Periampullary adenocarcinoma (PAC) is defined as tumors arising ≤2 cm from the ampulla of Vater and can originate from the pancreatic head, duodenum, distal bile duct, or ampulla of Vater. All four subtypes of PAC typically present with obstructive jaundice but have variable survival rates depending on site and specific histology. Pancreas protocol computed tomography (CT) scan is the most useful imaging modality for PACs, and surgical resection offers the best chance of cure. Proceeding to surgery based on pancreas protocol CT findings alone is acceptable, but certain clinical scenarios may require further preoperative workup with biliary drainage procedures or tissue diagnosis. The standard procedure for PAC is pancreaticoduodenectomy (PD) with the goal of complete tumor resection with negative oncologic margins. Clinicopathologic staging is based on the American Joint Committee on Cancer (AJCC) Cancer Staging Manual for each location of PAC origination. Key factors in staging and adjuvant treatment are resection margins, nodal involvement, microvascular invasion, and perineural invasion. Features important for chemotherapy and immunotherapy include histomolecular markers such as key results areas (KRAS) and ductal pancreatic adenocarcinoma (DPAC) that offer opportunity for targeted therapy. Multidisciplinary oncologic care for PAC is essential along the continuum of treatment.
KeywordsPeriampullary carcinoma Ampullary carcinoma Distal bile duct carcinoma Duodenal adenocarcinoma Pancreatic ductal adenocarcinoma Pancreas protocol computed tomography scan Pancreaticoduodenectomy Biliary drainage procedure Neoadjuvant therapy Adjuvant therapy
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