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Organizers Mercedes Robledo; José María de Campos; James Gnarra; Eric Jonasch Mª Elena Kusak; Susi Martinez; Ilene Sussman; Karina Villar Scientific Committee José María de Campos; Carlos Cenjor; Graeme Eisenhofer; Eric Jonasch; Mª Elena Kusak; Eamonn Maher; Marcos Malumbres; Giuseppe Opocher; Mercedes Robledo; Karina Villar October 22, 2014 19.00 p.m. Registration and Reception at the Hotel October 23, 2014 8.30 Registration (at the Symposium venue) 8.45 Welcome - Mercedes Robledo 9.00 Opening Lecture Title pending to be defined Karel Pacak National Institutes of Health, Bethesda, USA Session 1 New insight from OMIC approaches (talks: 20 minutes + 5 for discussion) 9.30 Metabolomic profiling Othon Iliopoulos Center for Cancer Research, Massachusetts General Hospital Cancer Center, Charlestown, USA

9.55 Title to be defined Amato Giaccia Stanford University 10.20 Exomes/genomes Eamonn Maher Department of Medical Genetics. University of Cambridge, UK 10.45 Transcript-omics in VHL disease and renal cell carcinoma Rathmell WK Lineberger Comprehensive Cancer Center, University of North Carolina, Chapel Hill, USA 11.10 Coffee Break and poster view Session 2: The Cilia Centrosome cycle and VHL (talks: 20 minutes + 5 for discussion) 11.40 Role of VHL in Ciliogenesis (pending title) Wilhelm Krek Institute of Molecular Health Sciences, ETH Zurich, Zurich, Switzerland. 12.05 Role of Aurora Kinase in modulating cilia centrosome cycle Speaker to be defined 12.30 13.15 Oral presentations (up to 3 short oral presentations) 13.30 Lunch and poster view Session 3. New insights on diagnostic and prognostic markers in VHL. (talks: 20 minutes + 5 for discussion) 15.00 Diagnosis and Clinical management of VHL disease Giuseppe Opocher Veneto Institute of Oncology, Padova, Italy 15.25 Title to be defined Zhuang Z Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, USA. 15.50 Pathology of VHL disease Alexander Vortmeyer Yale University School of Medicine, New Haven, CT 06520, USA. 16.15 Coffee Break and poster view

16.45 Screening and diagnostic aspects of Endolymphatic Sac Tumors ELSTs Marie Louise Mølgaard Binderup. Dept. of Cellular and Molecular Medicine, University of Copenhagen, Denmark 17.10 Pregnancy and VHL. Title to be defined Jacques Lenders Radboud University Medical Center Nijmegen, The Netherlands. 17.35 Genetic counseling on VHL Ignacio Blanco Genetic Counselling Unit L Hospitalet, ICO-Hospital Duran i Reynals, Barcelona, 18.00 Oral presentations (up to 3 short oral presentations) 18.50 Bus to the Hotel 20.45 Dinner October 24, 2014 Session 4. Current therapeutic scenario: from the animal models to the patient (talks: 20 minutes + 5 for discussion) 9.00 State-of-the-art of the therapeutic approaches Eric Jonasch Department of Genitourinary Medical Oncology, University of Texas MD Anderson Cancer Center, Houston, USA 9.25 A zebrafish model to study and therapeutically manipulate hypoxia signaling in tumorigenesis Rachel Giles University Medical Center Utrecht, The Netherland 9.50 Mouse models of cooperative tumour suppression in clear cell renal cell carcinoma Ian Frew Institute of Physiology, University of Zurich, Zurich, Switzerland 10.15 Coffee Break and poster view 10.45 Treatment options in pancreatic neuroendocrine tumours Pascal Hammel Service de Gastroenterologie-Pancreatologie, Hôpital Beaujon, AP-HP, Faculté Denis Diderot (Paris VII), Clichy, France

11.10 Therapeutic markers of antiangiogenic drugs Cristina Rodríguez-Antona Hereditary Endocrine Cancer Group, CNIO, Madrid,. 11.35 Therapeutic challenges in ocular involvement José García Arumi Department of Ophthalmology, Hospital Valle de Hebrón, Barcelona 12.00 12.45 Oral presentations (up to 3 short oral presentations) 13.00 Lunch and poster view Session 5. Challenges in surgery of VHL patients (talks: 20 minutes + 5 for discussion) 14.30 Therapy for sporadic and von Hippel Lindau related hemangioblastomas of the central nervous system Sven Gläsker Department of Neurosurgery, Freiburg University Medical Center, Freiburg, Germany. 14.55 Neurosurgery management of hemangioblastomas in peculiar locations: Brain stem and spinal root hemangioblastomas José María de Campos Department of Neurosurgery, VHL Unit Care, Fundación Jiménez Díaz, Madrid 15.25 Radiosurgery in CNS hemangioblastomas. When and how? Mª Elena Kusak Department of Neurosurgery, Radiosurgery Unit, Hospital Ruber Internacional 15.50 Coffee Break and poster view 16.20 Minimally Invasive Surgery for Pheochromocytomas and Retroperitoneal Paragangliomas Martin K. Walz Kliniken Essen-Mitte, Klinik für Chirugie und Zentrum für Minimal Invasive Chirugie, Essen, Germany 16.45 Surgery for EndolymphaticSac Tumors (ELST) and auditory rehabilitation Carlos Cenjor Fundación Jiménez Díaz, Madrid,

17.10 Challenges in RCC surgery Jean-Jacques Patard CHU, Bicêtre, France 17.35 18.20 Oral presentations (up to 3 short oral presentations) 18.40 Bus to the Hotel 20.30 Speaker s dinner 8.30 9.00 a.m. Registration October 25, 2014 VHL for families 9.00 The VHL disease explained to the families Karina Villar Health Department of Castile-La Mancha, Toledo Vice-president Alianza Española VHL 9.30 The experience of the Spanish VHL Care Unit José María de Campos Department of Neurosurgery, VHL Care Unit, Fundación Jiménez Díaz, Madrid, 10.00 Dealing with the Emotions of VHL Patient. Interactive activity with patients conducted by a psychologist 11.00 Break Roberto Álvarez Fundación Instituto San José. Hermanos de San Juan de Dios, Madrid 12.00 VHLFA s International Patient Registry Ilene Sussman VHL Family Alliance, USA 12.30 Spanish VHL Alliance: Working together to find a cure Susi Martínez Presidenta de la Alianza VHL, 13.00 Closure of the Symposium 14.30 Lunch José María de Campos Department of Neurosurgery, VHL Care Unit, Fundación Jiménez Díaz, Madrid,