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Ampullary cancer

Definition
Ampullary cancer is a rare malignancy that originates in the ampulla of Vater.
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Pathophysiology
The pathophysiology of ampullary cancer is complex and involves a variety of molecular alterations. The most common subtype is adenocarcinoma, which can have either intestinal or pancreatobiliary differentiation. Genomic sequencing has identified several driver genes, including ELF3, TP53, KRAS, and APC, that play a role in the development of this cancer.
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Epidemiology
The incidence of ampullary cancer in the Unites States is estimated at 0.59 per 100,000 person-years.
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Disease course
Clinically, patients with ampullary cancer often present with symptoms such as jaundice, abdominal pain, weight loss, and pale stools. In some cases, the disease may be asymptomatic and detected incidentally during routine medical examinations.
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Prognosis and risk of recurrence
The prognosis of ampullary cancer varies widely and is influenced by factors such as disease stage at diagnosis, histopathological subtype, and the presence of lymph node metastases. Early detection and intervention can significantly improve survival rates.
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Key sources
The following summarized guidelines for the evaluation and management of ampullary cancer are prepared by our editorial team based on guidelines from the European Society of Gastrointestinal Endoscopy (ESGE 2021) and the American Society for Gastrointestinal Endoscopy (ASGE 2021; 2013).
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Guidelines

1.Classification and risk stratification

Staging
Obtain EUS and abdominal MRCP for the staging of ampullary tumors.
B
Consider using the latest TNM classification for the staging of ampullary tumors.
C
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2.Diagnostic procedures

Upper gastrointestinal endoscopy: consider using the cap-assisted method when the papilla is not seen during forward-viewing endoscopy.
C
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  • EUS

  • Intraductal ultrasound

  • Biopsy and histopathology

  • Evaluation after initial negative histopathology

3.Therapeutic procedures

Endoscopic biliary stenting: perform retrograde cholangiopancreatography with self-expandable metal stent insertion in patients with ampullary tumors and biliary obstruction in palliative settings.
A
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  • Ablative techniques

4.Surgical interventions

Endoscopic papillectomy, indications: do not perform diagnostic/therapeutic papillectomy when adenoma has not been proven.
D
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  • Endoscopic papillectomy (technical considerations)

  • Endoscopic papillectomy (prevention and management of postprocedural complications)

  • Surgical papillectomy

  • Pancreaticoduodenectomy

5.Specific circumstances

Patients with gastric outlet obstruction: consider placing self-expandable metallic stents or performing surgical gastrojejunostomy in patients with incurable malignant gastric outlet obstruction undergoing palliative intervention. Decide between these approaches based on patient characteristics and preferences, multidisciplinary input, and local expertise.
C
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6.Follow-up and surveillance

Postoperative surveillance: obtain long-term monitoring of patients after endoscopic papillectomy or surgical ampullectomy, based on duodenoscopy with biopsies of the scar and of any abnormal area, within the first 3 months, at 6 and 12 months, and thereafter yearly for at least 5 years.
B

More topics in this section

  • Management of recurrent disease