.
The content of the cyst can be rather watery and this type of cystic tumour is called serous cystadenoma. This type of tumour is benign, meaning non-malignant and non-cancerous. There are three sub-types:
- serous microcystic adenoma(SMA), mainly in occur in the body-tail region and almost all in elderly women, consist of numerous small cysts around a central scar. It accounts for 1% or less of exocrine tumours and between 4 and 10% of all cystic lesions.
- serous oligocystic and ill-demarcated adenmoa (SOIA), mainly in head of pancreas and occur in both sexes, consist of a few relatively large cysts
- von Hippel-Lindau associated cystic neoplasms (VHL-CN). they arise at multiple sites and in advanced disease may merge and involve the entire pancreas and occur in both sexes.
The content of the cyst can be filled with mucin and this type of cystic tumour is called a mucinous cystadenocarcinoma. This type of tumour is malignant, meaning cancerous. MCNs (mucinous cystic neoplasms) affect almost exclusively women (middle aged ladies), predominantly affect the tail of the pancreas, do not impact on the ductal system and may be larger than 20cm. They account for 1 to 2% of exocrine pancreatic tumours
If the cyst presses on the bile duct then this will cause obstructive jaundice. The best treatment is resection. The success rate depends on whether all of the cancer could be properly removed.
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These are cancers that arise from cells lining the main pancreatic duct. These tumours secrete a large amount of mucous (much more than usual) and so the pancreatic duct dilates. The tumours may be benign or malignant.
The tumours may occur in only a small part of the main pancreatic duct or affect the whole length of the pancreatic duct. Small swellings may develop from the sides of the main pancreatic duct full of mucous to give the appearance of cysts. If the mucous enters the bile duct then this will cause obstructive jaundice.
Usually the best treatment of IPMNs is resection. The success rate of treatment of IPMNs is much, much better than the results of treatment of common pancreatic cancer. They are also referred to as IPMTs ( intra ductal papillary mucinous tumours).
They are very slow growing cancers and often cause acute or chronic pancreatitis and they are often diffuse or multifocal (arising simultaneously in more than one part of the organ). They are usually surgically resectable, but sometimes require removal of the whole of the pancreas. Although slow growing about 30-50% do eventually become invasive and metastatize. Among those with a good prognosis are ones occuring in the secondary ducts of the pancreas.
They were originally thought to be very rare but diagnosis has improved and they now account for about 3% of pancreatic cancers and 24% of the cystic type. IPMNs occur slightly more frequently in men than women and the age ranges from 37 to 80 with a mean of 64 years, most patients are older than 50 years.
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These are also called PNETs, short for pancreatic neuroendocrine tumours. These tumours may arise from the endocrine cells in the islets of Langerhans in the pancreas (islet cell tumours) or so called neuroendocrine cells either in the pancreas or ampulla of Vater (carcinoid tumours) or the duodenum (gastrinomas). The success rate of treatment of these PNETs is much better than the results of treatment of other types of pancreatic cancer. They account for less than 2% of pancreatic cancers.
PNETs can secrete one of six or more different hormones causing different types of illness (or syndrome) with strange symptoms and are called functioning neuroendocrine tumours. Most tumors produce an excess of a single type of hormone. However neuroendocrine tumours may not secret any hormones and are then called non-functioning neuroendocrine tumours. The symptoms are different for each type, depending on the hormone the tumour produces. They are:
- Insulinomas produce too much insulin, causing weakness, loss of energy, dizziness and drowsiness. These tumours are nearly always benign, meaning non-malignant and non-cancerous. Insulinoma is the most common and well-known islet cell tumor of the pancreas, accounting for more than 75-80% of sporadic functioning pancreatic islet cell tumors.
- Gastrinomas produce too much gastrin, causing peptic ulcers in the stomach or duodenum. This leads to severe pain, bleeding causing black tarry stools (faeces), and diarrhoea. They occur in the pancreas and in the duodenum. These tumours are mostly malignant, meaning cancerous.
- Somatostatinomas produce too much of a hormone called somatostatin. This causes gall stones, diabetes and diarrhoea with bulky fatty and smelly stools (steatorrhoea). These tumours are mostly malignant, meaning cancerous.
- VIPomas produce too much of a hormone called VIP. This causes a great deal of watery diarrhoea, flushing of the face, and high blood pressure. These tumours are mostly malignant, meaning cancerous.
- Glucagonomas produce too much of a hormone called glucagon. This causes a very specific type of skin rash (redness, ulceration and scabbing), anaemia, mouth ulcers and diarrhoea. These tumours are mostly malignant, meaning cancerous.
- PPomas are also usually malignant, meaning cancerous.
Unlike the common exocrine ductal pancreatic cancer endocrine tumours can affect individuals at any age and they can even arise in small babies. In particular they occur in young-to-middle aged patients. Except for gastrinoma, almost all tumors have a slightly higher incidence in females than in males. No significant sex predilection exists. The average time from onset of symptoms to diagnosis of the tumor is 2.7 years.
PNETs may be inherited and the two main types of hereditary pancreatic neuroendocrine tumours are found in multiple endocrine neoplasia type 1 (MEN-1) and von Hippel-Lindau disease (VHL), but also in the rarer disorders of neurofibromatosis type 1 and tuberous sclerosis. Sporadic islet cell tumors are more common than islet cell tumors as a part of MEN type-I syndrome.
More information on PNETs or the alternative name Gastroenteropancreatic tumours (GEPs) can be found on our page on neuroendocrine cancer.
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The acinar cells are the cells at the ends of the ducts that produce the pancreatic juices.
Acinar cell carcinomas are rare pancreatic neoplasms associated with postresection survival longer than ductal adenocarcinoma but shorter than endocrine carcinoma. Survival for inoperable patients is also longer than for those with common pancreatic cancer.
Pancreatic acinar cell carcinoma is a rare neoplasm, accounting for only about 1% of all pancreatic tumours. These tumours are associated with subcutaneous nodules and ectopic pancreatic fat necrosis. However, little is known about the long-term behavior of these tumours or about their responsiveness to treatment. A study has shown that nearly half of cases had metastases at the time of diagnosis. These lesions were nearly evenly distributed throughout the pancreas (53% in the head, 47% in the body/tail).
The tumours may also contain endocrine cells and when more than 30% of the cells are endocrine they are called mixed acinar-endocrine carcinoma.
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This is a very rare childhood tumour (predominantly age 1 to 8 years) that is even more rarely seen in adults. They are predominantly made up of acinar cells but may show some ductal elements and endocrine cells. Their features overlap those of acinar cell carcinomas. It may be associated with Beckwith-Wiedermann syndrome. Boys are more frequently affected than girls. Unfortunately most are unresectable and the prognosis is poor.
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This is extremely uncommon. Lymphoma is a cancer of the lymphatic system of the body. As the lymphatic system runs throughout the body, it can crop up anywhere. Because it is a different type of cancer, it is treated differently to the other types of pancreatic cancer. More information can be found on our page on lymphoma of the pancreas. Primary or secondary lymphoma may occur in the pancreas. Most primary pancreatic lymphomas (PPL) are non-Hodgkin’s Lymphomas. Malignant lymphomas account for about 3% of all pancreatic tumours.
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Sarcomas are very rare cancerous tumours (less than 1% of all pancreatic tumours) that arise from cells in the connective tissue holding together the cells of the pancreas ie fat, muscles, nerves or blood vessels in or around the pancreas. They most often occur in children. The one we see most often is the GIST (Gastro-Intestinal Stromal Tumour) which develops from muscle cells in the wall of the gut. They often grow very slowly pushing other structures out of the way. Due to their slow progression they often do not cause many symptoms, thus by the time patients are aware of them they may be very large and attached to several different organs. However, because of their less aggressive nature it means that surgery to remove even massive tumours is worthwhile because a cure may still be possible. This extensive surgery may involve removal of parts of other adjacent organs (such as liver, kidney, stomach or colon) as well as part of the pancreas.
They can be primary tumours or metastases to the pancreas from sarcomas originating elsewhere.
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Solid and papillary neoplasms are rare, benign or low-grade malignant tumors more commonly seen in girls and young women. The mass is most frequently located in the tail of the pancreas with solid and cystic components. They have a mean size of 10 cm. These are also called pancreatic papillary cystic neoplasm, solid and cystic neoplasm or papillary epithelial neoplasm. They are of uncertain origin and may possibly be acinar cell or endocrine. The majority behave benignly. It occurs predominantly in young black women with a mean age of 24 years. After complete resection papillary cystic neoplasm has a good prognosis with a low rate of recurrence. Liver metastases have been reported only occasionally.
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This is a rare tumor of the pancreas. Paragangliomas are tumors that arise within the sympathetic nervous system, originating anywhere from the neck to the pelvis in locations paralleling the sympathetic ganglion chain. Tumors of both adrenal and extra-adrenal origin are often active secretors of catecholamines and can cause symptoms such as labile hypertension, palpitations, headache, and sweating. Most tumors are benign but about 10% of paragangliomas may metastasize. They may also be nonfunctional. They show similar characteristics to neuroendocrine tumors of the pancreas. Both paragangliomas and neuroendocrine tumors of the pancreas belong to the category of Amine Precursor Uptake and Decarboxylation (APUD) tumors (APUDomas). Thus, it is important to examine the serum level of pancreatic endocrine hormones and a variety of peptides to differentiate paragangliomas of the pancreas from other pancreatic tumors. Paragangliomas of the pancreas grow slowly, so radical resection is recommended to achieve curability with a good prognosis.
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Rare carcinomas include medullary carcinoma, clear cell carcinoma, ductal adenocarcinoma with a foamy gland pattern and mixed ductal-endocrine carcinoma
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Metastatic disease to the pancreas is rare and can occur from the following:
- Renal cell carcinoma
- Leiomyosarcoma
- Melanoma
- Carcinoid
- Adrenal carcinoma
- Thyroid carcinoma
Angiosarcoma
Other primary sites include the following:
- Lung cancer
- Breast carcinoma
- Ovarian carcinoma
- Hepatocellular carcinoma
In a study of patients referred to the Mayo clinic in the USA the most frequent metastasis to the pancreas was from renal cell carcinoma. This easily can be diagnosed by demonstrating the primary renal tumour but may be more problematic if the tumour was resected and was low-grade.
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In most people the pancreas duct and bile duct join together just before they open into the duodenum through a large fleshy nipple called the ampulla of Vater (after the person who described this). Tumours may arise from the ampulla of Vater. They also cause obstructive jaundice and they tend to affect older patients. Due to their location some ampullary carcinomas have a genetic fingerprint that is similar to PDAC whereas others are more like intestinal carcinomas. The best treatment is resection. The role of adjuvant chemotherapy is uncertain and therefore you should join a clinical trial if possible. The success rate of treatment of these 'ampullary' tumours is much, much better than the results of treatment of common pancreatic cancer. this is usually the easiest of the pancreatic cancers to treat because even very small tumours cause jaundice which means that the cancer is often picked up at an early stage which makes the surgery easier and likely to be more successful.
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A cancer may arise in the bile duct as it travels through the pancreas. They also cause obstructive jaundice and are usually confused with the common pancreatic cancer. In fact the only way of knowing this is for the pathologist to examine the resected specimen under the microscope. The best treatment is resection. The role of adjuvant chemotherapy is uncertain and therefore you should join a clinical trial if possible. The success rate of treatment of these bile duct cancers is much better than the results of treatment of common pancreatic cancer. Lower bile duct tumours are often treated the same as pancreatic cancers because they are hard to tell apart and behave in the same way. This tumour also usually causes jaundice at an early stage. Information on treatment of tumours higher up in the bile duct can be found in sources of information on liver cancer. There is information on trials for bile duct cancer on our trials page.
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Tumours may arise from the duodenum and may cause anaemia bleeding or vomiting but sometimes they also cause obstructive jaundice. The best treatment is resection. The role of adjuvant chemotherapy is uncertain and therefore you should join a clinical trial if possible. The success rate of treatment of these duodenal tumours is much better than the results of treatment of common pancreatic cancer. This rare tumour usually causes a blockage to the exit of the stomach so it is common to get bloating and vomiting as the initial symptoms. Some patients also get anaemia because of blood loss into the gut.
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- Prof J P Neoptolemos, Royal Liverpool University
- Cancer Help UK
- http://www.emedicine.com/radio/topic363.htm
- Exocrine Pancreas Cancer , The European Pancreatic Cancer-Research Cooperative (EPC-RC) edited by T.M. Gress, J.P.Neoptolemos, N.R.Lemoine and F.X.Real ISBN 3-00-016372-7
- http://www.medscape.com/viewarticle/460476_1 Current Opinion in Gastroenterology 2003 vol 19 no 5 Pancreatic Neoplasms Posted 09/16/2003 John P. Duffy, Howard A. Reber
- Cancer of the Pancreas R.C.G. Russell, P.J. Ross and D.C. Cunningham in Oxford Handbook of Cancer
- Types of pancreas tumors from Johns Hopkins Pathology
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