title: Neuroendocrine tumors of the ampulla of vater: presentation, pathology and prognosis.
abstract: BACKGROUND
  Neuroendocrine tumors of the pancreatic ampulla are uncommon. The final diagnosis is based on histology, and at times, it may be difficult to diagnose them pre-operatively since they present with a similar clinical picture to adenocarcinomas of this region.
  
  
  OBJECTIVE
  To identify neuroendocrine tumors of the ampulla, as well as their presentation and management.
  
  
  METHODS
  A retrospective review of patients treated at a tertiary care institute was performed over a six-year period from 2005 to 2010.
  
  
  METHODS
  Cases with periampullary cancers were investigated.
  
  
  METHODS
  The case records were scrutinised for the clinical presentation, management and outcomes.
  
  
  RESULTS
  A total of 4 cases (7.7%) of neuroendocrine tumors of the ampulla were identified from 52 patients with periampullary lesions, at a mean age of presentation of 49 years. The common mode of presentation was progressive jaundice (3 of 4 patients); pancreaticoduodenectomy was performed in 3 patients. One patient underwent palliative endoscopic stenting for metastatic disease. On histopathology, 2 of the patients had poorly differentiated (neuro)endocrine carcinoma (high grade), and 2 had well differentiated (neuro)endocrine carcinoma (1 low grade and 1 intermediate). All the tumors stained positively with chromogranin A. The patients who underwent pancreaticoduodenectomy are on regular follow-up and remain free of disease.
  
  
  CONCLUSIONS
  Neuroendocrine tumors of the ampulla are distinct entities presenting clinically with jaundice. They stain positive with chromogranin A on histopathology. Pancreaticoduodenectomy should be performed as it is associated with good outcome.
authors.affiliation: Department of Surgery, Government Medical College and Hospital, Chandigarh, India. mayankjayant@rediffmail.com
authors.forename: Mayank
authors.initials: M
authors.lastname: Jayant
authors.forename: Rajpal
authors.initials: R
authors.lastname: Punia
authors.forename: Robin
authors.initials: R
authors.lastname: Kaushik
authors.forename: Rajeev
authors.initials: R
authors.lastname: Sharma
authors.forename: Atul
authors.initials: A
authors.lastname: Sachdev
authors.forename: Nikhil K
authors.initials: NK
authors.lastname: Nadkarni
authors.forename: Ashok
authors.initials: A
authors.lastname: Attri
mesh_terms: D000328:Adult; D000368:Aged; D014670:Ampulla of Vater / Q000378:metabolism / Q000473:pathology*; D053379:Chromogranin A / Q000378:metabolism; D003138:Common Bile Duct Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000473:pathology*; D005260:Female; D006801:Humans; D007565:Jaundice / Q000175:diagnosis* / Q000209:etiology; D008297:Male; D008875:Middle Aged; D018358:Neuroendocrine Tumors / Q000150:complications / Q000175:diagnosis* / Q000473:pathology*; D016577:Pancreaticoduodenectomy; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D053379:Chromogranin A