Abstract library

2393 results for "Gastrointestinal Stromal Tumors (GISTs); Neuroendocrine Tumor (NETs); Multiple Endocrine Neoplasia type 1 (MEN-1) syndrome; Gastrinoma".
#18 Long-acting release octreotide induce complete response in type 1 gastric carcinoid tumors
Introduction: Gastric endocrine tumors (GET) are increasingly recognized due to expanding indications of upper gastrointestinal endoscopy. Often silent and benign, GET may also be aggressive when sporadic and may sometimes mimic the course of gastric adenocarcinoma. Current incidence of GETs is estimated at around 8% of digestive endocrine tumors. Yearly age-adjusted incidence is around 0.2 per population of 100,000. Gastric carcinoids (ECLomas) develop from gastric enterochromaffin-like cells (ECL cells) in response to chronically elevated gastrin. Type 1 tumors (ECLomas in the course of atrophic gastritis) may occur in conditions of achlorhydria secondary to auto-immune atrophic fundic gastritis. It occurs mostly in women and they are non-functioning tumors, typically found during upper GI endoscopy performed for dyspepsia. ECLomas present frequently as multiple polyps, usually < 1 cm in diameter in the gastric fundus. Type 1 tumors are almost exclusively benign lesions with little risk of deep invasion of the gastric parietal wall. The neoplastic ECL cells become progressively dedifferentiated with an increasing number of Ki-67 immunoreactive (IR) cell nuclei. In addition, there is a substantial decrease in argynophil and IR NE cells that can be visualized by conventional methods. ECLomas secondary to hypergastrinemia should be closely followed for signs of clinical and histopathological tumor progression. Such ECLomas deserve early, active, radical surgical treatment.
Traditionally, gastric carcinoid type 1 (GCA1s) are endoscopically or surgically removed, depending on the number, appearance and size of the tumors. Antrectomy, with surgical excision of the majority of the G cells, is thought to facilitate regression of these tumors by removing the source of excessive gastrin secretion; however, the long-term benefits of antrectomy still remain uncertain. Although proton pump inhibitors are effective in reducing hypergastrinemia-induced gastric acid hypersecretion in GCA2, they do not affect ECL-cell hyperplasia, and therefore their role in GCA1 is limited. Moreover, in selected cases, significant reduction of hypergastrinemia does not prevent development of ECL carcinoid, suggesting that, in addition to hypergastrinemia, other pathogenic or genetic factors may be involved. Treatment with somatostatin analogues (SSA) might impede ECL-cell hyperplasia by suppressing gastrin secretion and/or by a direct anti-proliferative effect on ECL cells. Treatment with SSAs in GCA1 leads to a substantial tumor load reduction, with a concomitant decrease of serum gastrin levels. Published data indicate an important anti-proliferative effect of SSA on ECL cells, providing clinical benefit and obviating, at least temporarily, the need for invasive therapies for GCA1. Morphometric studies demonstrated that, while antrectomy specifically decreased the volume of ECL cells versus the total volume of endocrine cells, octreotide reduces the overall endocrine cell volume. Although the number of treated patients is small, it has been suggested that SSA may exert important anti-proliferative effects either directly, by inhibiting ECL-cells proliferation, or indirectly through suppression of gastrin hypersecretion.
Conference: 7th Annual ENETS Conference (2010)
Category: Clinical
Presenting Author: MD Ricardo Caponero
#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
#40 Clinical and prognostic implications of the genetic diagnosis of hereditary NET syndromes in asymptomatic patients
Introduction: Neuroendocrine tumors (NETs) are rare neoplasms, heterogeneous, and with variable biological behavior. NETs can be sporadic or they can arise in complex endocrine hereditary syndromes such as Multiple Endocrine Neoplasias (MEN), Familial Paragangliomatosis (FPGL), Neurofibromatosis type 1 (NF1), von Hippel-Lindau Disease (VHL), Tuberous Sclerosis (TSC) and Carney Complex (CC). By performing genetic screening, patients with hereditary NETs can be identified before the development of tumors.
Conference: 7th Annual ENETS Conference (2010)
Category: Clinical
Presenting Author: Antongiulio Faggiano
#58 Surgical approaches in 84 patients with insulinomas in multiple endocrine neoplasia type 1 (MEN 1)
Introduction: Management of insulinomas in the setting of Multiple Endocrine Neoplasia type 1 (MEN1) remains controversial.
Conference: 7th Annual ENETS Conference (2010)
Category: Clinical
Presenting Author: Dr Delphine Vezzosi
#83 Surgical treatment of duodenopancreatic neuroendocrine tumors (pNETs) in patients with multiple endocrine neoplasia type 1 (MEN 1): a Dutch consensus statement
Introduction: Duodenopancreatic neuroendocrine tumors (pNETs) in multiple endocrine neoplasia type 1 (MEN 1) are the most important cause of MEN 1-related death. Surgery is the only curative treatment, but controversy exists on the optimal strategy. Recent guidelines on pNETs have limited recommendations specific for MEN 1. Therefore, a Dutch multidisciplinary consensus meeting was organized.
Conference: 7th Annual ENETS Conference (2010)
Category: Clinical
Presenting Author: MD Carolina RC Pieterman
#157 Multiple Endocrine Neoplasia Type 1 (MEN-1), Hadassah-Hebrew University Medical Center experience
Introduction: MEN 1 is an autosomal dominant genetic disorder with a prevalence of 2-4 per 100,000. The main manifestations are parathyroid (PT), gastroenteropancreatic (GEP) and pituitary tumors, but may affect other organ systems as well. MEN 1 is associated with significant morbidity and mortality, with up to 50% dying before the age of 50. Treating MEN 1 patients presents a unique diagnostic and therapeutic challenge.
Conference: 7th Annual ENETS Conference (2010)
Category: Clinical
Presenting Author: Dr. Sameer Kassem
Authors: Kassem S, Glaser B, Barak D, Fraenkel M, ...
#212 Reduced MEN1 Gene Expression in Pulmonary Carcinoids Is Associated With Metastatic Disease
Introduction: Lung carcinoids are neuroendocrine tumors that can be classified as typical (TC) or atypical (AC) carcinoids. Previously, MEN-1 mutations and associated LOH of 11q13 have been implied in tumorigenesis.
Conference: 8th Annual ENETS Conference (2011)
Category: Basic
Presenting Author: MSc Dorian R.A. Swarts
#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 ENETS Conference (2011)
Category: Clinical
Presenting Author: Dr Vasiliki Daraki
#348 Management of Multiple Endocrine Neoplasia Type 1 in Patients with Pancreatic Neuroendocrine Tumors
Introduction: Screening for MEN-1 is an integral part of examination of patients with pNETs because revelation of this hereditary pathology radically influences tactics of treatment.
Conference:
Category: Basic
Presenting Author: Ivan Vasiliev
#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