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

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.
Aim(s): To present a patient with the coexistence of Neurofibromatosis Type 1 (NF-1), somatostatinoma and stromal tumor of the gastrointestinal tract (GIST).
Materials and methods: A 55-year-old female patient presented with two tumors: one at the ampulla of Vater and a second at proximal jejunum. The presence of cafe-au-lait macules, neurofibromas, axillary freckling (Crowe sign), and Lisch nodules of the iris set the diagnosis of NF-1 in this patient. Therefore she was investigated for a neuroendocrine tumor.
Conference: 8th Annual ENETS Conference (2011)
Category: Clinical
Presenting Author: Dr Vasiliki Daraki

To read results and conclusion, please login ...

Further abstracts you may be interested in

#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 ENETS Conference (2014)
Category: Clinical cases/reports
Presenting Author: Cristina Ridolfi
#37 Gastric GIST with synchronous neuroendocrine tumor of the pancreas, Case Report and Literature Review
Introduction: The Gastrointestinal Stromal Tumors (GIST) are the most common mesenchymal tumors of the gastrointestinal tract. These are rare tumors with an incidence of 15 new cases per million per year. They often occur in individuals over the age of 40 years, without gender predominance. The clinical behavior is variable and benign tumors are the most common. They can develop anywhere in the GI tract, but are more frequent in the stomach and small intestine. The primary treatment, when located, is the surgical resection, which can be complemented with the use of imatinib.
The occurrence of neuroendocrine tumors of the pancreas is rare, representing 1-5% of pancreatic cancers, and it is estimated that its incidence does not exceed 5 to one million. The tumors considered nonfunctioning (15-32% of pancreatic neuroendocrine tumors) are not associated with any syndrome, and are usually incidental. They have a slight predominance in males, and are more frequent in the 6th decade of life. Nonfunctioning tumors of islet cells are virtually all malignant tumors, and the treatment consists of surgical resection.
Conference: 7th Annual ENETS Conference (2010)
Category: Clinical
Presenting Author: Dra Amelia B Tavares
#340 Aggressive Somatostatinoma, with Associated Pheocromocytoma in a Neurofibromatosis Patient
Introduction: Neurofibromatosis is associated with a neuroendocrine phenotype as pheocromocytoma, primary hyperparathyroidism and gastropancreatic neuroendocrine tumors.
Conference: 8th Annual ENETS Conference (2011)
Category: Clinical
Presenting Author: Catalina Poiana
Authors: Poiana C, Carsote M, Radoi V, Ene C, ...
#615 Gastrinoma and Neurofibromatosis Type 2: Is There an Association?
Introduction: Gastroenteropancreatic neuroendocrine tumors have been seldom described in association with neurofibromatosis type-1, whereas an association with neurofibromatosis type-2 (NF2) has never been reported.
Conference: 10th Annual ENETS Conference (2013)
Category: Epidemiology/Natural history/Prognosis - Descriptive epidemiology
Presenting Author: Dr Roberta E Rossi
#349 Gastrointestinal Stromal Tumor (GIST) Of The Esophagus In A Patient With MEN-1 And-Related Pancreatic Gastrinoma
Introduction: Both Multiple Endocrine Neoplasia type 1 (MEN-1; OMIM #613733)-related-gastrinomas and Gastrointestinal Stromal Tumors (GISTs; OMIM #606764) are rare neoplasms and their association has been rarely reported so far.
Conference:
Category: Basic
Presenting Author: Dr Roberta E Rossi