Abstract library

931 results for "Von Hippel-Lindau disease".
#1924 Multimodal Treatment of a Metastatic Pancreatic NeuroEndocrine Tumor in Von Hippel Lindau Disease
Introduction: Von Hippel-Lindau disease (VHL) is an inherited neoplastic syndrome: pancreatic lesions in 35-77% of cases, usually serous cystic adenoma (SCA), but 5-17% are non-functioning (NF) NeuroEndocrine tumors (NETs).
Conference: 14th Annual ENETS conference (2017)
Category: Medical treatment - Targeted therapies
Presenting Author: Dr Anna Caterina Milanetto
#416 A Rare Case of Cushing Syndrome Caused by an Ectopic ACTH-Secreting Pancreatic Neuroendocrine Tumor in a Patient with Von Hipple-Lindau Disease
Introduction: Von Hipple-Lindau (VHL) disease is a rare autosomal dominant disorder, that causes the susceptibility to various tumors, both benign and malignant. The most frequent tumors are central nervous system haemangioblastomas, especially cerebellum and retinal haemangioblastomas, renal cell carcinoma /renal cysts and pheocromocytoma.
Conference: 9th Annual ENETS Conference (2012)
Category: Clinical
Presenting Author: Andrea Lania
#626 Preliminary Results of a Nationwide Survey of Pancreatic Neuroendocrine Tumors in von Hippel–Lindau Disease in Japan
Introduction: von Hippel–Lindau disease (VHL) is an intractable disease characterized by various tumors including pancreatic neuroendocrine tumors (PNET).
Conference: 10th Annual ENETS Conference (2013)
Category: Epidemiology/Natural history/Prognosis - Registries, nationwide and regional surveys
Presenting Author: Dr. Hisato Igarashi
#806 Long-Term Prognosis of Pancreatic Neuroendocrine Tumors in Von Hippel-Lindau Disease
Introduction: Management of pancreatic neuroendocrine tumours (PNET) associated with von Hippel-Lindau (VHL) disease is challenging because of their malignant potential and poorly predictable prognosis.
Conference: 11th Annual ENETS Conference (2014)
Category: Epidemiology/Natural history/Prognosis - Prognosis
Presenting Author: Louis De Mestier
#1854 VHL Genotype Is Associated with Pancreatic Neuroendocrine Tumors (PNETs) Phenotype in Patients with Von Hippel-Lindau Disease (VHLD)
Introduction: 8-20% of patients with VHLD develop PNETs. However, there are no markers for PNETs progression in VHLD patients. The type of mutation in VHL gene is associated with clinical phenotype of VHLD.
Conference: 14th Annual ENETS conference (2017)
Category: Epidemiology/Natural history/Prognosis - Prognosis
Presenting Author: MD Amit Tirosh
Keywords: VHL, mutation, prognosis
#1928 Pancreatic Involvement in VHL Disease: The Turin Experience
Introduction: Von Hippel-Lindau disease (VHL) is a rare heritable genetic syndrome. Its typical manifestations concern CNS but it may affect other organs such as the pancreas.
Conference: 14th Annual ENETS conference (2017)
Category: Epidemiology/Natural history/Prognosis - Descriptive epidemiology
Presenting Author: Pablo Cortegoso Valdivia
Keywords: VHL
#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
#104 Myocardial motion abnormalities in patients with carcinoid disease without overt carcinoid heart disease
Introduction: Cardiac abnormalities occur in 15-70% of patients with carcinoid disease, typified by fibrosis of the right-sided valves. Myocardial motion abnormalities in subjects without overt carcinoid heart disease have not been previously described. Tissue Doppler Imaging (TDI) allows quantative analysis of myocardial motion. We used this technique to investigate whether myocardial motion is reduced, suggesting fibrosis, in patients with carcinoid disease.
Conference: 7th Annual ENETS Conference (2010)
Category: Clinical
Presenting Author: Christopher Wong
#84 Rare association between pancreatic insulinoma and Graves's disease: fortune or misfortune?
Introduction: In spite of low frequency ( 4 cases in 1 million population), insulimoma is a significant pathology because hypoglycemia causes severe neurologic lesions and death.
Conference: 7th Annual ENETS Conference (2010)
Category: Clinical
Presenting Author: Dr Carmen Barbu
Authors: Barbu C, Stefan C, Miron A, Giulea C, ...
#105 A pilot study using biochemical markers to predict acrcinoid heart disease in patients with carcinoid disease
Introduction: Carcinoid heart disease (CHD) affects up to 15-70% of patients with carcinoid disease. Recent ENETS guidelines recommend regular echocardiographic assessment to screen for CHD. Chromogranin A (CgA) is a peptide widely used to monitor carcinoid disease progression. Neurokinin A is a tachykinin which may be a more sensitive measure of carcinoid disease progression and prognosis. N-terminal pro Brain Naturetic Peptide is used as a screening tool for heart failure and recent results suggest utility in the diagnosis of CHD.
Conference: 7th Annual ENETS Conference (2010)
Category: Clinical
Presenting Author: Dr Christopher Wong
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