Thursday 4th September BASIC AND MOLECULAR BIOLOGY OF vHL
09:00 a.m.
REGISTRATION
10:30 a.m.
OPENING
Chair: Patrick Maxwell and Thomas van Oveerem Hansen
10:40 a.m.
Peter Staller: The role of hypoxia-inducible factors in aggressive cancer behavior.
11:20 a.m
Patrick Maxwell: A critical role for VHL in glucose-sensing.
11:50 a.m
Rachel Giles: Tumor suppressor pVHL regulates kidney cell polarity 3D epithelial morphogenesis.
12:05 a.m
Norma Masson: Further studies of the pVHL-Collagen IV interaction.
12:20 a.m.
James Gnarra: The VHL E3 ubiquitin ligase complex and post-transcriptional regulation of hypoxia-induced gene expression.
12:35 a.m.
Eijiro Nakamura: Up regulation of JunB by the inactivation of von Hippel-Lindau tumor suppressor protein induces MMP-2 expression and enhances the invasive ability of renal cell carcinoma cells in vitro.
12:50 a.m. Summary of the morning program.
12:55 a.m. Messages
1:00 p.m. Lunch
Chair: Stephen Lee and Lennart Friis-Hansen
2:00 p.m.
Stephen Lee: Understanding the HIF_ Oncogenic Pathway in Human Cancer.
2:30 p.m.
Xiaoqing Wu: The tumour suppressor von Hippel Lindau sensitizes cells to hypoxia via regulation of survivin.
2:45 p.m
Arnin Pause: The role of VHL in tumor suppression through regulation of ECM assembly.
3:00 p.m.
Ian Frew: Cooperative suppression of renal cysts and epididymal cystadenoma by the VHL and PTEN tumour suppressors.
3:15 p.m.
Maria F Czyzyk-Krezeska: Regulation on RNA Polymerase II by VHL and prolyl hydroxylases in kidney cancer.
3:30 p.m. Coffee break
4:00 p.m.
Rachel Giles: Animal models in VHL.
4:30 p.m.
Ellen van Rooijen: The von Hippel-Lindau (VHL) tumor suppressor gene ortholog vhl regulates zebra fish hematopoisis.
4:50 p.m.
Tien Hsu: Up-regulation of FGF receptor signaling in VHL mutant cells – from flies to humans.
5:05 p.m.
Ding Xing-hua: Preliminary analysis of the gene expression profiles of central nervous system hemangioblastoma with cDNA microarray.
5:20 p.m.
Michael Wiesener: The lusyl oxidases LOX and LOXL2 are necessary and sufficient to repress E-cadherin in hypoxia – insights into cellular transformation processes mediated by HIF-1.
5:35 p.m.
Eric Jonasch: Comparative assessment of Hemangioblastoma and Renal Cell Carcinoma Tissue Using laser Scanning Cytometry.
5:50 p.m. Summary of the afternoon program
6:00 p.m. Messages
7:30 p.m. Get together buffet and visit to Viking ship museum
Friday 5th September CLINICAL AND GENETIC ASPECTS OF vHL
Chair: Eamonn Maher and Carsten Brandt
09:00 a.m. Messages
9:05 a.m.
Eamonn Maher: Genetic aspects of tumourigenesis in VHL disease.
9:50 a.m.
J.C. Casali-da-Rocha: Identification of germline mutations in the VHL gene of families with the von Hippel-Lindau disease from Brazil.
10:05 a.m.
Mette Dandanell Nielsen: Characterization of germ-line mutations in the VHL gene in von Hippel Lindau disease patients from Denmark.
10.20 a.m
Ashok Pillai: Von Hippel Lindau Disease in Kerala, India: a clinical and genetic study.
10.35 a.m.
L. Andrews: Von Hippel Lindau Syndrome - a single institution’s 12 year experience
11:10 a.m. Coffee break
Chair: Eamonn Maher and Mette Klarskov Andersen
11.15 a.m.
Hartmut Neumann: Familial Pheochromocytoma: Genetic Susceptibility, Clinical Diagnosis and Treatment Options.
11:55 a.m.
Julia R. Forman: Predicted mechanisms for renal cell carcinoma and pheochromocytoma in von Hippel-Lindau disease.
12:10 a.m.
Nicola Bradshaw: A Large Scottish VHL Kindred with Variable Penetrance.
12:25 a.m.
Lone Sunde: Reduced penetrance of c.407C>T, p.Ser65Leu in the VHL-gene.
12:40 a.m.
Sophie Giraud: Contribution of the dHPLC to the diagnosis of VHL somatic mosaicism.
12:55 a.m
Carsten A Brandt: vHL families without detected mutations in the VHL gene: is “Hereditary hemangioblastomatosis a separate entity”?
1:10 p.m. Summary of the morning program.
1:15 p.m. Lunch
Chair: Hartmut Neumann and Michael Kosteljanetz
2:10 p.m.
Alexander Vortmeyer: VHL disease - Evolution of Tumorigenesis in Human Tissues.
2:50 p.m.
V. Van Velthoven: Hemorrhagic Complications in Treatment of CNS Hemangioblastoma.
3:05 p.m.
Sven Gläsker: Essential and Pitfalls in Treatment of CNS Hemangioblastoma and von Hippel-Lindau Disease.
3:20 p.m.
Sven Gläsker: Nitric oxide as possible growth regulator of hemangioblastomas.
3:35 p.m.
Matthieu Peyre: Natural history of supratentorial hemangioblastomas in von Hippel-Lindau disease.
3:50 p.m. Coffee break
4:15 p.m.
Marianne Wegener: Involvement of the eye in von Hippel-Lindau disease: update on diagnosis and therapy.
4:30 p.m
J.M. Corréas: Radiofrequency ablation of renal tumours in von Hippel-Lindau disease.
4:45 p.m.
H. Jeffrey Kim: Surgical Management of Endolymphatic Sac Tumors in von Hippel-Lindau Disease.
5:00 p.m.
Pascal Hammel: Endocrine pancreatic tumors in von Hippel-Lindau disease: clinical, histological and genetic features.
5:15 p.m.
Charline Ladroue: New germline PHD2 mutations in patients with congenital polycythemia.
5:30 p.m.
Betty Gardie: Unexpected truncating VHL germline mutation in a patient with polycythemia.
5:45 p.m.
Eric Jonasch:Updated Results for Ongoing Phase II Clinical Trial for Sunitinib in Patients with von Hippel Lindau Disease
6:00 p.m. Summary of the afternoon program.
6:05 p.m. Messages
7:30 p.m. Congress dinner
Saturday 6th September MANAGEMENT AND HANDLING OF vHL
Chair Paul M. Zeltzer and Lone Sunde
09:00 a.m. Messages
9:05 a.m.
Paul M. Zeltzer: How the Internet has changed the classical doctor-patient relationship – or visit Genesis before you Google.
9:25 a.m.
Ying Hunang: A Three-Gene Expression Signature Model to Predict Clinical Outcome of Clear Cell Renal Carcinoma.
9:45 a.m.
Thoshinari Yamasaki: Existence of endothelial fenestrations in tumor microvasculature associated with VHL gene alteration is a potent predictive marker of anti-VEGF therapy in clear cell renal cell carcinoma in vivo.
10:05 a.m.
Marie Louise Mølgaard Poulsen: Prophylaxis in von Hippel-Lindau disease.
10:25 a.m.
Mette Bertelsen: An evaluation of Danish national clinical guidelines of von Hippel-Lindau (VHL) – Experience from a single institution.
10:45 a.m. Coffee break
11:15 a.m.
Frauke Pelz : 20 years of VHL clinic in South Wales
11:35 a.m.
L. Andrews: Unmet information and support needs amongst individuals affected by VHL or MEN2A.
11:55 a.m
C.R.M. Lammens: The psychosocial impact of von Hippel-Lindau Disease
12:15 a.m Summary and closing remarks.
12:30 p.m. Lunch
13:30 p.m. vHL leadership meeting