What Is Ampullary Cancer?
Ampullary cancer is an uncommon form of carcinoma that develops in the ampulla of Vater. The small orifice known as the ampulla of Vater is found where the pancreatic and bile ducts converge. The pancreas and liver are two other digestive system organs that are close to the ampulla of Vater. In approximately 47 percent of cases, ampullary carcinoma is in the intestinal epithelial, and almost 24 percent of cases are in the pancreatic-biliary epithelial. Ampullary carcinoma should be distinguished from periampullary tumors, such as extra biliary or pancreatic tumors, as there is a vast difference in clinical symptoms and therapeutic management.
What Are the Causes of Ampullary Cancer?
It is a rare condition and generally haphazardly or irregularly occurring. However, genetic conditions such as hereditary polyposis syndrome or nonpolyposis colorectal cancer can raise the risk of incidence 200 times.
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Hereditary Polyposis Syndrome: Development of various polyps (lumps formation from normal tissue) in the digestive tract, especially the colon or rectum. This is a hereditary condition that is inherited from parents.
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Hereditary Nonpolyposis Colorectal Cancer Syndrome: It is an autosomal inherited condition that is associated with a higher risk of developing colon cancer as well as other malignancies such as endometrial cancer, stomach, ovary, small intestine, hepatobiliary, and many more.
Who Is Affected by Ampullary Cancer?
The majority of cases of ampullary carcinoma occur in those over 70. The illness is more likely to affect men than women.
What Are the Symptoms of Ampullary Cancer?
The general symptom during diagnosis is obstructive jaundice due to blockage in the biliary duct.
Other symptoms which can be present are:
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Weight loss and alteration in appetite.
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Diarrhea: Passing watery, loose stools at least three or more times a day.
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Radiation of Pain: The pain due to malignancy radiates to the back of the body.
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Fatigue: Tiredness and weakness.
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Occult Bleeding: Small stool blood can be detected only in feces.
How to Diagnose Ampullary Cancer?
1. Endoscopic Retrograde Cholangiopancreatography (ERCP): It is a diagnostic procedure used to investigate and treat the disorder in the liver, gallbladder, bile duct, and pancreas. A long, flexible, and light tube called an endoscope is used with X-rays.
2. Fine Needle Aspiration Cytology (FNAC): This technique is used to evaluate the cytological nature of an outgrowth or tumor by inserting a fine needle and collecting the sample. It is a rapid and reliable diagnostic procedure.
3. CT-Scan: A computed tomography scan of the abdomen, chest, and pelvis is essential to evaluate the size and position of cancer for staging. The TNM system from the American Joint Committee on Cancer (AICC) is used for staging ampullary cancers.
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Stage-I: Vegetating tumor, which is limited to the epithelium with no involvement of the sphincter of Oddi.
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Stage- II: Tumor localized in the duodenal submucosa, without the duodenal muscularis propria, but there can be possible involvement of the sphincter of Oddi.
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Stage- III: Tumor in the duodenal muscularis propria.
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Stage- IV: Tumor of the periduodenal area of the pancreas, with proximal or distal lymph node involvement.
4. Other Imaging Tests: Other tests such as magnetic resonance cholangiopancreatography and endoscopic ultrasound also help determine the tumor's spread beyond the ampulla of Vater.
What Is the Treatment of Ampullary Carcinoma?
Although surgery offers a chance of curing disease early, approximately 40 to 45 percent of treated cases develop recurrence over a while. Therefore adjuvant treatment modalities such as radiation and chemotherapy can benefit the patients.
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Surgical Oncology: Surgical resection with pancreaticoduodenectomy also called the Whipple procedure, with or without preserving the pylorus is the only curative potential treatment. This procedure removes the head of the pancreas, a portion of the small intestine (duodenum), the gallbladder, and part of the bile ducts. The perioperative mortality (death rate) is less than about five percent. However, the perioperative morbidity (chances of diseases) is approximately 20 to 40 percent, with delayed gastric emptying and anastomotic leak. Ampullectomies is a less aggressive surgical method used to treat ampullary cancer; most patients are older in their sixth or seventh decade of life.
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Radiation Oncology: It is a preventive measure in patients with a higher risk of recurrence. Generally, a dose of 50.4 grays is given in four to six weeks concurrently with Fluoropyrimidine or Gemcitabine. Radiation therapy can be either sandwiched between the chemotherapy or after four to five months of chemotherapy.
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Medical Oncology: In cases of recurrence risk, especially when staging is T2 or above, alternative treatment with systemic administration of Gemcitabine or Fluoropyrimidine for six months is done.
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Targeted Therapy: Utilizing medications that target particular compounds in cancer cells is known as targeted therapy. Targeted therapies can kill cancer cells by preventing these substances from functioning. When ampullary cancer returns after treatment or spreads to other regions of the body, targeted therapy is utilized to treat it. It is only applied in specific circumstances.
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Combined Radiation and Chemotherapy: Chemotherapy uses powerful medications to treat cancer. Radiation therapy uses intense energy beams to treat cancer. Protons, X-rays, and other sources are possible sources of energy. When combined, these therapies might be more successful in treating ampullary tumors. To increase the likelihood that cancer can be removed during surgery, chemotherapy, and radiation therapy may be administered in combination prior to the procedure. After surgery, the combination treatment can be utilized to eradicate any cancer cells that may still be present.
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Immunotherapy: A medical procedure called immunotherapy stimulates the immune system to destroy cancer cells. The immune system targets bacteria and other foreign cells in the body to prevent illness. Cancer cells evade the immune system in order to survive. Immunotherapy aids in the immune system's ability to identify and eliminate cancerous cells. If ampullary cancer returns after therapy or spreads to other parts of the body, it might be a possibility. Immunotherapy is reserved for specific circumstances.
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Treatment to reduce the pain and discomfort is done by placing a stent (generally made of plastic) to drain the fluid, which causes the eyes and skin to be yellow. It helps to remove the obstruction and relieve the pain.
What Is the Prognosis of Ampullary Cancers?
The prognosis widely depends upon the staging of tumors, their local imitation, the status of surgical margins after definitive surgery, and the presence of nodal metastasis. Node positivity is the most crucial factor which determines the overall prognosis. The five-year survival rate of patients ranges from 70 to 80 percent for node-negative cases compared to 20 to 50 percent for node-positive diseases.
Which Diseases Are Similar to Ampullary Cancer?
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Cholangiocarcinoma (Distal Biliary Ductal Cancer): A rare malignancy formed in the bile duct that carries bile from the liver to the gallbladder and then to the intestine.
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Colon Cancers: The last part of the gastrointestinal tract is the colon. Usually, colon cancers originate as small, benign lumps of cells called polyps which gradually become malignant.
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Pancreatic Cancers: Pancreas is an organ that is both exocrine and endocrine. It releases enzymes that involve digestion and produces hormones that help manage and regulate blood sugar levels.
Conclusion:
Ampullary carcinoma is a serious condition as it is malignant. It can affect surrounding organs such as the liver, intestines, and stomach and lead to lethal complications. Early diagnosis of the disease according to symptoms is necessary as the surgical method is successful only in the early stages of cancer. After surgical treatment, curative and preventive strategies of radiation and chemotherapy are essential to avoid the recurrence of the malignancy.
