Table of ContentsDefinition / general | Essential features | ICD coding | Epidemiology | Clinical features | Diagnosis | Case reports | Treatment | Clinical images | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Cytology images | Molecular / cytogenetics description | Differential diagnosis | Board review style question #1 | Board review answer #1 | Board review style question #2 | Board review answer #2
Cite this page: Gonzalez RS. Adenoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/ampullaadenoma.html. Accessed August 3rd, 2020.
Definition / general
- Dysplastic, premalignant lesion of ampulla of Vater
- ICD-10: D37.6 - Neoplasm of uncertain behavior of ampulla of Vater
- Endoscopic impression, confirmed on biopsy
- 49 year old woman with intussusception (Case Rep Gastroenterol 2016;10:545)
- 53 year old man with common bile duct stricture (Cytojournal 2017;14:19)
- 74 year old man with concurrent ampullary small cell neuroendocrine carcinoma (World J Gastroenterol 2008;14:4709)
- 78 year old man with ampullary adenoma (J Med Case Rep 2014;8:228)
- 81 year old man with progression to carcinoma (Am J Case Rep 2015;16:586)
- Early or relatively confined lesions may be excised by endoscopic polypectomy or ampullectomy (Am J Clin Pathol 2009;132:506)
- Otherwise, requires pancreatoduodenectomy
- Polypoid / exophytic mass
Microscopic (histologic) description
- Tubular, villous or mixed, similar to adenomas in colon, with approximately half tubular and half villous (Am J Clin Pathol 2009;132:506)
- Dysplastic epithelium may have only subtle changes of mild cellular stratification and fine chromatin pattern
- Often contain prominent Paneth cells (with coarse, large, red-pink, refractile granules in supranuclear cytoplasm), endocrine cells (dark, red-purple, fine small granules in basal cytoplasm) and goblet cells
- May show high grade dysplasia or give rise to adenocarcinoma
Microscopic (histologic) images
- Endoscopic brush cytology is sensitive and specific for adenoma / carcinoma, although diagnosis of adenoma does not exclude coexisting carcinoma (Am J Clin Pathol 1998;109:540)
Molecular / cytogenetics description
Board review style question #1
Which of the following is true about adenomas of the ampulla of Vater?
- BRAF mutations are rarely seen
- Cases must be managed with pancreatoduodenectomy
- Patients with Lynch syndrome are at significantly increased risk
- Progression to adenocarcinoma is unusual
Board review answer #1
A. BRAF mutations are rarely seen. KRAS and APC mutations are more common, both in sporadic cases and in those from patients with familial adenomatous polyposis.
Board review style question #2
Board review answer #2
B. KRAS mutations are most commonly seen in ampullary adenomas, along with APC mutations.