What is Intraductal Papillary Mucinous Neoplasm of the Pancreas?
Intraductal papillary mucinous neoplasms (IPMN) are growths in the pancreas that form within the ducts of the organ and produce a substance called mucin. These growths have the potential to turn into cancer. Because of this, guidelines have been established to identify which patients will require surgery. These guidelines usually depend on different factors identified through body scans which include the size of the growth, if a small bump is present, changes in the structure of the growth, blockage of the main duct, and if symptoms are present.
What Causes Intraductal Papillary Mucinous Neoplasm of the Pancreas?
It’s not entirely known what causes IPMNs, which are a type of pancreatic cyst. However, there are a few conditions that might make a person more likely to develop IPMNs. These include diabetes (especially if a person is using insulin treatments), chronic inflammation of the pancreas, called pancreatitis, and a family history of a specific type of pancreatic cancer known as pancreatic ductal adenocarcinoma.
Smoking has been identified as a risk factor for developing pancreatic cancer, and it is also associated with an increased risk of developing IPMNs.
Risk Factors and Frequency for Intraductal Papillary Mucinous Neoplasm of the Pancreas
The actual rate at which IPMNs (intraductal papillary mucinous neoplasms) occur is uncertain since they often don’t show any symptoms and are quite small. Research that has examined the internal pictures of patients without any prior pancreatic health issues found that between 2.6% to 13.5% of adults possess pancreatic cysts. This occurrence is connected to older age. Typically, these conditions present themselves between the age of 50 and 70.
Signs and Symptoms of Intraductal Papillary Mucinous Neoplasm of the Pancreas
Intraductal papillary mucinous neoplasms (IPMNs), a type of pancreatic tumor, can exhibit a range of symptoms that lead to their identification. Sometimes these tumors don’t cause any noticeable symptoms and are only discovered during medical imaging for an unrelated reason. If symptoms do appear, they tend to be fairly general and can include:
- Abdominal pain
- Back pain
- Nausea
- Vomiting
- Loss of appetite
- Weight loss
- Jaundice (yellowing of skin and eyes) due to blockage of the bile ducts
- Symptoms similar to pancreatitis, caused by obstruction of the main pancreatic duct by a thick, jelly-like substance called mucin.
Testing for Intraductal Papillary Mucinous Neoplasm of the Pancreas
When your doctor thinks you might have an intraductal papillary mucinous neoplasm (IPMN), or in simpler words – a type of growth that occurs within your pancreatic ducts, they start by ordering certain scans to get a closer look at the growth along with the nearby structures. They also try to determine if the disease has spread to other parts of the body, which is known as metastasis.
The first imaging method usually used is an MRI (Magnetic Resonance Imaging), combined with MRCP (Magnetic Resonance Cholangiopancreatography). This is sort of a supercharged MRI which gives your doctor detailed images of your pancreas. However, sometimes, an MRI might not be appropriate for every patient. In these cases, another type of scan, a CT (Computed Tomography), is the go-to method. It also gives a detailed picture of your pancreas and the surrounding area.
If your scans reveal some features that might suggest the possibility of cancer, your doctor may advise an endoscopic ultrasound (EUS) with fine-needle aspiration (FNA). This method involves inspecting the growth using an ultrasound probe fitted on a flexible tube (the endoscope), which is introduced via your mouth and stomach into the first part of your intestine which is closest to the pancreas. If needed, they can insert a thin needle to take a small fluid sample from the growth (this is the FNA part).
The fluid collected can be tested in a lab for the presence of abnormal or cancerous cells. Also, the levels of a certain marker, carcinoembryonic antigen (CEA) are measured in the fluid, as they can help further with the diagnosis.
Based on these results, if the growth contains a solid part, or the main pancreatic duct itself is dilating (widening), or if there are signs of cancer picked up from the ultrasound or the fluid sample, the doctor might suggest removing the growth surgically. If none of these concerning features are present, your doctor would likely advise periodic MRIs to monitor the growth.
Treatment Options for Intraductal Papillary Mucinous Neoplasm of the Pancreas
If patients with IPMNs (Intraductal Papillary Mucinous Neoplasms, which are non-cancerous growths in the pancreatic ducts) show signs of high-grade dysplasia (abnormal cells) or if the IPMNs turn into invasive carcinoma (cancer), surgery is usually needed. Surgery is also generally recommended for all mucinous neoplasms (another type of non-cancerous growth) and main duct neoplasms (tumors in the main pancreatic duct).
However, when it comes to branch duct cysts (cysts or sacs in the smaller ducts of your pancreas), doctors may choose to just keep an eye on them instead of resorting to surgery. If a patient with invasive ductal adenocarcinoma of the pancreas (a common type of pancreatic cancer) undergoes surgery, post-surgery supplementary treatment can improve their chances of survival. This is even true for patients where cancer cells are found at the surgery’s edges or in nearby lymph nodes. But, we don’t have a consensus on what the best post-surgery treatment is yet.
Doctors usually reserve surgeries like pancreaticoduodenectomy (Whipple’s procedure – removing the head of the pancreas) or distal pancreatectomy (removing the tail of the pancreas) for severe cases due to the significant risks and potential complications associated with these procedures. When deciding whether surgery is the best option, a variety of factors need to be considered. These include the patient’s age, their overall health, the risk of the lesion turning cancerous, and how suspicious doctors are that it could be malignant. If a cyst does not meet the necessary criteria for surgery, it is typically monitored periodically using imaging techniques.
If the main pancreatic duct has enlarged to 1.5 cm or more, doctors generally recommend surgical removal. If the disease affects the entire duct, clinicians often suggest removing the entire pancreas. If only the head of the pancreas is affected, they usually recommend going with the Whipple’s procedure. Lastly, if the disease is present in the tail of the pancreas, they could recommend removing the tail of the pancreas, and maybe even the spleen.
What else can Intraductal Papillary Mucinous Neoplasm of the Pancreas be?
There are several medical conditions that can be mistaken for the disease called Intraductal Papillary Mucinous Neoplasm (IPMN). These include, but are not limited to:
- Pancreatic pseudocysts
- Serous cyst tumors
- Mucinous cyst tumors
- Solid pseudopapillary neoplasms
To tell these conditions apart, doctors often use imaging techniques such as MRI scans, MRCP, and EUS with FNA.
Surgical Treatment of Intraductal Papillary Mucinous Neoplasm of the Pancreas
In a particular surgical procedure, a cut is made in the upper middle part of your belly. After this, a special part of the stomach, called the lesser sac, is opened using a device known as a harmonic scalpel. The duodenum (first part of your small intestine) is then moved out of the way in a process called Kocherization.
Upon identifying the pancreas, the surgeon will carefully remove the affected tissue that is causing the Intraductal Papillary Mucinous Neoplasm (IPMN), which is a type of pancreatic tumor. During this process, the surgeon tries to keep as much of the healthy pancreatic tissue as possible to avoid potential side effects, such as diabetes and problems relating to the body’s ability to digest food, which can happen if too much pancreas is removed.
One technique used during surgery is to feel the pancreatic duct. If it feels larger than normal, it may need to be removed. This is because a larger duct might indicate that there is too much mucus being produced inside the pancreas, which is not normal. Once the tissue is removed, it is quickly sent to a laboratory for a “frozen section” procedure. This technique allows the pathologist to rapidly analyze the tissue and determine if all the tumor has been removed. This confirmation is essential for the success of the surgery.
What to expect with Intraductal Papillary Mucinous Neoplasm of the Pancreas
The five-year survival rate (which means the percentage of people who live at least five years after being diagnosed) for IPMNs, a type of pancreatic cyst, is around 45 to 50%. This is in comparison to pancreatic adenocarcinoma, a type of pancreatic cancer, which has a five-year survival rate of 8%.
Possible Complications When Diagnosed with Intraductal Papillary Mucinous Neoplasm of the Pancreas
After an operation, there can be unwanted issues like bleeding, a leak in the surgical join, an abscess, or even death. One particular complication to look out for is a fistula (an abnormal or surgically made passage) between the pancreas and the skin. Usually, this is managed by:
- Administering total parenteral nutrition (TPN), which provides all the nutrients the patient needs intravenously (via a drip)
- Not allowing the patient to eat or drink anything
- Drainage of the fistula
- Potentially using somatostatin analogs, a type of medicine, to treat the condition [16].
Recovery from Intraductal Papillary Mucinous Neoplasm of the Pancreas
It’s generally advised that patients begin walking around as soon as possible after a surgical procedure. If a drain was put in place during the surgery, it should only be removed once the patient is able to eat solid food without an increase in the fluid coming out of the drain. If there’s a chance that a fistula, or abnormal connection, has formed in the pancreas, the fluid from the drain should be tested for increased levels of enzymes called amylase and lipase, which are usually higher if a fistula is present.
Preventing Intraductal Papillary Mucinous Neoplasm of the Pancreas
Patients who have diabetes need to learn more about their condition. They should have regular follow-up appointments with their primary care doctor. The doctor can help guide them on how to manage their diabetes and prevent it from getting worse. For patients who smoke, it’s important to seek help to quit. There are many resources available that can assist in quitting smoking.
