Abstract library

445 results for "paraganglioma syndrome".
#274 An Unexpected Diagnosis
Introduction: Paragangliomas (PGL) are defined as rare tumors arising from extra-adrenal sympathetic and parasympathetic paraganglia and malignant paragangliomas are defined by the presence of metastases.
Conference: 8th Annual ENETS Conference (2011)
Category: Clinical
Presenting Author: Dr.ssa Chiara Martini
#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
#22 A Case Illustrative of Phenotypic Heterogeneity and Challenges in the Management of Paraganglioma
Introduction: Paragangliomas (PGLs) are extra-adrenal, usually benign, highly vascularized tumors that originate from neural-crest-derived chromaffin cells. These tumors are subdivided as either sympathetic or parasympathetic, depending on their location and catecholamine production. Sympathetic PGLs are situated along the abdominal sympathetic trunk and usually produce catecholamines, whereas parasympathetic PGLs are located in the head and neck, and these usually do not produce catecholamines. PGLs may present as sporadic or inherited tumor syndrome, including MEN 2, with RET germline mutations, von Hippel-Lindau (VHL) disease due to germline mutations in VHL gene, and pheochromocytoma-PGL syndrome. The latter is frequently a hereditary condition and is caused by germline mutations in the SDHB, SDHC, or SDHC genes. Patients with familial PGLs may present at a younger age, often as multifocal tumors, with an increased risk of recurrence and a higher frequency of malignancy in those with SDHB mutations. SDH mutations induce angiogenesis and tumorogenesis through the inhibition of hypoxia-inducible factors (HIF)-propyl hyroxylase. A younger age at onset, malignancy, and a positive family history are clinical parameters of high specificity, but low sensitivity for diagnosis. Genetic analysis for mutations in SDH genes for the patient and family members, and surveillance for the affected patient and family members, are necessary where there are no clear clinical or family indicators for the syndrome. We present a case of a large abdominal malignant PGL in a 20-year-old pt. that went on without clinical detection for at least three years.
Conference: 7th Annual ENETS Conference (2010)
Category: Clinical
Presenting Author: Dr Mohammed Ahmed
#2972 Characteristics and Management of Paragangliomas, 10 Years Experience
Introduction: Paraganglioma (PG) is a rare extra-adrenal neuroendocrine tumor, with common sites of presentation being abdomen, and head and neck region. The majority of PGs appear to be sporadic. However, almost half of cases are associated with an inherited syndrome. The highest malignancy rates are seen in paragangliomas (PGs) associated with SDHB mutations, which are usually abdominal and secretory.
Conference: 17th Annual ENETS Conference 2020 (2020)
Category: Epidemiology/Natural history/Prognosis - Registries, nationwide and regional surveys
Presenting Author: MD Emilia Sardo
#1729 Outstanding Response to Sunitinib in a Patient with Unresectable Retroperitoneal Paraganglioma
Introduction: Pheochromocitomas and Paragangliomas (PCs/PGLs) are rare neuroendocrine tumors arising from the adrenal medulla and extra-adrenal ganglia. Around 30-40% of these neoplasms are genetically determined. PCs/PGLs arising from the sympathetic chain can synthesize bioactive amines leading to typical syndromes, in contrast to parasympathetic PGLs that are mainly non-secretory.
Conference: 14th Annual ENETS conference 2017 (2017)
Category: Clinical cases/reports
Presenting Author: MD Sara Pusceddu
#944 Pheochromocytoma and Paraganglioma in Pediatric Age
Introduction: Pheochromocytoma (PHE) and paraganglioma (PGL) are rare tumors of the adrenal medulla and extra-adrenal sympathetic chromaffin tissue. Nearly 30% of them are familiar or associated with genetic syndromes (VHL, MEN2, NF1, SDH mutation, Pacak-Zhuang). Only 20% PHE occur before 20 years of age. Incidence of pediatric PHE is 0.3:1 million.
Conference: 11th Annual ENETS Conference 2014 (2014)
Category: Clinical cases/reports
Presenting Author: MD, PhD Viera - Bajciova
Authors: Bajciova V, Kren L, Vicha A, ...
#1305 PRRT for Malignant Pheochromocytomas and Paragangliomas: The Singapore General Hospital Experience
Introduction: In recent years, the use of 177Lu-DOTATATE radioisotope therapy has been expanded to patients with metastatic pheochromocytomas and paragangliomas (PCC/PGL). Although this is not the standard of care, treatment options are limited for those with MIBG negative disease. Our centre has treated over 40 patients with NETS with PRRT since 2012.
Conference: 13th Annual ENETS conference 2016 (2016)
Category: PRRT-Ablative therapies-Endoscopic treatment
Presenting Author: Dr Lih Ming Loh
#124 Management and outcomes of malignant paragangliomas and phaeochromocytomas
Introduction: Chromaffin-cell tumors include phaeochromocytomas and paragangliomas, represent a rare group of neuroendocrine tumors and are located in adrenal medulla or extra-adrenal sites, respectively. Ten percent of phaeochromocytomas and 15-35% of paragangliomas are malignant.
Conference: 7th Annual ENETS Conference (2010)
Category: Clinical
Presenting Author: DR Christos Toumpanakis
#3071 Hormonal Ectopic Secretion Syndromes Associated to Neuroendocine Neoplasia
Introduction: Neuroendocrine neoplasias (NEN) are able to produce and secrete bioactive peptides and cause distinct syndromes related to their biological effects. The outcome will depend on the oncological disease as well as the hormonal syndrome
Conference: 17th Annual ENETS Conference 2020 (2020)
Category: Biomarkers
Presenting Author: MD Julieta Tkatch
#145 Paragangliomas: a series of five cases
Introduction: Paraganglioma is a rare extraadrenal neuroendocrine tumor, composed of chromaffin cells and arising from the neural crest. The head, neck, and retroperitoneum are the most common sites for paraganglioma.
Conference: 7th Annual ENETS Conference (2010)
Category: Clinical
Presenting Author: Assoc. Professor Catalina Poiana
Authors: Poiana C, Carsote M, Paun D, Hortopan D, ...