title: Ampulla of Vater carcinoma in real-world clinical practice: a case series.
abstract: OBJECTIVE
  The aim of this report was to describe the way in which a rare and niche disease like ampulla of Vater carcinoma (AVC) was treated in real-world clinical practice.
  
  
  METHODS
  A retrospective analysis of consecutive patients with a diagnosis of AVC treated at our medical oncology unit between August 2004 and August 2013 was performed.
  
  
  RESULTS
  We evaluated 8 consecutive patients with a median age of 60 years (range 56-84). At the last follow-up, 4 patients were alive without evidence of disease and 4 patients had died. The median follow-up time was 21.51 months (range 1-100.43), the median overall survival 23.19 months (range 7.07-102.2), and the median disease-free survival 18.26 months (range 0-102.2). Six patients underwent surgery, which consisted of pylorus-preserving pancreaticoduodenectomy, R0 in all cases. Tumor histology was adenocarcinoma in all patients. Two patients presented with locally advanced disease. Only 1 patient presented with metastases while 3 patients subsequently developed metastases. Two patients received chemotherapy for metastatic disease; in both cases disease progression was observed at the first disease evaluation.
  
  
  CONCLUSIONS
  We can consider AVC as a pathology niche and pancreaticoduodenectomy as the effective treatment for these patients.
authors.affiliation: Department of Oncology, Mater Salutis Hospital, ASL 21 della Regione Veneto, Legnago (Verona) - Italy.
authors.forename: Jacopo
authors.initials: J
authors.lastname: Giuliani
authors.forename: Paolo
authors.initials: P
authors.lastname: Piacentini
authors.forename: Andrea
authors.initials: A
authors.lastname: Bonetti
mesh_terms: D000328:Adult; D000368:Aged; D014670:Ampulla of Vater; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use*; D002166:Camptothecin / Q000008:administration & dosage / Q000031:analogs & derivatives; D000069287:Capecitabine; D002277:Carcinoma / Q000175:diagnosis* / Q000628:therapy*; D003138:Common Bile Duct Neoplasms / Q000175:diagnosis* / Q000628:therapy*; D003841:Deoxycytidine / Q000008:administration & dosage / Q000031:analogs & derivatives; D018572:Disease-Free Survival; D005260:Female; D005472:Fluorouracil / Q000008:administration & dosage / Q000031:analogs & derivatives; D005500:Follow-Up Studies; D006801:Humans; D000077146:Irinotecan; D053208:Kaplan-Meier Estimate; D002955:Leucovorin / Q000008:administration & dosage; D008297:Male; D008875:Middle Aged; D020360:Neoadjuvant Therapy / Q000379:methods*; D059351:Organ Sparing Treatments; D009944:Organoplatinum Compounds / Q000008:administration & dosage; D016577:Pancreaticoduodenectomy; D011708:Pylorus; D012189:Retrospective Studies; D016896:Treatment Outcome; D000093542:Gemcitabine
chemical_list: D009944:Organoplatinum Compounds; D003841:Deoxycytidine; D000069287:Capecitabine; D000077146:Irinotecan; D002955:Leucovorin; D005472:Fluorouracil; D002166:Camptothecin; D000093542:Gemcitabine