Dr. Aditya Kulkarni is a Consultant Laparoscopic and Robotic Gastrointestinal, Hepato-biliary-pancreatic and Cancer Surgeon at the renowned Ruby Hall Clinic,
Periampullary region; the circle shaded blue is the area where periampullary carcinoma arises
The ampulla of Vater is a mound-like opening in the duodenum. The duodenum is a C shaped loop of bowel at the beginning of the intestine. It is where the bile duct bringing bile from the liver and pancreatic duct which brings pancreatic juice from pancreas meets and then opens into the intestine. The term periampullary cancer includes cancer occurring around the ampulla of Vater. These are the cancer of the head of the pancreas, distal bile duct (cholangiocarcinoma), duodenum and ampulla. These cancers are clubbed together as a group because the clinical features and treatment are similar for them. All of them are adenocarcinomas but the prognosis varies for them. Surgical removal of these tumours, if possible, offers the best chances of cure.
PERIAMPULLARY CANCER TYPES
- Pancreatic cancer: This cancer originates from the cells of the pancreas. Read more here.
- Distal bile duct cancer (cholangiocarcinoma): This cancer originating from the lining of the lowermost part of the bile duct where it passes through the pancreas, joins the pancreatic duct and ends in the intestine.
- Duodenal cancer: It arises from the lining of the duodenal mucosa. Majority of duodenal cancers originate in the second part of the duodenum, where ampulla is located.
- Ampullary cancer: It arises from the lining of the ampulla of Vater.
RISK FACTORS OR CAUSES
The risk of someone getting periampullary cancer increases with age. Another common risk factor is regular smoking and drinking of alcohol. Increased intake of sugar and red meat with reduced intake of fruits and vegetables has also been incriminated.
Pancreatic cancer: you can read about risk factors for pancreatic cancer here .
Risk factors for bile duct cancer (cholangiocarcinoma) are infestation with some parasites such as liver flukes, primary sclerosing cholangitis, gallstones, choledochal cysts, diabetes and obesity.
Risk factors for duodenal cancer include inherited conditions like Familial adenomatous polyposis (FAP), Gardner syndrome, HNPCC or Lynch syndrome, juvenile polyposis syndrome and Peutz-Jeghers syndrome. Crohn’s disease, celiac disease and duodenal polyps also increase the risk.
Risk factors for ampullary cancer are similar to the above types.
SIGNS AND SYMPTOMS
Periampullary cancers compress the bile duct, blocks the flow of bile and cause yellowing of eyes and urine called jaundice. This is usually accompanied by pale stools and itching. Other warning signs of periampullary cancer can be weight loss, loss of appetite and abdominal pain. Besides, duodenal and ampullary cancers bleed causing black stools (melena) and anaemia.
Signs and symptoms of periampullary cancer
- Jaundice (yellowing of skin, eyes and urine with pale stools)
- Abdominal pain
- Weight loss and loss of appetite
- Recurrent vomiting
- Black stools
DIAGNOSIS AND STAGING
Once periampullary cancer is suspected, it is diagnosed and staged by a high resolution, thin cut, triple-phase CT scan or PET scan. Blood tests will be done to quantify your jaundice, check your kidney function, haemoglobin and blood clotting parameters. A tumour marker called CA19.9 will also be checked. A chest X-ray or a CT scan of chest will be done to look for any tumour in the chest.
Side viewing endoscopy: it is a procedure in which a thin flexible tube is passed into the intestine through the mouth. It has a camera at the tip of the tube and a view of the ampulla is obtained, showing any cancerous growth.
Biopsy: if a tumour is found at the ampulla, then a small sample from it is obtained called as biopsy and examined under microscope confirming the diagnosis.
Endoscopic ultrasound (EUS): It aids diagnosis in cases where CT/MRI do not show cancerous growth, while it is suspected on clinical features. It is also used to take a sample from the tumour by passing a needle called fine needle aspiration cytology (FNAC) for confirmation of diagnosis if required.
TREATMENT OF PERIAMPULLARY CANCER
The treatment depends upon the stage of the disease and degree of jaundice. Surgery is the best possible option and can be considered if the cancer is diagnosed at a stage where it can be completely removed by surgery. It may be necessary to decrease jaundice before surgery by doing a procedure called endoscopic retrograde cholangiopancreatography (ERCP) and stenting. In this, a plastic or metallic tube called stent is placed in the bile duct which is blocked by tumour and opens it up from inside.
If the tumour is advanced then neoadjuvant treatment (chemotherapy or chemoradiotherapy) to decrease the size of the tumour may be given before surgery. For unresectable tumours, an attempt to made to downstage them to a stage where they can be surgically removed. For this neoadjuvant treatment is administered and some of these patients will become operable.
The operation to surgically remove periampullary cancer is called Whipple operation , also known as pancreaticoduodenectomy . In this, head of the pancreas is removed along with duodenum, bile duct, gall bladder, part of the stomach, a small part of the small intestine and adjacent lymph nodes. To restore gastrointestinal continuity, the small intestine is then joined to the pancreas (sometimes pancreas is joined to the stomach), remaining bile duct and stomach.
Chemotherapy uses drugs to destroy cancer cells. For cancers which have spread to distant Surgery in organs of the body (metastatic), surgery is not an option. After FNAC/biopsy and stenting (if jaundiced) chemotherapy is given.
Endoscopic retrograde cholangiopancreatography (ERCP) and stenting: In this, a plastic or metallic tube called stent is placed in the bile duct which is blocked by tumour and opens it up from inside alleviating jaundice.
If the tumour is causing blockage of food passage then a metal stent is placed in the food passage endoscopically. If this fails then a bypass surgery will be required.