What is Periampullary Tumors?
Ampullary cancer develops from a part of the body called the ampulla of Vater, which is at the intersection of two pipes in your body: one from your liver and gallbladder (also known as the distal common bile duct), and the other from your pancreas. It’s crucial to distinguish this type of cancer from nearby tumors, like those found outside the bile duct or in the pancreas. This is because, treatment methods and patient outcomes can differ greatly. For example, cancers in the pancreas or in the bile ducts (such as cholangiocarcinoma) usually have a more severe forecast and fewer treatment choices compared to ampullary cancers. This remains true even when these diseases are detected early. Recent research has also looked at the types of cells from which ampullary cancers originate. About 47% of ampullary cancers grow from intestinal cells, and about 24% come from cells in the pancreas or bile ducts.
What Causes Periampullary Tumors?
Periampullary tumors are quite rare, accounting for only 6 percent of all cases in that specific area of the body. These tumors generally occur randomly without any specific cause. However, if you have a genetic predisposition, like hereditary polyposis syndrome or hereditary nonpolyposis colorectal cancer, your probability of getting these kinds of tumors can increase by up to 200 times.
Risk Factors and Frequency for Periampullary Tumors
This disease typically takes place later in life, around the age of 70. However, individuals with certain predisposing syndromes might experience it at an earlier age. This early detection is often thanks to screening programs targeting these specific groups.
Signs and Symptoms of Periampullary Tumors
People who are diagnosed with tumors associated with obstructive jaundice usually experience a blockage in the lower part of the bile duct. This often results in the need for further medical evaluation. There are some symptoms that might come along with these tumors. These include:
- Diarrhea
- Weight loss
- Pain that spreads to the back
- Undetected bleeding in the digestive system
- Fatigue
Testing for Periampullary Tumors
For diagnosing and determining the stage of these cancers, doctors use the TNM system, a process developed by the American Joint Committee on Cancer (AJCC). Diagnosis is generally established using specific tests and techniques. These may include an endoscopic ultrasound, which uses a small probe on the end of a tube to create sound waves and make images of the inside of your body. Another test used is Endoscopic retrograde cholangiopancreatography (ERCP), a procedure that combines X-rays and the use of an endoscope (a long, flexible, lighted tube).
Doctors might also use Fine-needle aspiration cytology (FNAC), a technique that uses a thin, hollow needle to remove samples of cells or fluid. If a patient comes in with obstructive jaundice, a condition where the skin and whites of the eyes turn yellow, these tests can allow for treatment intervention and possible placement of a biliary stent, which is a tube inserted into the bile duct to keep it open.
Since many patients end up undergoing surgery, doctors often prefer to use temporary plastic stents rather than metallic ones. After being diagnosed with ampullary cancer, all patients need to be evaluated further using a type of scan known as a computerized tomography (CT) scan of the chest, abdomen, and pelvis. This scan creates detailed images of these areas.
It can often be challenging to differentiate primary ampullary cancers from other cancers around the same area, such as the pancreas, biliary duct, or intestine, during the time of a diagnostic evaluation.
Treatment Options for Periampullary Tumors
Surgery can often cure early-stage diseases. However, it’s been noted that roughly 45% of patients experience a recurrence of their illness over time. Because of this, some patients could benefit from additional treatments such as chemoradiation or chemotherapy after their surgery.
The challenge is, it’s hard to identify the exact best treatment after surgery because this disease is rare, and there haven’t been many exclusive controlled trials. Current information comes from looking back at past data or combined results where these tumors were treated alongside other related cancers.
For advanced cases of this disease that have spread to other parts of the body, surgery might not be the best option. In these scenarios, these tumors are typically treated with chemotherapy regimens, similar to treatments used for pancreatic or colorectal cancers.
What else can Periampullary Tumors be?
Ampullary cancer symptoms can often look very similar to symptoms of other cancers, specifically cancers of the distal biliary duct (cholangiocarcinomas), pancreas, or colon. A type of test known as FNAC might find it hard to tell the difference between ampullary cancer and other cancers that are located around the same area.
Only a definitive surgery can clearly identify the type of cancer based on examining the cells (histology). Looking at how the disease progresses, ampullary cancers are known to have a better prognosis, meaning patients generally have a better chance of recovery compared to other cancers in the surrounding area.
Surgical Treatment of Periampullary Tumors
Ampullary cancer, a type of cancer occurring in the bile duct, can potentially be cured through surgical removal. This often involves a procedure known as a pancreaticoduodenectomy, or a ‘Whipple procedure’, which may or may not involve preserving the part of the stomach called the ‘pylorus’. Best outcomes from this surgery are seen in hospitals where the procedure is performed frequently.
The risk of dying during or just after the surgery is less than 5%. However, 20 to 40 percent of patients do experience complications such as leaks in the connections made during surgery or delayed emptying of the stomach. These complications are often seen in older patients – who are most likely to get ampullary cancer – particularly when they have other health conditions at the same time.
In some cases, less intensive surgical procedures like ‘ampullectomies’ can be considered. But as these are less frequently used treatments, we don’t have much data on their effectiveness. Similarly, there isn’t much evidence or consensus on the use of treatments before surgery in specific groups of patients. It is highly recommended that a team of different health professionals evaluate each patient to figure out the best treatment approach for them.
What to expect with Periampullary Tumors
The chance of recovery or prognosis largely depends on how far the disease has spread locally, how successful the surgery was in removing the disease, and whether it has spread to the lymph nodes. The presence of disease in the lymph nodes is a key factor in predicting the overall chance of recovery. If the disease hasn’t spread to the lymph nodes, the survival rate is 70% to 80% for five years. However, if the disease has spread, the survival rate decreases to 20% to 50%.
Recently, research has suggested that the type of cell examined under the microscope (histology phenotype) might play a role in predicting the chance of recovery. Specifically, pancreatobiliary type cells (cells that originate from the pancreas or bile duct) have a worse prognosis compared with intestinal type ones.
Possible Complications When Diagnosed with Periampullary Tumors
Cancer in the ampulla — the area where your bile and pancreatic ducts meet and empty into your small intestine — often blocks your bile duct, causing complications. There can also be side effects related to the treatment of ampullary cancer, including complications from surgery, chemotherapy, and radiation treatment.
Side Effects:
- Bile duct obstruction
- Surgery-related complications
- Chemotherapy side effects
- Radiation treatment complications
Recovery from Periampullary Tumors
Currently, there are no specific rules on how to monitor patients after cancer treatment. However, many cancer doctors tend to see their high-risk patients every 3 to 6 months for about 5 years to check for any signs of the disease coming back. It is often suggested that patients who have had a small surgical procedure to remove the cancer (local resection) should have a medical exam to look inside the body (surveillance endoscopy) every six months for two years, then once a year for up to 5 more years. The usefulness of frequent body scans (CT scans) for this monitoring process is less certain.
Preventing Periampullary Tumors
Ampullary cancer is often seen in older people. If you notice that your urine has changed color or your skin has turned yellowish, these could be signs that you have this type of cancer, and you should seek medical attention immediately. Jaundice, which is the yellowing of the skin or the whites of the eyes, can be a symptom of ampullary cancer. The good news is, if detected early on, this kind of cancer can be cured. That’s why it’s so important to get diagnosed promptly and if necessary, undergo surgery.
