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Somatostatin-producing neuroendocrine tumors of the duodenum and pancreas: incidence, types, biological behavior, association with inherited syndromes, and functional activity.
Endocr Relat Cancer. 2008 Mar; 15(1):229-41.ER

Abstract

Somatostatin-producing neuroendocrine tumors (SOM-NETs) of the duodenum and pancreas appear to be heterogeneous. To determine their clinicopathological profiles, respective data were analyzed on a series of 82 duodenal and 541 pancreatic NETs. In addition, the clinical records of 821 patients with duodenal or pancreatic NETs were reviewed for evidence of a somatostatinoma syndrome. Predominant or exclusive expression of somatostatin was found in 21 (26%) duodenal and 21 (4%) pancreatic NETs. They were classified as sporadic (n=31) or neurofibromatosis type 1 (NF1)-associated duodenal NETs (n=3), gangliocytic paragangliomas (GCPGs; n=6), or poorly differentiated neuroendocrine carcinomas (pdNECs; n=2). In addition, five duodenal and four pancreatic SOM-NETs were found in five patients with multiple endocrine neoplasia type 1 (MEN1). Metastases occurred in 13 (43%) patients with sporadic or NF1-associated SOM-NETs, but in none of the duodenal or pancreatic MEN1-associated SOM-NETs or GCPGs. Sporadic advanced (stage IV) SOM-NETs were more commonly detected in the pancreas than in the duodenum. None of the patients (including the 821 patients for whom only the clinical records were reviewed) fulfilled the criteria of a somatostatinoma syndrome. Our data show that somatostatin expression is not only seen in sporadic NETs but may also occur in GCPGs, pdNECs, and hereditary NETs. Surgical treatment is effective in most duodenal and many pancreatic SOM-NETs. MEN1-associated SOM-NETs and GCPGs follow a benign course, while somatostatin-producing pdNECs are aggressive neoplasms. The occurrence of the so-called somatostatinoma syndrome appears to be extremely uncommon.

Authors+Show Affiliations

Department of Pathology, University of Kiel, Michaelisstr 11, Kiel, Germany. ngarbrecht@path.uni-kiel.deNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Multicenter Study
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

18310290

Citation

Garbrecht, Nele, et al. "Somatostatin-producing Neuroendocrine Tumors of the Duodenum and Pancreas: Incidence, Types, Biological Behavior, Association With Inherited Syndromes, and Functional Activity." Endocrine-related Cancer, vol. 15, no. 1, 2008, pp. 229-41.
Garbrecht N, Anlauf M, Schmitt A, et al. Somatostatin-producing neuroendocrine tumors of the duodenum and pancreas: incidence, types, biological behavior, association with inherited syndromes, and functional activity. Endocr Relat Cancer. 2008;15(1):229-41.
Garbrecht, N., Anlauf, M., Schmitt, A., Henopp, T., Sipos, B., Raffel, A., Eisenberger, C. F., Knoefel, W. T., Pavel, M., Fottner, C., Musholt, T. J., Rinke, A., Arnold, R., Berndt, U., Plöckinger, U., Wiedenmann, B., Moch, H., Heitz, P. U., Komminoth, P., ... Klöppel, G. (2008). Somatostatin-producing neuroendocrine tumors of the duodenum and pancreas: incidence, types, biological behavior, association with inherited syndromes, and functional activity. Endocrine-related Cancer, 15(1), 229-41. https://doi.org/10.1677/ERC-07-0157
Garbrecht N, et al. Somatostatin-producing Neuroendocrine Tumors of the Duodenum and Pancreas: Incidence, Types, Biological Behavior, Association With Inherited Syndromes, and Functional Activity. Endocr Relat Cancer. 2008;15(1):229-41. PubMed PMID: 18310290.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Somatostatin-producing neuroendocrine tumors of the duodenum and pancreas: incidence, types, biological behavior, association with inherited syndromes, and functional activity. AU - Garbrecht,Nele, AU - Anlauf,Martin, AU - Schmitt,Anja, AU - Henopp,Tobias, AU - Sipos,Bence, AU - Raffel,Andreas, AU - Eisenberger,Claus F, AU - Knoefel,Wolfram T, AU - Pavel,Marianne, AU - Fottner,Christian, AU - Musholt,Thomas J, AU - Rinke,Anja, AU - Arnold,Rudolf, AU - Berndt,Uta, AU - Plöckinger,Ursula, AU - Wiedenmann,Bertram, AU - Moch,Holger, AU - Heitz,Philipp U, AU - Komminoth,Paul, AU - Perren,Aurel, AU - Klöppel,Günter, PY - 2008/3/4/pubmed PY - 2008/5/9/medline PY - 2008/3/4/entrez SP - 229 EP - 41 JF - Endocrine-related cancer JO - Endocr Relat Cancer VL - 15 IS - 1 N2 - Somatostatin-producing neuroendocrine tumors (SOM-NETs) of the duodenum and pancreas appear to be heterogeneous. To determine their clinicopathological profiles, respective data were analyzed on a series of 82 duodenal and 541 pancreatic NETs. In addition, the clinical records of 821 patients with duodenal or pancreatic NETs were reviewed for evidence of a somatostatinoma syndrome. Predominant or exclusive expression of somatostatin was found in 21 (26%) duodenal and 21 (4%) pancreatic NETs. They were classified as sporadic (n=31) or neurofibromatosis type 1 (NF1)-associated duodenal NETs (n=3), gangliocytic paragangliomas (GCPGs; n=6), or poorly differentiated neuroendocrine carcinomas (pdNECs; n=2). In addition, five duodenal and four pancreatic SOM-NETs were found in five patients with multiple endocrine neoplasia type 1 (MEN1). Metastases occurred in 13 (43%) patients with sporadic or NF1-associated SOM-NETs, but in none of the duodenal or pancreatic MEN1-associated SOM-NETs or GCPGs. Sporadic advanced (stage IV) SOM-NETs were more commonly detected in the pancreas than in the duodenum. None of the patients (including the 821 patients for whom only the clinical records were reviewed) fulfilled the criteria of a somatostatinoma syndrome. Our data show that somatostatin expression is not only seen in sporadic NETs but may also occur in GCPGs, pdNECs, and hereditary NETs. Surgical treatment is effective in most duodenal and many pancreatic SOM-NETs. MEN1-associated SOM-NETs and GCPGs follow a benign course, while somatostatin-producing pdNECs are aggressive neoplasms. The occurrence of the so-called somatostatinoma syndrome appears to be extremely uncommon. SN - 1351-0088 UR - https://www.unboundmedicine.com/medline/citation/18310290/Somatostatin_producing_neuroendocrine_tumors_of_the_duodenum_and_pancreas:_incidence_types_biological_behavior_association_with_inherited_syndromes_and_functional_activity_ L2 - https://erc.bioscientifica.com/doi/10.1677/ERC-07-0157 DB - PRIME DP - Unbound Medicine ER -