title: Neuroendocrine tumours of the duodenum. Clinical aspects, pathomorphology and therapy.
abstract: OBJECTIVE
  Neuroendocrine tumours (NTs) of the duodenum are uncommon neoplasms. They represent a heterogeneous spectrum of subtypes and may be associated with von Recklinghausen's disease type I (VRD) and multiple endocrine neoplasia type I. There are few studies concerning the biological characteristics and adequate therapy of these tumours.
  
  
  METHODS
  We report on a retrospective analysis of 12 patients with NTs of the duodenum: six non-ampullary (naNTs) and six ampullary tumours (aNTs). These patients were treated between January 1992 and January 2001. Clinical and histopathological features, therapy and follow-up were evaluated retrospectively and compared with the literature.
  
  
  RESULTS
  All tumours were located in the first and second portions of the duodenum. Three of six aNTs presented with jaundice, and four of six naNTs were incidental findings. Two patients with naNTs showed Zollinger-Ellison syndrome and two with aNTs VRD. Of the six patients with naNTs, four were treated by local excision (two endoscopically, two surgical resection), one by Kausch-Whipple operation and in one patient the tumour was an incidental finding in the Billroth II specimen. Four of the six patients with aNTs underwent Kausch-Whipple procedure, one patient ampullectomy (gangliocytic paraganglioma) and one patient palliative chemotherapy. The size of the naNTs were less than 0.6 cm, whereas the size of the aNTs ranged from 1.5 cm to 4 cm. Tumour size of aNTs had no correlation with depth of invasion and metastases. Metastases were found in two aNTs and none of the naNTs. Immunohistochemically tumour cells expressed somatostatin in 5 of 6 aNTs and gastrin in 1 of 6 aNTs and in two gastrinomas. There was no hospital mortality. Two patients died for reasons not related to the tumour. Tumour excision of both patients with gastrinomas was not curative. Three patients with naNTs and four with aNTs are alive without disease. One patient with palliative treatment of a metastasising aNT is alive 66 months after diagnosis.
  
  
  CONCLUSIONS
  Non-ampullary duodenal NTs differ clinically, histologically and immunohistochemically as well as with respect to the extent of resection from NTs of the ampulla of Vater.
authors.affiliation: Department of Abdominal, Transplantation and Vascular Surgery, University of Leipzig, Liebigstrasse 20a, 04103 Leipzig, Germany. with@medizin.uni-leipzig.de
authors.forename: Helmut
authors.initials: H
authors.lastname: Witzigmann
authors.forename: Clemens
authors.initials: C
authors.lastname: Loracher
authors.forename: Felix
authors.initials: F
authors.lastname: Geissler
authors.forename: Theodor
authors.initials: T
authors.lastname: Wagner
authors.forename: Andrea
authors.initials: A
authors.lastname: Tannapfel
authors.forename: Dirk
authors.initials: D
authors.lastname: Uhlmann
authors.forename: Karel
authors.initials: K
authors.lastname: Caca
authors.forename: Johann
authors.initials: J
authors.lastname: Hauss
authors.forename: John Adolf
authors.initials: JA
authors.lastname: Hehl
mesh_terms: D000328:Adult; D000368:Aged; D014670:Ampulla of Vater / Q000473:pathology / Q000503:physiopathology / Q000601:surgery; D004379:Duodenal Neoplasms / Q000473:pathology* / Q000503:physiopathology* / Q000601:surgery; D004386:Duodenum / Q000473:pathology* / Q000503:physiopathology* / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D018358:Neuroendocrine Tumors / Q000473:pathology* / Q000503:physiopathology* / Q000601:surgery; D012189:Retrospective Studies; D016896:Treatment Outcome