Recent advances in ideas on the molecular pathology and clinical aspects of Von Hippel-Lindau disease

International Journal of Clinical Oncology
Taro ShuinShingo Ashida

Abstract

Von Hippel-Lindau (VHL) disease is an autosomal dominant disorder that is associated with various tumors and cysts in the central nervous system (CNS) and visceral organs. Inactivation of the VHL tumor-suppressor protein and subsequent loss of function in the VHL, and Elongin BC (VBC) complex result in dysfunction in the ubiquitination of hypoxia-inducible factor (HIF), which is an important step in the development of angiogenic tumors. The most frequent disorders in VHL disease are hemangioblastoma in the CNS and retina, pheochromocytoma in the adrenal gland, renal cell carcinoma, and pancreatic neuroendocrine tumor. Here, we review recent ideas on the pathogenesis and clinical diagnosis and treatment of VHL disease. Progress in molecular diagnosis and molecular targeting therapy is expected for improvement in the diagnosis and treatment of this disease. The family's support for patients with VHL disease is important, being mutually helpful to overcome various social and psychological problems in the patients.

Citations

Mar 17, 2009·Journal of Molecular Medicine : Official Organ of the Gesellschaft Deutscher Naturforscher Und Ärzte·Jing-Shan HuangChi-Jung Huang
Dec 13, 2006·Familial Cancer·Lindsay FraserShirley Hodgson
Dec 15, 2012·Chinese Journal of Cancer·Cynthia RoRun Yu
Nov 13, 2010·Radiographics : a Review Publication of the Radiological Society of North America, Inc·Venkata S KatabathinaSrinivasa R Prasad
May 2, 2013·Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia·Dandan JiaHong Jiang
Jul 5, 2006·Seminars in Pediatric Neurology·Garilyn JentarraVinodh Narayanan
Jun 6, 2006·Experimental Eye Research·Olli Arjamaa, Mikko Nikinmaa
Sep 18, 2007·International Journal of Radiation Oncology, Biology, Physics·Eng-Siew KohNormand J Laperriere
Apr 20, 2006·International Journal of Clinical Oncology·Shigeru KandaHiroshi Kanetake
Aug 23, 2019·International Journal of Gynecological Pathology : Official Journal of the International Society of Gynecological Pathologists·Ana Marques, Raquel Portugal
May 18, 2011·Acta Oto-laryngologica·Tadashi KitaharaHidenori Inohara

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