What is an ampulloma (adenoma of the ampulla of Vater)?

From the Guidelines

Ampuloma should be managed with endoscopic resection or surgical removal, depending on the size and characteristics of the tumor, to prevent potential malignant transformation and complications such as bile flow obstruction, jaundice, or pancreatitis. Ampuloma is a rare tumor that arises in the ampulla of Vater, where the pancreatic and bile ducts empty into the small intestine. These tumors are often adenomas, which are glandular tumors with malignant potential, meaning they can transform into cancer over time 1. Key considerations in managing ampuloma include:

• Size and characteristics of the tumor
• Presence of symptoms such as abdominal pain or jaundice
• Risk of malignant transformation
• Need for regular follow-up with endoscopic surveillance after removal For patients with familial adenomatous polyposis (FAP), there is a high incidence of periampullary cancers, including ampullary carcinomas, and regular duodenoscopy and biopsy of suspicious lesions are recommended, starting when colorectal polyps are diagnosed, with intervals depending on the stage of polyposis 1. In terms of treatment, endoscopic ampullectomy is preferred for smaller lesions, while larger tumors or those with concerning features may require pancreaticoduodenectomy (Whipple procedure). Early detection and treatment are crucial to prevent complications and potential malignant transformation. Patients with stage 4 polyposis should be advised to have surgical resection by pylorus preserving pancreaticoduodenectomy 1.

From the Research

Definition and Diagnosis of Ampuloma

• Ampuloma refers to a tumor originating from the ampulla of Vater, which is the area where the bile and pancreatic ducts empty into the small intestine 2.
• The diagnosis of ampullary tumors can be challenging due to the lack of clear clinical symptoms, and multiple imaging techniques such as abdominal ultrasonography, enhanced computed tomography, magnetic resonance cholangiopancreatography, and endoscopic retrograde cholangiopancreatography may be used to detect ampullary carcinoma 3.
• The European Society of Gastrointestinal Endoscopy (ESGE) recommends endoscopic ultrasound and abdominal magnetic resonance cholangiopancreatography for staging of ampullary tumors 4.

Treatment of Ampuloma

• The treatment of ampullary adenocarcinoma depends on the stage and subtype of the tumor, and may include surgical resection, chemotherapy, and radiation therapy 2, 5, 6.
• The ESGE recommends endoscopic papillectomy in patients with ampullary adenoma without intraductal extension, and suggests considering surgical treatment when endoscopic resection is not feasible 4.
• Adjuvant therapy may be considered in resected disease, and therapy selection should take tumor biology, stage, resection margins, as well as patient comorbidities and performance status into account 6.

Subtypes of Ampuloma

• Ampullary adenocarcinomas can be classified into different subtypes, including intestinal and pancreatobiliary subtypes, which have different prognoses and treatment approaches 5, 6.
• The intestinal subtype has a better prognosis than the pancreatobiliary subtype, and treatment approaches may differ depending on the subtype 6.

Molecular Testing and Targeted Therapy

• Molecular testing using next-generation sequencing and testing for microsatellite instability (MSI) should be performed on all tumors, as this can impact treatment decisions and provide further insights into the biology of the disease 6.
• Targeted therapies such as pembrolizumab and larotrectinib may be used in patients with specific molecular alterations, and referral to genetics counselors and germline testing is warranted in a significant proportion of patients with ampullary tumors 6.