10 Miscellaneous Non-Pancreatic Non-Endocrine Tumors

Non-endocrine, non-pancreatic periampullary tumors are generally classified as arising from the ampulla of Vater, distal common bile duct or duodenum. The most common clinical finding on presentation is obstructive jaundice. These lesions may occur spontaneously or as part of a hereditary syndrome (familial adenomatous polyposis, Gardner’s syndrome, inflammatory bowel disease). The most effective diagnostic strategies for determining extent of disease and resectability of periampullary tumors include dual-phase computed tomography and endoscopic ultrasound. Small, benign periampullary lesions may be amenable to endoscopic resection. For benign lesions 3 cm or suspicion of invasive carcinoma should undergo a pancreaticoduodenectomy. Five-year survival for duodenal, ampullary, and distal common bile duct carcinomas are 51–59, 37–39 and 23–27%, respectively. For each of these tumors, both lymph node status and negative margins are significant predictors of outcome. At this point, neoadjuvant and adjuvant therapies have not clearly demonstrated a survival benefit for non-pancreatic periampullary cancers. The future success in treating these cancers likely rests in the development of novel biological and targeted therapies in the setting of well designed multi-institutional clinical trials. This chapter will focus on benign and malignant nonpancreatic and non-neuroendocrine periampullary tumors and will include the pathology, clinical presentation, diagnostic work-up, and management strategies to approach these neoplasms.