Makindo Medical Notes

💡 Ampullary carcinoma is a rare but surgically curable tumour arising from the ampulla of Vater — where the bile duct and pancreatic duct merge into the duodenum. It grows slowly and often presents with painless jaundice. Differentiating it from cholangiocarcinoma or pancreatic cancer is crucial, as it carries a much better prognosis when resected.

📘 About

• Uncommon tumour causing painless obstructive jaundice.
• Originates at the ampulla of Vater — the confluence of the distal bile duct, pancreatic duct, and duodenal mucosa.
• Represents around 0.2% of all gastrointestinal malignancies.
• High potential for cure with surgical resection due to earlier presentation compared with pancreatic cancer.

⚠️ Risk Factors

• Pre-existing adenoma of the ampulla (can undergo malignant transformation).
• Familial adenomatous polyposis (FAP) and Gardner’s syndrome — associated with ampullary adenomas and carcinoma.
• Chronic inflammation of the biliary tract.

🩺 Clinical Features

• 🏵️ Painless jaundice — classic presentation due to bile duct obstruction.
• Pruritus, pale stools, and dark urine.
• Weight loss and malaise.
• Occasional melaena or cholangitis.
• Dull epigastric or right upper quadrant pain may occur.

🧪 Investigations

• 📈 LFTs: elevated ALP and bilirubin (obstructive pattern).
• 🩻 Ultrasound: dilated intrahepatic ducts and common bile duct.
• 💻 CT/MRI: defines local extent and excludes pancreatic invasion.
• 🧬 ERCP: may visualise a mass at the ampulla — allows biopsy ± sphincterotomy to improve diagnostic yield.
• 🔬 Histology: confirms adenocarcinoma subtype (intestinal, pancreaticobiliary, or mixed type).

🧫 Pathology

• Most are adenocarcinomas derived from the intestinal or pancreaticobiliary epithelium.
• Subtyping (intestinal vs pancreaticobiliary) has prognostic value — intestinal type generally has a better outcome.

🔍 Differential Diagnosis

• Pancreatic carcinoma — more aggressive, often with pain and later presentation.
• Distal cholangiocarcinoma — originates from the distal bile duct, typically lacks mucosal lesion at ampulla.
• Duodenal carcinoma — arises from adjacent duodenal mucosa.

🩹 Management

• 🎯 Curative resection (Whipple procedure / pancreaticoduodenectomy) is the treatment of choice.
• Approximately 75% of patients are suitable for surgery due to earlier presentation.
• Five-year survival: up to 60% if resection margins are clear (R0 resection).
• Adjuvant chemotherapy may be considered in high-risk or node-positive disease.
• Palliative options include biliary stenting or bypass in unresectable cases.

📊 Prognosis

• Much better prognosis than pancreatic ductal adenocarcinoma due to earlier detection.
• Median survival after curative surgery: 3–5 years.
• Recurrence typically occurs within 2 years post-resection — follow-up with imaging and CA19-9 monitoring is advised.

📚 References

• Treatment Approach to Adenocarcinoma of the Ampulla of Vater — Springer, 2021
• Albores-Saavedra J et al. Pathologic classification and prognosis of ampullary carcinomas. Cancer. 2009;115(4):557–567.
• UK NICE Guidance: Biliary Tract and Pancreatic Cancer Pathways (NG85, 2018)

💡 Teaching tip: When faced with painless jaundice, always consider ampullary carcinoma — it’s surgically curable, unlike most pancreatic and biliary malignancies. Early imaging and ERCP biopsy can make the crucial diagnostic distinction