Peri-Ampullary Cancer Survivor

Endoscopy pictures courtesy of GastroLab


Hooray !!! May 16 will be my 17th anniversary as a cancer survivor. Had a whipple operation (pancreaticduodenectomy) after DX of Peri-Ampullary CA in 1991.


I would like to write about this cancer which is rare type, if diagnosed early it would save many people from dying., when it's not discovered, it is usually advanced. Had I waited for the doctor to guess my disease, I would have died long ago. I was insistent and a demanding patient. I asked too many questions. Get 2 or 3 doctor opinions. I thank God that I had early symptoms.
I was a healthy, non-smoker/drinker female, but I recall that when I was younger, I disliked vegetables, milk, and picky on foods that I ate, irregular bowel movements, etc.

I never stop reading and researching about Pancreatic CA. There was no internet way back in 1991 so reading about this subject is limited to medical textbooks and medical journals only. I joined and read the forums of cancer organizations to see how survivors are doing and how they are coping with their health problems. People who have underwent whipples like me, will understand and feel what its like to be on the same boat. It's a tough ride, especially when you don't know yet your death sentence, but once you are diagnosed and staged of your Cancer, you'll feel relax but the battle is a long journey. Prayer is powerful, only God knows when our time will come. I highly recommend that surgery is the only potential curative treatment if its operable. CAT Scan is very expensive here in the Philippines but this is the fastest way to pinpoint the problem. I wish CTscan prices will be just like the ordinary X-ray test which is affordable.


FACTS:
Peri-ampullary
Around the ampulla of Vater. The peri-ampullary region is comprised of 4 structures; the ampulla, the duodenum, the bile duct and the head of the pancreas. It is sometimes difficult to tell which structure a tumor originated in. In such cases the diagnosis will be a peri-ampullary tumor.

Periampullary cancer, a pancreatic cancer affecting the ampulla Vater region, is a subgroup of pancreatic cancers. About 10-15% of all pancreatic cancers are located in this region. It is often impossible to decide if the tumour is arising from the distal bile duct or from the pancreas in an endoscopic finding like this. The typical symptom is obstructive jaundice.

Periampullary cancers constitute a distinct entity compared to the classical pancreatic head cancer. Their diagnosis and the approach to their treatment has improved considerably in the last two decades. Endoscopy, high-resolution imaging, and endosonography have resulted in improved diagnosis and staging. A pancreatoduodenectomy offers the only chance of cure and improves survival. Superior results are seen with high volume centres. While endoscopy plays an invaluable role in the palliation of obstructive jaundice in unresectable lesions, its role in preoperative stenting remains uncertain. Adjuvant treatment modalities have so far failed to significantly improve survival. These tumours carry a better prognosis than the more dismal pancreatic head cancer, possibly because of the activation of different molecular pathways in the process of carcinogenesis. This article reviews the current understanding and various treatment options of periampullary carcinomas other than the classical pancreatic head cancer.

Periampullary tumours are defined as those that arise within 2 cm of the major papilla in the duodenum. They encompass tumours of the ampulla of Vater, the distal common bile duct (intrapancreatic distal common bile duct), duodenal tumours (usually the second part) involving the papilla and tumours of the pancreatic head involving the ampulla.Thus, these tumours are classified on the basis of their tissue of origin. They constitute a separate entity from the classical adenocarcinoma of the pancreatic head. While these tumours can be benign, they are most commonly malignant and periampullary adenomas are well-known premalignant lesions. Periampullary adenocarcinomas carry a better prognosis than adenocarcinoma of the pancreatic head. While these tumours have different origins, the complex regional anatomy dictates a common operative approach. Malignant periampullary tumours are best treated by a pancreatoduodenectomy. Resection is the only option that improves survival. This review article discusses the current understanding about periampullary carcinomas, other than the classical pancreatic head cancer, and the various treatment options.

Historical Facts about the Pancreas
The pancreas was first described in 300 B.C by Herophilus of Chalcedon. In 100 A.D Aretaus described the term diabetes. It was Rufus of Ephesus who coined the term "pan-kreas" meaning 'all flesh'. In 1541 Andreas Vesalius first illustrated the pancreas and in 1642 Johann Wirsung discovered the pancreatic duct. In 1654 Francis Glisson of Cambridge described the sphincter mechanism at the end of the common bile duct. In 1674 Thomas Willis of Oxford described diabetes mellitus. In 1869 Paul Langerhans discovered the Islets of the pancreas where Insulin was made and in 1872 Alexander Danilewski discovered trypsin, the protein digesting enzyme, made in the pancreas. In 1922 Banting and Best discovered Insulin a hormone that controls blood sugar levels.

Pancreatic Surgery


Pancreatic surgery really started in 1879 when Thiersch first drained a fluctuating tumour in the abdomen which was a pancreatic cyst. The resulting pancreatic fistula eventually dried up spontaneously. However, the first serious pancreatic surgery began in the 1900s and in 1909 Coffey performed the first pancreatic anastomosis (join) to the bowel. In 1923 Jedlicka successfully anastomosed a pancreatic cyst to the back of the stomach and in 1946 Konig drained a pancreatic cyst into a Roux-en-Y loop of jejunum. In 1882 Trendelenburg carried out the first distal pancreatectomy (left sided pancreatic resection). The first successful removal of a peri-ampullary cancer was carried out by William Halsted in 1898 and this involved local excision.
The first true pancreatico-duodenectomy was carried out by Kausch in 1909 operating on a 49 year old man. This was a two stage procedure. Subsequently in 1935 A.O.Whipple performed a two stage pancreatico-duodenectomy. By 1940 he had perfected the single stage operation and by 1941 over 40 cases had been reported with an operative mortality of 27 %. To date there are many variations in the type of reconstruction of the Kausch-Whipple procedure. The procedure of pylorus-preserving pancreatico-duodenectomy was popularised by Traverso and Longmire in 1978.


Some symptoms of pancreatic cancer:
JaundiceThe head of the pancreas is located close to the common bile duct and small bowel (duodenum) so tumors located in the head of the pancreas may grow and block these structures. Bile duct blockage can lead to jaundice in 70-85% of patients with tumors in the head of the pancreas. Symptoms of jaundice include:
yellowing of the skin
yellowing of the whites of the eyes
light-colored (clay colored) bowel movements
dark-colored urine
itching
Nausea, Vomiting, Weight Loss Stomach emptying can be delayed when the small bowel is compressed. This causes a feeling of fullness and contributes to symptoms of nausea and vomiting. These symptoms are initially present in 35-45% of patients with pancreatic cancer. Patients sometimes have a loss of appetite and nausea that leads to weight loss.In advanced disease, up to 5% of patients have tumors that cause complete blockage of the small bowel. In this case, surgery may be done to bypass the blockage and improve digestion.
FatigueIn more advanced disease, patients can have a feeling of being tired and fatigued. There are many causes of fatigue in patients with cancer.
Abdominal Pain 75-90% of patients with pancreatic cancer have abdominal pain. This may be described as cramping or gas-like pain. Pain may also spread, or radiate, to the back and shoulders.
Blood Clots - There is an increased risk for blood clots in patients with pancreatic cancer. A blood clot can be a first symptom of pancreatic cancer. The cancer itself causes changes in the blood that increases the risk for blood clots. Some clots have no symptoms, but swelling, pain and redness can be present in the area of clot.
DiabetesThe onset of diabetes or difficulty in controlling blood sugar levels is also associated with pancreatic cancer. Diabetes may be diagnosed before or after the diagnosis of pancreatic cancer. Diabetes occurs when the body does not produce enough of the hormone, insulin, or is unable to use insulin properly. Insulin helps your body regulate the blood sugar level, so without it blood sugar levels are often high.
Pancreatic InsufficiencyThe pancreas secretes digestive enzymes, or juices, into the small intestine. These juices help digest food in the intestines. Patients with pancreatic cancer may not have adequate amounts of these enzymes, and food isn't digested normally. This is called pancreatic insufficiency.Symptom include diarrhea and cramping often after eating. This happens because food passes through the digestive tract not fully digested. As the indigestible food passes through, it pulls water into the intestines, causing diarrhea and cramping.


How is it diagnosed?
Patients with symptoms suspicious for pancreatic cancer will undergo tests to determine the cause of these symptoms. Below are some test and procedures used for diagnosis:
Ultrasound of the abdomen - An ultrasound can identify a tumor or mass in the pancreas or bile duct system that may be causing blockage or jaundice.
Endoscopic Ultrasonography (EUS)The EUS test is done with a lighted tube that is inserted through the mouth and placed into the stomach. Ultrasound images of the pancreas are obtained through the stomach wall. It is highly sensitive for diagnosing pancreatic cancer. EUS is particularly useful for detecting small (less than two centimeters) tumors which may not be well visualized by CT. It can also identify tumors that may involve important blood vessels. The procedure can provide details about the arteries and veins next to the pancreas.A biopsy with a small or 'fine' needle aspiration (FNA) of the tumor may also be performed during an EUS to diagnose pancreatic cancer. Intravenous sedation is used for this procedure.
ERCP (endoscopic retrograde cholangiopancreatography)An ERCP is done with a lighted tube called an endoscope to look at the bile ducts. It can also be used to place a stent or tube to open a blocked bile duct for drainage. Intravenous sedation is most commonly used for this procedure. The patient is not awake during the test. This procedure helps to determine what is causing the blockage.Some causes of these blockages include ampullary tumors, cholangiocarcinoma (bile duct cancer), inflammation or cancer of the pancreas. Bile duct juice and tissue samples may be obtained and sent to the pathologist to evaluate for cancer cells.
Computed Tomography (CT)The CT scan can show small tumors as well as important blood vessels that the tumor might be growing into or around. A CT scan can also look at surrounding organs for spread (metastasis) of the cancer into the lymph nodes, liver and other areas. The doctor may suggest a pancreatic mass CT to be done for the first clinic visit to better assess the tumor for size, location and involvement of surrounding vessels and organs. This is a special CT scan done at the Cancer Center that examines the pancreas very closely. A special dye is used for the CT, to give additional information to the radiologist; therefore a temporary IV (intravenous catheter) will be placed prior to the scan.
Interventional Radiology - In some cases the radiology department can perform needle biopsies using the CT or ultrasound technique to locate the mass (tumor) and obtain a tissue sample through the abdomen for a diagnosis. At the UM Interventional Radiology department, there are specialists who may be consulted to do one of these procedures.



Diagnosing pancreatic cancer can be a challenge. It is often difficult to get an adequate biopsy for the pathologist to look at under the microscope. It is frustrating for the patient and the doctor who want to move ahead quickly in making a diagnosis and beginning treatment.


What are the types of cancer found in the pancreas?
There are many different types of pancreatic tumors; however, pancreas cancer is mainly (95%) ductal adenocarcinoma. This type of pancreatic cancer arises from the lining of the pancreatic duct which is the exocrine part of the pancreas that produces the digestive juices.
Tumors may arise from the endocrine part of the pancreas in approximately 5% of cases. This is sometimes referred to as Islet (insulin producing) cell or neuroendocrine cancer. Even rarer tumors are sometimes found, such as sarcomas or lymphomas.


Ampullary Carcinoma
Ampullary carcinoma is suspected based upon demonstration of obstructive jaundice, often with dilation of the pancreatic and biliary ducts seen on abdominal imaging studies. A discrete mass may or may not be identifiable using standard transabdominal ultrasound or helical computed tomography (CT) scanning. Endoscopic retrograde cholangiopancreatography (ERCP) allows for direct identification and biopsy confirmation, although biopsy is not 100% accurate . Magnetic resonance cholangiopancreatography (MRCP) may allow identification of the lesion and obviate diagnostic ERCP. Endoscopic ultrasound (EUS) allows for more accurate diagnosis and staging of these lesions than CT, and also allows for forceps and fine-needle aspiration (FNA) tissue sampling. EUS may also allow selection of patients that can undergo local resection instead of pancreaticoduodenectomy (Whipple operation). Once the lesion is identified and staged, palliation of jaundice or operative resection for cure is similar as is discussed for carcinoma of the pancreatic head.



Sources: pancreatic cancer UK/indianjsurg.com/emedicine.com