Four Neuroendocrine Tumor Types and the 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

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Abstract

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 non-functioning 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 (23) were significantly associated with larger size, higher proliferative grade, lymphovascular invasion, deep invasion and local lymph node metastasis. Conclusion: Careful analysis of a large series of duodenal NENs identifies five histologically and prognostically different histotypes, of potential clinical relevance.

Original languageEnglish
JournalNeuroendocrinology
DOIs
Publication statusAccepted/In press - Feb 25 2016

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Neuroendocrine Carcinoma
Neuroendocrine Tumors
Gastrinoma
Duodenum
Somatostatin
Duodenal Neoplasms
Paraganglioma
Neoplasm Metastasis
Neoplasms
Lymph Nodes
Histology
Pathology
Survival

ASJC Scopus subject areas

  • Endocrinology
  • Endocrinology, Diabetes and Metabolism
  • Endocrine and Autonomic Systems
  • Cellular and Molecular Neuroscience

Cite this

@article{b456dfe6bc4d4de7be42f25b0e7626c3,
title = "Four Neuroendocrine Tumor Types and the Neuroendocrine Carcinoma of the Duodenum. Analysis of 203 Cases",
abstract = "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 non-functioning 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 (23) were significantly associated with larger size, higher proliferative grade, lymphovascular invasion, deep invasion and local lymph node metastasis. Conclusion: Careful analysis of a large series of duodenal NENs identifies five histologically and prognostically different histotypes, of potential clinical relevance.",
author = "Alessandro Vanoli and {La Rosa}, Stefano and Catherine Klersy and Federica Grillo and Luca Albarello and Frediano Inzani and Roberta Maragliano and Rachele Manca and Ombretta Luinetti and Massimo Milione and Claudio Doglioni and Guido Rindi and Carlo Capella and Enrico Solcia",
year = "2016",
month = "2",
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doi = "10.1159/000444803",
language = "English",
journal = "Neuroendocrinology",
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T1 - Four Neuroendocrine Tumor Types and the Neuroendocrine Carcinoma of the Duodenum. Analysis of 203 Cases

AU - Vanoli, Alessandro

AU - La Rosa, Stefano

AU - Klersy, Catherine

AU - Grillo, Federica

AU - Albarello, Luca

AU - Inzani, Frediano

AU - Maragliano, Roberta

AU - Manca, Rachele

AU - Luinetti, Ombretta

AU - Milione, Massimo

AU - Doglioni, Claudio

AU - Rindi, Guido

AU - Capella, Carlo

AU - Solcia, Enrico

PY - 2016/2/25

Y1 - 2016/2/25

N2 - 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 non-functioning 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 (23) were significantly associated with larger size, higher proliferative grade, lymphovascular invasion, deep invasion and local lymph node metastasis. Conclusion: Careful analysis of a large series of duodenal NENs identifies five histologically and prognostically different histotypes, of potential clinical relevance.

AB - 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 non-functioning 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 (23) were significantly associated with larger size, higher proliferative grade, lymphovascular invasion, deep invasion and local lymph node metastasis. Conclusion: Careful analysis of a large series of duodenal NENs identifies five histologically and prognostically different histotypes, of potential clinical relevance.

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