Program

Date: MAY 20 (Thursday) -22(Saturday),2004
Location: Kochi City Culture Plaza CUL-PORT & Hotel Nikko Kochi Asahi Royal, Kochi, Japan

Registration is also available on May 19 (wed) : 16:00 - 18:00
The meeting on May 23 has been cancelled.

Thursday, May 20, 2004

Workshop I ( 9:00 - 12:00 )

"Hypoxia Inducible Factors and Regulation of Hypoxia Responses"
Chair: Kazuhiro Sogawa and Takumi Kamura, Japan

Inactivation of the HIF prolyl hydroxylase (PHD) enzymes in mice
Patrick H. Maxwell, UK

Negative feedback regulation of hypoxia-inducible gene expression by a bHLH/PAS factor IPAS
Yuichi Makino, Arvydas Kanopka, Hiroshi Nakamura, Lorenz Poellinger, Hirotoshi Tanaka, Japan and Sweden

Regulation of HIF-prolyl hydroxylase activity by functional gas molecules, NO and H2O2
Ken-ichi Yasumoto, Junko Sugiyama, Chikahisa Takasaki, Kazuhiro Sogawa, Japan

VHL-mediated functional interaction of both HIF-1α-hydroxylation systems on the hypoxia-induced HIF-1α-transactivation
Kiichi Hirota, Japan

VHL-regulated ubiquitination of Rpb1 by oxidative stress and hypoxia
Maria F. Czyzyk-Krzeska, Monika L. Ignacak, Svetlana Kolobanova, USA

VHL and hypoxia regulate kidney compartmentalization through Eph/Ephrins
Rachel Giles, Martijn Lolkema, Cristel Snijckers, Sabine Elowe, Anita Jorna, Edu Batlle, Tony Pawson, Emile Voest, The Netherlands

C-terminus of the conserved SOCS-box motif present in members of the SOCS-box protein families is indispensable for interaction with Cul5 and Rbx2
T Kamura, RC Conaway, JW Conaway, KI Nakayama, Japan and USA

The regulation of HIF alpha is necessary and sufficient for tumor suppression by pVHL
Keiichi Kondo, William G. Kaelin, Jr., Japan and USA

New Proteomic Approach for Identification and Validation of Hypoxia and pVHL regulated proteins
Eijiro Nakamura, William G. Kaelin, Jr., Japan and USA

Lunch Time Seminar ( 12:15 - 13:15 )

Chair: Shigeo Ohno, Japan

"Functions of the VHL Tumor Suppressor Protein"
W. Kaelin, J. Brugarolas, W. Kim, K. Kondo, E. Nakamura, S. Schlisio, M. Sagran, H. Yang, USA

(Sponsored by:Otsuka Pharmaceutical Co.,Ltd.)

Session I ( 13:30 - 15:00 )

"Basic study in the role VHL protein for tumor formation and basic therapeutic approach"
Chair: William G. Kaelin, Jr. and Laura S. Schmidt, USA

Conditional inactivation of the VHL gene during mouse embryo development
Seung-Beom Hong, Mutsuo Furihata, Berton Zbar, Laura S. Schmidt, USA

Tumor suppression by the von Hippel-Lindau protein is dependent on casein kinase II-mediated phosphorylation of the N-terminal acidic domain
Martijn P.J.K. Lolkema, Michelle L. Gervais, Natalie H. Stickle, Heng Qi, Cristel M.J.T. Snijckers, Richard P. Hill, Rachel H. Giles, Emile E. Voest, Michael Ohh, The Netherlands and Canada

Role of VHL protein in neuronal differentiation and therapeutic application for neuronal regeneration
Hiroshi Kanno, Hidetoshi Murata, Mari Dezawa, Toshiro Mimura, Jun Suenaga, Shuichi Nakano, Naoki Sugimoto, Hiroyuki Aburatani, Isao Yamamoto, Japan

Inactivation of VHL gene induces constitutive phosphorylation of MET protein in clear cell renal carcinoma
Noboru Nakaigawa, Masaya Baba, Takeshi Kishida, Masahiro Yao, Yoshinobu Kubota, Japan

Genotype-Phenotype Correlations in von Hippel-Lindau disease
Eamonn R. Maher, UK

Session II ( 15:15 - 17:30 )

"Genotype-Phenotype study and clinical study in the VHL disease and familial kidney tumor syndromes I"
Chair: Hirotsugu Uemura and Masahiro Yao, Japan

Improved detection of large intragenic deletions in the von Hippel-Lindau Gene
Gabi Wildhardt, Ulrich Lentes, Dirk Reutzel, Ekkhardt Lausch, Silke D üsterhöft, Dirk Prawitt, Christian Spangenberg, Bernhardt Zabel, Daniela Steinberger, Jochen Decker, Germany

VHL-dependent and -independent regulation of MN/CA9 in renal cell carcinoma cell lines
Youichi Kawada, Hirotsugu Uemura, Yoshihiko Hirao, Japan

Frequent losses at 6q in sporadic and VHL-associated capillary hemangioblastomas
Lemet S, Husgafvel-Pursiainen K, Böhling T, Finland

Identification of Differentially Expressed Genes in Renal Parenchymal Tumors Using High-density Oligonucleotide Microarray: Adipophilin as a Potential Diagnostic and Prognostic Biomarker for Clear-cell Renal Carcinoma
Masahiro Yao, Hisahiro Tabuchi, Yoji Nagashima, Masaya Baba, Noboru Nakaigawa, Kenji Hamada, Takeshi Kishida, Keiichi Kondo, Hisafumi Yamada-Okabe, Japan

A new potential cause of occupational cancer without somatic VHL gene mutations
Stéphane Richard, Sophie Ferlicot, Marie-Noëlle Carrette, Christophe Béroud, Ellen Imbernon, Yuriko Iwatsubo, Hubert Egloff, Dominique Sordet, Jean-Michel Salé, France

The expression of HIF-1α, 2α in Chinese sporadic clear cell renal cell carcinoma and their relationships to the mutations of VHL gene
Kan Gong, Ning Zhang, Xi Na, Guan Wu, Yanqun Na, China and USA

Co-expression of erythropoietin and erythropoietin-receptor in VHL disease-associated tumors
Lee YS, Vortmeyer AO, Lonser RR, Oldfield EH, Zhuang Z, USA and Korea

Evening Seminar ( 17:30- 18:30 )

Chair: Eamonn R. Maher

The role of Hypoxia-inducible factor 1 in cancer
Patrick H. Maxwell, UK
(Sponsored by: Chikamori-kai Medical Corporation Chikamori Hospital)

Welcome Reception at Restaurant Monte, 1F Kochi City Culture Plaza CUL-PORT ( 19:00 - 21:00 )

(Sponsored by: Chikamori-kai Medical Corporation Chikamori Hospital)

Friday, May 21, 2004

Session III ( 8:30 - 10:20 )

"Genotype-Phenotype study and clinical study in the VHL disease and familial kidney tumor syndromes II"
Chair: Laura S. Schmidt, USA and Okio Hino, Japan

Mutations in BHD, a novel tumor suppressor gene, predispose to hamartomas of the hair follicle, spontaneous pneumothorax and an increase risk for kidney cancer
Laura S. Schmidt, Michael L. Nickerson, Michelle B. Warren, Jorge R. Toro, Gladys Glenn, Maria L. Turner, Maria Merino, Peter L. Choyke, Cathy Vocke, Christian P. Pavlovich, Nirmala Sharma, Masaya Baba, Wei Zhen, W. Marston Linehan, Berton Zbar, USA

Mutation in the Birt-Hogg-Dube syndrome gene in sporadic renal cell carcinomas
Takeshi Kishida, Takayuki Murakami, Masaya Baba, Noboru Nakaigawa, Masahiro Yao, Yoshinobu Kubota, Berton Zbar, Laura Schmidt, Japan and USA

Hereditary renal carcinogenesis
Okio Hino, Japan

Histological classification of renal cell carcinomas (RCCs). Up-to-date and problems
Yoji Nagashima, Masahiro Yao, Japan

Histogenesis of VHL disease-associated hemangioblastoma
Vortmeyer AO, Lee YS, Lonser RR, EH Oldfield, Z Zhuang, USA

Genotype-Phenotype Study and Clinical Study in VHL and Familial Kidney Tumor Syndromes
Peter L. Choyke, USA

Session IV ( 10:30 - 12:05 )

"Clinical progress in VHL disease and its Ethical, Legal and Social Issues"
Chair: Hartmut P. Neumann, Germany and Michiya Ito, Japan

The growing Family of Pheochromocytoma and Paraganglioma Syndromes
Hartmut P.H. Neumann, Germany

Retrolabyrinthine posterior petrosectomy for hearing preservation and resection of endolymphatic sac tumors
H. Jeffrey Kim, Russell R. Lonser, John A. Butman, Carmen Brewer, Christopher Zalewski, Gladys Glenn, Marston W. Linehan, Alexander O. Vortmeyer, Edward H. Oldfield, USA

Endolymphatic sac tumors in von Hippel-Lindau disease: implications for natural history, diagnosis, and treatment
Russell R. Lonser, Hetty L. DeVroom, H. Jeffrey Kim, John A. Butman, Alexander O. Vortmeyer, Daniel I. Choo, Edward H. Oldfield, USA

The psychological impact of clinical monitoring for Von Hippel-Lindau disease
Sally Watts, Lindsay Fraser, Stephen Sutton, Anna Cargill, Shirley Hodgson, UK

Ethical, Legal, and Social issues of Familial cancer, focusing on discrimination of Life insurance
Michiya Ito, Nobuo Koinuma, Tomoko Saeki, Hideki Ishikawa, Japan

Lunch Time Seminar ( 12:15 - 13:15 )

Chair: Hisayuki Ueno and Keiji Shimizu, Japan

Surgical experience of spinal cord hemangioblastoma
Isao Yamamoto, Japan

Treatment of Retinal Capillary Hemangioma (von Hippel Disease)
Shin Yoneya, Japan

Workshop II ( 13:30 - 15:20 )

"Clinical experiences in the diagnosis and treatment in VHL and other familial kidney tumors"
Chair: Tsuneharu Miki, Japan and Peter L. Choyke, USA

The experience of non-ischemic partial nephrectomy with microwave tissal coagulator for multiple renal cell carcinomas
Yoshihiko Hirano, Kiyohide Fujimoto, Hirotsugu Uemura, Masaki Cho, Yoshinori Nakagawa Japan

MR-imaging guided percutaneous cryoablation of renal cell carcinoma: Short term follow up
Tadashi Shimizu, Akihiro Sawada, Yoshihisa Kodama, Yusuke Sakuhara, Daisuke Abo, Kazuo Miyasaka, Japan

Percutaneous Radio Frequency Ablation of Renal Cell Carcinoma
Shin Ebara, Tomoko Kobayashi, Takashi Saika, Yasutomo Nasu, Tsushima Tomoyasu, Kumon Hiromi, Japan

Radiofrequency Ablation Combined with Selective Renal Arterial Embolization of Small Renal Cell Carcinoma : Early Experience
Takehisa Ohnishi, Kiminobu Arima, Yoshiki Sugimura, Japan

Radio-frequency ablation - Future application for renal tumor with VHL disease -
Koji Okihara, Osamu Ukimura, Soh Ushijima, Akira Fujito, Yoichi Mizutani, Akihiro Kawauchi, Tsuneharu Miki, Japan

Imaging in the Diagnosis and Treatment of VHL And Other Familial Kidney Tumors
Peter L. Choyke, USA

Session V ( 15:30 - 17:40 )

"Poster Discussion Session"
Chair: Teijiro Aso and Nobuo Shinohara, Japan

Mutant VHL-induced molecular and phenotypic changes in VHL-proficient cells
Yih-Horng Shiao, Janet R. Fields, Lucy M. Anderson, Laura W. Fornwald, USA

The immunohistochemical study of nephritis rats in terms of HIF1 alpha expression
Yoshihiro Kudo, Yoshihiko Kakinuma, Norihito Morimoto, Takashi Karashima, Mutsuo Furihata, Noriko Kishimoto, Yasukiyo Mori, Yuji Otsuki, Taro Shuin, Tetsuro Sugiura, Japan

Renal Cell Carcinoma- and Pheochromocytoma-specific Altered Gene Expression Profiles in VHL Mutant Clones
Mutsumi I. Tsuchiya, Heiwa Okuda, Yasuyuki Takaki, Shingo Ashida, Masaya Baba, Syu-ichi Hirai, Shigeo Ohno, Taro Shuin, Japan

Promoter hypermethylation of multiple genes in renal cell carcinoma
Heiwa Okuda, Minoru Toyota, Mutsumi Tsuchiya, Takashi Tokino, Taro Shuin, Japan

VHL is a target of WNT signaling and may regulate the angiogenic switch in colorectal carcinoma
Rachel Giles, Cristel Snijckers, Anita Jorna, Hans Clevers, Emile Voest, The Netherlands

Tumor suppressor VHL regulates microtubule dynamics through Kinesin II interaction
Moniek van Beest, Martijn P. Lolkema, Cristel M. Snijckers, Mascha van Noort, Dorus A. Mans, Anita S. Jorna, Rachel H. Giles, Emile E. Voest, The Netherlands

Association of Chinese sporadic renal clear cell carcinomas with somatic mutations of the von Hippel-Lindau(VHL) tumor suppressor gene
Kan Gong, Ning Zhang, Hongfeng Guo, Xi Na, Guan Wu, Yanqun Na, China and USA

Clinical significance of association of allelic loss of chromosome 3p25 with alterations of chromosome 5q22.3-q23.2 in conventional renal cell carcinoma
Kazuhiro Nagao, Shiro Yamaguchi, Satoru Yoshihiro, Hideyasu Matsuyama, Kazuo Oba, Yoshihito Korenaga, Hiroshi Hirata, Koji Fukunaga, Katsusuke Naito, Japan

A genetic study of inherited kidney cancers in France
Sophie Giraud, Betty Gardie, Rosette Lidereau, Christophe Béroud, Dominique Chauveau, Jean-Michel Corréas, Yves Chrétien, Laurent Boccon-Gibod, Nicolas Thiounn, Gérard Benoît, Jean-Pierre Grünfeld, Stéphane Richard, France

A novel family of type2A von Hippel-Lindau (VHL) disease with an R167W mutation in the VHL gene
Ken Kishida, Jun-ichiro Miyagawa, Makoto Moriwaki, Kohei Okita, Hiromi Iwahashi, Akihisa Imagawa, Kazuya Yamagata, Tohru Funahashi, Yuji Matsuzawa, Japan

Clinical features and prognosis of renal cell carcinoma in Japanese patients with von Hippel-Lindau disease
Nobuo Shinohara, Toru Harabayashi, Kiyoshi Koshida, Sei-ichiro Ozono, Hayakazu Nakazawa, Yoshiki Sugimura, Masahiro Yao, Katsuya Nonomura, Japan

Clinical manifestations of pancreatic neuroendocrine tumors associated with von Hippel-Lindau disease in Japan
Ichiro Yamasaki, Isao Nishimori, Kenji Tamura, Shingo Ashida, Taro Shuin, Japan

A case of strongly suspected von Hippel-Lindau disease associated with renal cell carcinoma and neuroendocrine tumors of the pancreas
Yasuhiro Ito, Koichi Aiura, Naokazu Chiba, Tetsu Hayashida, Shigeyuki Kawachi, Minoru Tanabe, Go Wakabayashi, Motohide Shimazu, Masakazu Ueda, Masaki Kitajima, Japan

Laser treatment for retinal angiomatosis in von Hippel-Lindau Disease. Follow-up of twenty-years
D. Schmidt, H. P.H. Neumann, Germany

Endoscopic Treatment of Pheochromocytomas and Paragangliomas in VHL Patients
M. K. Walz, K. Peitgen, H. P.H. Neumann, German

Multiple spinal hemangioblastomas: Manifestation of von Hippel-Lindau's syndrome
Fredrick Junn, Shaden Marzouk, Ghaus Malik, USA

A case of a renal mimicking tumor in a VHL disease patient during the postoperative follow-up of bilateral RCC
Nozomu Kawata, Nagane Yusuke, Koutaro Morita, Akiko Kitajima, Tomohiro Igarashi, Takahiro Sakuma, Katsuhiko Sato, Takumi Igarashi, Hitoshi Hirakata, Yukie Takimoto, Japan

A case of retinal hemangioma on the optic disc and multiple retinal lesions associated with Von Hippel disease
Katsuaki Miki, Mitumasa Wada, Kanji Takahasi, Tetuya Nisimura, Miyo Matsumura, Japan

The Importance of Group Support
Joyce Graff, Jennifer Kingston, Florian Hofmann, USA, Australia and Germany

London Dysmorphology Database and Geneeye Databases Illustrated by the entry on Von Hippel Lindau disease
H U Moller, Denmark

Evening Seminar ( 17:50 - 18:50 )

Chair: Harmut P. Neumann, Germany

(Sponsored by:SHIONOGI & CO.,LTD.)

Laparoscopic Adrenalectomy: New Standard
Eiji Higashihara, Japan

Laparoscopic partial nephrectomy
Toshiro Terachi, Yukio Usui, Masanori Shima, Yasuyuki Kobayashi, Hiroshi Sasaki, Hideyuki Yamashita, Mana Aoki, Hajime Onda, Masatoshi Tokunaga, Satoshi Watanabe, Hiroaki Inatsuchi, Hideshi Miyakita, Japan

Banquet at Hotel Nikko Royal Kochi Asahi (19:10 - 21:10 )

Saturday, May 22, 2004

Special Lecture ( 9:00 - 10:00 )

Chair: Eiji Higashihara, Japan

Clinical Aspects of hereditary cancer
Tadao kakizoe, Japan

Workshop III ( 10:00 - 12:00 )

"Clinical experiences in the diagnosis and treatment of the Central Nervous System tumors"
Chair: Masao Matsutani and Junkoh Yamashita, Japan

The natural history of central nervous system tumors in von Hippel-Lindau disease: a 10-year follow-up
Joshua Ammerman, Russell R. Lonser, John A. Butman, Hetty L. DeVroom, Edward H. Oldfield, USA

Surgical management of large brainstem hemangioblastoma in patients with VHL disease
Yutaka Sawamura, Japan

Aquaporin, claudin 5, and erythropoietin-receptor (Epo-R) expression in cerebellar hemangioblastomas - Clinicopathological and immunohistochemical study -
Osamu Tachibana, Chen Yizao, Mitsuhiro Hasegawa, Junkoh Yamashita, Japan

Treatment based on gene diagnosis for CNS hemangioblastomas
Hiroshi Kanno, Isao Yamamoto, Masahiro Yao, Taro Shuin, Japan

Hemangioblastomas in the central nervous system based on the data of Brain Tumor Registry of Japan (BTRJ)
Soichiro Shibui, Kazuhiro Nomura, Japan

Surgical Outcome of Patients with Medullary and Spinal Nerve Hemangioblastomas
Sven Gläsker, Vera van Velthoven, Neumann P.H. Hartmut, Germany

Diagnosis and surgical outcome of central nervous system hemangioblastomas with or without von Hippel-Lindau disease
Hisashi Hatano, Toshihiko Wakabayashi, Jun Yoshida, Japan

Lunch Time Seminar ( 12:15 - 13:15 )

"Case presentation and Discussion in the diagnosis and treatment for the VHL and other familial kidney tumors"
Chair: Russell Lonser and Peter L. Choyke, USA

(Sponsored by: Mishima Surgical Gastro Intestinal Clinic)

VHL or just a coincidence ?
H U Moller, Lone Sunde, Denmark

How can we manage multiple adrenal, renal and pancreatic tumors in a patient with von Hippel-Lindau disease?
Ichiro Yamasaki, Isao Nishimori, Kenji Tamura, Shingo Ashida, Taro Shuin, Japan

Case presentation of a VHL family: its clinical management and problems
Yasumoto Nasu, Shin Ebara, Takashi Saika, Hiromi Kumon, Japan

Session VI ( 13:30 - 16:30 )

"Discussion with Basic Scientists, Clinical Doctors and Patients in the World"
Chair: Taro Shuin, Japan and Russell R. Lonser, USA

Cerebellar and spinal hemangioblastomas in von Hippel-Lindau disease: diagnosis, treatment, and follow-up
Hiroshi Kanno, Japan

Adjournment

The meeting on May 23 has been cancelled.