Abstract Library

#985 Periampulary and Pancreatic Neuroendocrine Neoplasms with Duodenal Stromal Gastrointestinal Tumors in Patients with Type 1 Neurofibromatosis: Two Case Reports

Introduction: We describe two cases of neuroendocrine tumors (NETs) in patients with neurofibromatosis type 1 (NF1) associated with gastrointestinal stromal tumors (GISTs).

Conference: 11th Annual ENETSConcerence (2014)

Presenting Author: Zerbi A

Authors: Ridolfi C, Gavazzi F, Spaggiari P, Carnaghi C, Lania A,

Keywords: pancreatic and ampullary neuroendocrine tumor, neurofibromatosis-1, GIST,

#701 High Expression of Mammalian Target of Rapamycin (mTOR) in NF1-Related Periampullary Neuroendocrine Tumors

Introduction: Neurofibromatosis type 1 (NF1) is a rare inherited disorder associated with cutaneous lesions and neoplasms in the nervous and gastroenteropancreatic systems, including periampullary NETs. NF1-coded protein, neurofibromin, is a tumor suppressor that negatively regulates the mammalian target of rapamycin (mTOR). Preclinical in vitro and in vivo studies demonstrated a constitutive activation of mTOR pathway associated with significant antineoplastic effects of mTOR inhibitors in NF1-related glial tumor models.

Conference: 10th Annual ENETSConcerence (2013)

Presenting Author: Razzore P

Authors: Razzore P, Volante M, Russolillo N, Motta M, Lacidogna G,

Keywords: NF1, periampullar NETs, mTOR ,

#525 Pancreatic Neuroendocrine Tumors Associated with Mesenchimal Neoplasms of the GI Tract

Introduction: Neuroendocrine pancreatic tumors (NPT) are occasionally associated with mesenchimal tumors in neurofibromatosis type 1 but rarely in sporadic cases. We report five cases of NPT associated with other rare mesenchimal neoplasms of the GI tract, observed in our Department from 2003 to 2011.

Conference: 9th Annual ENETSConcerence (2012)

Presenting Author:

Authors: Moletta L, Milanetto A, Alaggio R, Sperti C, Pedrazzoli S,

Keywords: pancreatic neuroendocrine tumors, GIST, pancreatic resection,

#279 Coexistence of a Somatostatin-Producing Carcinoma of Duodenum and a Jejuna Gastrointestinal Stromal Tumor (GIST) in a Patient with Von Recklinghausen’s Disease

Introduction: Duodenal neuroendocrine tumors comprise 2-3% of all neuroendocrine tumors of the gastrointestinal tract. Coexistence of NF-1, NET and GIST is rare and only five cases have been described in the literature.

Conference: 8th Annual ENETSConcerence (2011)

Presenting Author:

Authors: Daraki V, Stamou K, ?amalaki E, Spyrou F, Kalikakis G,

Keywords: somatostatinoma, Neurofibromatosis Type 1 , GIST, ,

#122 Molecular genetic findings in gastric carcinoid suggest possible neurofibromin/Ras pathway involvement in carcinoid pathogenesis

Introduction: The molecular pathogenesis of gastrointestinal carcinoid tumours is poorly understood. We have been undertaking molecular genetic investigations of a young woman with neurofibromatosis type 1 (NF1) in whom a gastric carcinoid tumor was detected following an episode of gastrointestinal bleeding. She was otherwise relatively mildly affected by NF1. In NF1, gastric carcinoids have been found previously only in patients with other predisposing factors, which were absent in this patient.

Conference: 7th Annual ENETSConcerence (2010)

Presenting Author: Tobias E

Authors: Tobias E, Fletcher A, Cooke A, Traynor J, Onen N,

Keywords: gastric carcinoid, neurofibromatosis, NF1, LOH, genetic, Ras,