Neuroendocrine Tumors of the Ampulla of Vater: Presentation, Pathology and Prognosis

Context 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. Design A retrospective review of patients treated at a tertiary care institute was performed over a six-year period from 2005 to 2010. Patients Cases with periampullary cancers were investigated. Main outcome measures 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 (highgrade), 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.


Atul Sachdev, Mayank Jayant, Rajpal Punia, Robin Kaushik, Rajeev Sharma, Nikhil K Nadkarni, Ashok Attri

Abstract | Full-Text | PDF

Share this  Facebook  Twitter  LinkedIn  Google+
30+ Million Readerbase
Recommended Conferences
Flyer image
Abstracted/Indexed in
  • Index Copernicus
  • Academic Journals Database
  • Genamics JournalSeek
  • Academic Keys
  • China National Knowledge Infrastructure (CNKI)
  • CiteFactor
  • Scimago
  • British Library
  • Electronic Journals Library
  • Directory of Research Journal Indexing (DRJI)
  • WorldCat
  • Emerging Sources Citation Index (ESCI)
  • EBSCO Host
  • MIAR
  • International Committee of Medical Journal Editors (ICMJE)
  • University of Zurich - UZH
  • University Grants Commission
  • SWB Online-Katalog
  • Scholarsteer
  • Geneva Foundation for Medical Education and Research
  • Secret Search Engine Labs