Four Neuroendocrine Tumor Types and Neuroendocrine Carcinoma of the Duodenum: Analysis of 203 Cases

Alessandro Vanoli, Stefano La Rosa, Catherine Klersy, Federica Grillo, Luca Albarello, Frediano Inzani, Roberta Maragliano, Rachele Manca, Ombretta Luinetti, Massimo Milione, Claudio Doglioni, Guido Rindi, Carlo Capella, Enrico Solcia

Risultato della ricerca: Contributo in rivistaArticolo in rivista

44 Citazioni (Scopus)


Background: Several types of neuroendocrine neoplasms (NENs) have been described in the duodenal tract, from low-grade tumors (NETs) to high-grade neuroendocrine carcinomas (NECs). A comprehensive analysis of histology, hormonal profile and prognostic parameters of a sufficiently large duodenal NEN series to cover all main kinds of neoplasms is however lacking. Methods: We collected a retrospective series of 203 duodenal wall and ampullary region NENs, from six specialized endocrine pathology centers. All were characterized histopathologically and histochemically, and 190 were followed for a median of 9 years. Results: Twenty-seven poorly differentiated NECs, mostly from the ampullary region, were identified and shown to lead to patient demise in a median of 10 months. Among 176 NETs, four subtypes were characterized, including 20 gastrinomas, 37 ampullary-type somatostatin-producing NETs (ASTs), 12 gangliocytic paragangliomas (GPs) and 106 nonfunctioning NETs (nfNETs). ASTs and GPs were mostly localized in the ampullary/periampullary region, while gastrinomas and nfNETs were mainly from the proximal duodenum. ASTs and gastrinomas showed high rates of local infiltration (especially lymphoinvasion and deep duodenal wall/pancreatic tissue invasion) and lymph node metastasis, while nfNETs had significantly lower and more size-dependent local invasive potential. Disease-specific survival differed significantly between NETs and NECs, though not among NET subtypes. NET cases with distant metastases (n = 23) were significantly associated with larger size, higher proliferative grade, lymphovascular invasion, deep invasion and local lymph node metastasis. Conclusion: Our careful analysis of a large series of duodenal NENs identified five histologically and prognostically different histotypes of potential clinical relevance.
Lingua originaleEnglish
pagine (da-a)112-125
Numero di pagine14
Stato di pubblicazionePubblicato - 2016


  • Ampulla
  • Cellular and Molecular Neuroscience
  • Disease-specific survival
  • Duodenum
  • Endocrine and Autonomic Systems
  • Endocrinology
  • Endocrinology, Diabetes and Metabolism
  • Histotypes
  • Hormones
  • Neuroendocrine neoplasms


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