Ampullary Adenocarcinoma: A Rare Malignancy

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Ampullary Adenocarcinoma: A Rare Malignancy
Ampullary Adenocarcinoma: A Rare Malignancy

Ampullary adenocarcinoma is a relatively rare cancer, representing approximately 0.2% of all gastrointestinal (GI) cancers and 6% of periampullary tumors.1,2 The periampullary region is made up of those organs located in the vicinity (within 2 cm) of the ampulla of Vater, which includes the pancreas, distal common bile duct, duodenum, and the ampulla of Vater itself. Ampullary adenocarcinoma is classified in two subtypes: intestinal-type (better prognosis) and pancreatobiliary (worse prognosis).1,2  

RELATED: Gastrointestinal Cancers Resource Center

Patients with ampullary adenocarcinoma typically present with jaundice, weight loss, signs and symptoms of chronic GI bleeding, abdominal pain, and nausea/vomiting in patients who are between age 60 and 70 years.1,2 Many of the patient's symptoms are a direct result of the location of the tumor at the ampulla of Vater. Initial imaging may include abdominal ultrasound, CT scan of the abdomen and pelvis, and/or MRI/magnetic resonance cholangiopancreatography of the abdomen. To further investigate an ampullary mass seen on imaging, endoscopic techniques such as endoscopic ultrasound and endoscopic retrograde cholangiopancreatography can be used in order to obtain a tissue sample for diagnosis. Many patients will have elevated serum aminotransferases consistent with a cholestatic pattern, along with elevated carcinoembryonic antigen and CA19.9 levels; however, no laboratory finding is diagnostic of ampullary adenocarcinoma. A recent study by Okano and colleagues identified several poor prognostic indicators in patients with ampullary adenocarcinoma, including pancreatobiliary subtype, preoperative biliary drainage, elevated CA19.9 and total bilirubin, evidence of nodal metastasis, pathological grade, and regional tissue invasion.2 

The first-line treatment of ampullary adenocarcinoma is typically resection, including pancreaticoduodenectomy (Whipple procedure), pylorus-preserving pancreaticoduodenectomy (PPPD), and local resection.1 The Whipple procedure or PPPD is typically favored over location resection in patients who are good surgical candidates secondary to the decreased rates of recurrence and better survival rates.1 Patients who are not good surgical candidates or who present with benign ampullary tumors can potentially undergo endoscopic resection.

Unfortunately, there is a paucity of data regarding the role of adjuvant chemotherapy and radiation therapy in patients with ampullary adenocarcinoma. Therefore, there are no distinct guidelines outlining the best protocol for which to treat these patients. The most commonly used chemotherapy agents used after surgery are 5-fluorouracil, gemcitabine, and cisplatin.1 Again, there are no standardized surveillance protocols for these patients, but frequent office visits that include histories and physicals, monitoring of tumor markers, and repeat endoscopy and cross-sectional imaging have all been used in managing ampullary adenocarcinoma.


  1. Romiti A, Barucca V, Zullo A, et al. Tumors of ampulla of Vater: a case series and review of chemotherapy options. World J Gastrointest Oncol. 2012;4(3):60-67.
  2. Okano K, Oshima M, Yachida S, et al. Factors predicting survival and pathological subtype in patients with ampullary adenocarcinoma. J Surg Oncol. 2014 Mar 12. doi: 10.1002/jso.23600. [Epub ahead of print]

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