ReviewOncoTargets and therapy2020
Von Hippel-Lindau Disease: Current Challenges and Future Prospects.
Review in OncoTargets and therapy, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 55 papers.
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Who cites it
55 citing papers in PubMed, 97 citations in OpenAlex.
- Somatic mutational landscape in von Hippel-Lindau familial hemangioblastoma.Molecular oncology · 2026Article
- Characterization of von Hippel Lindau gene variants in an African American cohort.HGG advances · 2026Article
- Monitoring and treatment patterns of von Hippel-Lindau disease-associated central nervous system hemangioblastomas.Hereditary cancer in clinical practice · 2026Article
- Ophthalmic screening in phakomatoses.Frontiers in cell and developmental biology · 2026Article
- Dysregulation of the ubiquitin-proteasome system in von Hippel-Lindau syndrome: molecular insights and clinical perspectives.Clinical and experimental medicine · 2025Review
- [A Family case of von Hippel-Lindau syndrome].Problemy endokrinologii · 2025Article
- Belzutifan for patients with Von Hippel-Lindau (VHL) disease-associated heterogeneous tumors - a retrospective single center analysis.BMC cancer · 2025Article
- A Sight-Threatening Genetic Disorder: A Rare Ocular Manifestation of Von Hippel-Lindau Syndrome.Cureus · 2025Article
- VON Hippel-Lindau syndrome: A rare case of epididymal cystadenoma associated with hemospermia.Urology case reports · 2025Article
- Hypoxia Inducible Factor-2α (HIF-2α) Pathway Inhibitors.Journal of kidney cancer and VHL · 2025Review
- Neuroendocrine Tumors: Germline Genetics and Hereditary Syndromes.Current treatment options in oncology · 2025Review
- Genetics, Pathophysiology, and Current Challenges in Von Hippel-Lindau Disease Therapeutics.Diagnostics (Basel, Switzerland) · 2024Review
- Expression of somatostatin receptors in hemangioblastomas associated with von Hippel-Lindau disease as a novel diagnostic, therapeutic, and follow-up opportunity: A case report and literature review.Archives of endocrinology and metabolism · 2024Review
- Epidemiology and economic burden of Von Hippel-Lindau Disease-associated central nervous system hemangioblastomas and pancreatic neuroendocrine tumors in the United States.Orphanet journal of rare diseases · 2024Article
- New Prospects on Neuroimaging in Von Hippel Lindau Disease-A Narrative Review.Diagnostics (Basel, Switzerland) · 2024Review
- A rare case of a sporadic retroperitoneal hemangioblastoma.Journal of surgical case reports · 2023Article
- Belzutifan in adults with VHL-associated central nervous system hemangioblastoma: a single-center experience.Journal of neuro-oncology · 2023Article
- Selective HIF2A Inhibitors in the Management of Clear Cell Renal Cancer and Von Hippel-Lindau-Disease-Associated Tumors.Medical sciences (Basel, Switzerland) · 2023Review
- Comprehensive characterization and building of National Registry of von Hippel-Lindau disease in Brazil.Molecular genetics & genomic medicine · 2023Article
- A complementary comment on primary hepatic angiosarcoma: A case report.World journal of clinical cases · 2023Article
Corrections and comments
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Authors and funding
5 authors at 4 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Understanding of molecular mechanisms of tumor growth has an increasing impact on the development of diagnostics and targeted therapy of human neoplasia. In this review, we summarize the current knowledge on molecular mechanisms and their clinical implications in von Hippel-Lindau (VHL) disease. This autosomal dominant tumor syndrome usually manifests in young adulthood and predisposes affected patients to the development of benign and malignant tumors of different organ systems mainly including the nervous system and internal organs. A consequent screening and timely preventive treatment of lesions are crucial for patients affected by VHL disease. Surgical indications and treatment have been evaluated and optimized over many years. In the last decade, pharmacological therapies have been evolving, but are largely still at an experimental stage. Effective pharmacological therapy as well as detection of biomarkers is based on the understanding of the molecular basis of disease. The molecular basis of von Hippel-Lindau disease is the loss of function of the VHL protein and subsequent accumulation of hypoxia-inducible factor with downstream effects on cellular metabolism and differentiation. Organs affected by VHL disease may develop frank tumors. More characteristically, however, they reveal multiple separate microscopic foci of neoplastic cell proliferation. The exact mechanisms of tumorigenesis in VHL disease are, however, still not entirely understood and knowledge on biomarkers and targeted therapy is scarce.
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