Von Hippel Lindau Disease Market

DelveInsight’s ‘Von Hippel-Lindau (VHL) disease - Market Insights, Epidemiology, and Market Forecast—2030’ report delivers an in-depth understanding of VHL disease, historical and forecasted epidemiology as well as the VHL disease market trends in the United States, EU5 (Germany, France, Italy, Spain, and the United Kingdom), and Japan.


The Von Hippel-Lindau (VHL) Disease market report provides current treatment practices, emerging drugs, Von Hippel-Lindau (VHL) Disease market share of the individual therapies, current and forecasted Von Hippel-Lindau (VHL) Disease market size from 2018 to 2030 segmented by seven major markets. The Report also covers current Von Hippel-Lindau (VHL) Disease diagnostic criteria, treatment practice/algorithm, market drivers, market barriers, and unmet medical needs to curate the best of the opportunities and assesses the underlying potential of the market.

Geography Covered

  • The United States
  • EU5 (Germany, France, Italy, Spain, and the United Kingdom)
  • Japan

Study Period: 2018–2030

Von Hippel-Lindau (VHL) disease: Disease Understanding and Treatment Algorithm

Von Hippel-Lindau (VHL) Disease Overview

Von Hippel–Lindau (VHL) disease is an autosomal dominantly inherited neoplastic disorder that demonstrates marked phenotypic variability and age-dependent penetrance. A germline mutation in the VHL gene predisposes carriers to the development of abundantly vascularized tumors in multiple organs. There is a familial occurrence in 80% of cases, and the remaining cases (20%) are de novo mutations. Tumors usually first appear in young adulthood. The types of tumors associated with VHL disease include hemangioblastomas (slow-growing tumors of the central nervous system); kidney cysts and clear cell renal cell carcinoma; pancreatic neuroendocrine tumors; pheochromocytomas (noncancerous tumors of the adrenal glands); and endolymphatic sac tumors.


VHL disease does not have a single primary symptom; this is in part because it does not occur exclusively in one organ of the body. It also does not always occur in a particular age group. Specific genotype–phenotype correlations in affected families led to the classification of VHL subtypes into types 1 and 2, primarily based on the presence of a pheochromocytoma (PCC). VHL is classified on the basis of absence or presence of pheochromocytoma. Those without PCC were categorized as VHL type 1 disease. Those with PCC were categorized as VHL type 2 disease. VHL type 2 disease is further classified into three categories: type 2A, type 2B, and type 2C. Type 2A VHL has PCC with other HB in the CNS but not with RCC. Type 2B exhibits PCC, RCC, and other CNS tumors. A recent notion is that type 2C disease has only PCC, with no other disease.


Without treatment, VHL patients may have blindness and/or permanent brain damage. Death usually results from the complications of brain tumors or kidney cancer. Most VHL lesions are much easier to treat when they are small. Several possible complications of VHL do not present with symptoms until the problem has developed to a critical level. Treatment may only be able to stop symptoms that have occurred; it is not always possible to reverse the changes and go back to normal.


Von Hippel-Lindau (VHL) Disease Diagnosis

In the presence of positive family history, VHL disease can be diagnosed clinically in a patient with at least one typical VHL tumor. Typical VHL tumors are retinal, spinal, and cerebellar hemangioblastoma, renal cell carcinoma, and pheochromocytoma. Endolymphatic sac tumors and multiple pancreatic cysts suggest a positive carriership (in the presence of a positive VHL family history) since they are uncommon in the general population. In contrast, renal and epididymal cysts frequently occur in the general population and are, as sole manifestation, not reliable indicators for VHL disease. In patients with a negative family history of VHL-associated tumors, a diagnosis of VHL disease can also be made when they exhibit two or more hemangioblastomas or a single hemangioblastoma in association with another typical manifestation.


The only way to determine for sure that someone does not have an altered VHL gene is through DNA testing. A clinical diagnosis can also be made when a person exhibits a tumor-specific VHL. Once a VHL diagnosis has been made, it is important to begin surveillance testing early before any symptoms occur. Initial screening workup includes a thorough history and clinical examination, an ophthalmologic examination including fundoscopy, MR imaging of the craniospinal axis, kidneys, pancreas, and liver, an audiological examination, and laboratory tests (to detect pheochromocytomas). Genetic testing of the peripheral leukocytes and/or other body tissues is performed to confirm the presence of a VHL gene mutation. Other than this, tumors at various sites are demonstrable by using different imaging modalities, including ultrasonography, CT, MRI, radionuclide studies, and angiography. Also, establishing the diagnosis of pheochromocytoma is dependent on the demonstration of significant catecholamine excess. Levels of epinephrine (adrenaline), norepinephrine (noradrenaline), and their metabolites (breakdown products of epinephrine and norepinephrine) can be measured in either urine or blood.


Von Hippel-Lindau (VHL) Disease Treatment

Treatment for VHL disease depends on the location and size of tumors. In general, the goal is to treat growths when they cause symptoms but are still small, so they do not cause permanent damage. Treatment usually involves surgical removal of tumors. Radiation therapy may be used in some cases. Although there is no cure for VHL, the associated tumors can be treated.


Primary treatment of all VHL-related tumors is local (i.e., surgical resection, RFA, radiotherapy). However, repeated local interventions at multiple sites or repeated recurrences in one area can increase morbidity. Therefore, in individual patients, systemic treatment may be an option. This includes cases of progression of inoperable and previously irradiated cerebral HB, RCH, PHEO, PNET, or ccRCC. Systemic treatment is recommended in the metastatic setting, addressing the same therapeutic principles as in sporadic malignancies. Due to the rarity of VHL, data about systemic treatment are sparse. VEGF-targeting agents seem to be a reasonable choice with regard to pathophysiology in most tumors. High levels of VEGF are found in HB, and it has been hypothesized that blocking the VEGF pathway might disrupt tumor angiogenesis and consequently tumor regression.

Von Hippel-Lindau (VHL) Disease Epidemiology

The Von Hippel-Lindau (VHL) Disease epidemiology division provides insights about historical and current Von Hippel-Lindau (VHL) Disease patient pool and forecasted trends for every seven major countries. It helps to recognize the causes of current and forecasted trends by exploring numerous studies and views of key opinion leaders. This part of the DelveInsight report also provides the diagnosed patient pool and their trends along with assumptions undertaken.


Key Findings

In the year 2020, the total prevalent cases of Von Hippel-Lindau (VHL) Disease was 21.6 thousand cases in the 7MM which are expected to grow during the study period, i.e., 2018–2030.


The disease epidemiology covered in the report provides historical as well as forecasted Von Hippel-Lindau (VHL) Disease epidemiology


The report provides historical as well as forecasted prevalence of VHL disease [segmented as Total Prevalent Cases of Von Hippel-Lindau (VHL) Disease, Total Symptomatic/Diagnosed Cases of Von Hippel-Lindau (VHL) Disease, Total Clinical Manifestation-specific Cases of Von Hippel-Lindau (VHL) Disease, and Total Treated Cases of Von Hippel-Lindau (VHL) Disease] in the 7MM covering the United States, EU5 countries (Germany, France, Italy, Spain, and the United Kingdom), and Japan from 2018 to 2030.

Country Wise- Von Hippel-Lindau (VHL) Disease Epidemiology

Estimates show that in 2020, the highest cases of Von Hippel-Lindau (VHL) Disease in among the 7MM were in the United States, followed by Japan, Germany, the United Kingdom, France, Italy, and Spain.

  • In the United States, the total number of prevalent cases of VHL disease was 9.2 thousand cases in the year 2020 which are expected to grow during the study period, i.e., 2018–2030.
  • In the year 2020, the total prevalent cases of Von Hippel-Lindau (VHL) Disease were 9.3 thousand cases in EU-5 which are expected to grow during the study period, i.e., 2018–2030.
  • In Japan, the total number of prevalent cases of Von Hippel-Lindau (VHL) Disease was 3.0 thousand cases in the year 2020 which are expected to grow during the study period, i.e., 2018–2030.

Von Hippel-Lindau (VHL) Disease Drug Chapters

The drug chapter segment of the Von Hippel-Lindau (VHL) Disease report encloses the detailed analysis of Von Hippel-Lindau (VHL) Disease marketed drugs and clinical stage (Phase-III, Phase-II and Phase I) pipeline drugs. It also helps to understand the Von Hippel-Lindau (VHL) Disease clinical trial details, expressive pharmacological action, agreements and collaborations, approvals and patent details, advantages and disadvantages of each included drug, and the latest news and press releases.


Von Hippel-Lindau (VHL) Disease Emerging Drugs


Belzutifan/MK 6482 (Merck)

Belzutifan (MK-6482) is currently being developed by Merck, to treat patients with Von Hippel–Lindau (VHL) disease-associated RCC with nonmetastatic RCC tumors less than 3 cm in size, unless immediate surgery is required. Belzutifan is a novel, potent, and investigational hypoxia-inducible factor-2α (HIF-2α) inhibitor. HIF Proteins, including HIF-2α, can accumulate in patients when VHL protein, a tumor-suppressor protein, is inactivated. If not properly regulated, the accumulation of HIF-2α can stimulate several oncogenes associated with cellular proliferation, angiogenesis, and tumor growth, leading to the growth of both benign and malignant tumors. This inactivation of VHL has been observed in more than 90% of clear cell RCC tumors. Research into VHL biology that led to the discovery of HIF-2α was awarded the Nobel Prize in Physiology or Medicine in 2019.


DFF332 (Novartis)

DFF332 is a small molecule being developed by Novartis as a single agent and in combination in patients with advanced clear cell RCC and other malignancies with HIF stabilizing mutations. The development in combination includes Everolimus (RAD001, an mTOR inhibitor), and also in combination with Spartalizumab (PDR001, an anti-PD1) plus Taminadenant (NIR178, an adenosine A2A receptor antagonist). DFF332 targets a protein called HIF2α; by acting on HIF2α, DFF332 may stop the growth of certain types of cancer. Hypoxia inactivates prolyl hydroxylases, leading to a lack of HIF hydroxylation. Nonhydroxylated HIF1-alpha and HIF2-alpha are not bound to the VHL protein complex for ubiquitination, and therefore, accumulate. The resulting constitutively high levels of HIF1-alpha and HIF2-alpha drive increased transcription of various genes, including growth and angiogenic factors, enzymes of the intermediary metabolism, and genes promoting stemness-like cellular phenotypes.

Note: Detailed emerging therapies assessment will be provided in the final report.

Von Hippel-Lindau (VHL) Disease Market Outlook

VHL disease is caused by a flaw in one gene, the VHL gene, which regulates cell growth. This flaw, for which the cause is unknown, leads to the abnormal growth of blood vessels in certain parts of the body. Instead of growing and spreading out normally (like a tree), in patients with VHL, the blood vessels grow into clumps. VHL disease is an autosomal-dominant familial neoplastic condition caused by germline mutations in the VHL gene located on chromosome 3p25-26. Common VHL-associated clinical manifestations include central nervous system hemangioblastoma (CHB), renal cell carcinoma or renal cyst (RCC), retinal angioma (RA), pancreatic tumor or cyst (PCT), pheochromocytoma and paragangliomas (PHEO), endolymphatic sac tumor, and epididymis or broad ligament cystadenoma. Individuals with a family history of VHL will be clinically diagnosed when they present with VHL-associated tumors that include CHB, RA, or RCC.


Currently, there is no cure for VHL, but the associated tumors can be treated. Early detection and treatment of tumors significantly improve a patient’s diagnosis. Left untreated, VHL may result in blindness, permanent brain damage, or death. For patients who have VHL disease and all patients who have hereditary cancer syndromes, the goal of treatment is cancer control, not to cure cancer, and preservation of functional parenchyma to avoid the morbidity associated with renal or adrenal loss. Retinal angiomas may be treated by laser or by freezing. Hemangioblastomas in the cerebellum or spine are usually removed through surgery if they cause symptoms. Renal cysts do not need treatment, but if a tumor or pheochromocytoma is detected, they will be surgically removed.


Specific pharmacologic treatment awaits further biologic understanding of critical pathogenic components. As per the study, Katherine Sims (2001) identified TGF-beta as a pVHL target gene under post-transcriptional regulation and inhibition of tumorigenesis in vivo (RCC) by TGF-beta neutralizing antibodies. Also, de Paulsen et al. (2001) documented increased oncogenic expression of TGF-alpha in VHL RCC cells and showed decreased cell proliferation using pharmacologic TGF-alpha receptor inhibition.


Much of the preclinical data that form the basis for current systemic treatment strategies stem from the study of VHL alteration. All the large randomized trials investigating aldesleukin, vascular endothelial growth factor (VEGF) receptor tyrosine kinase inhibitors, mTOR inhibitors, and checkpoint inhibitors are based on data from the treatment of sporadic clear cell kidney cancer. Despite limited studies investigating these agents in the VHL population with metastatic kidney cancer, they are believed to be efficacious and are available as treatment options.


Recently, some developmental initiatives have been taken toward the management of VHL disease. As some interesting therapies like the HIF2α inhibitors are heading down the pipeline, the condition may be evolving. At present, a few companies have initiated clinical trials that are investigating new treatment options. Key players such as Merck (Belzutifan) and Novartis (DFF332) are investigating their candidates to manage VHL disease in the 7MM.


Key Findings

The Von Hippel-Lindau (VHL) Disease market size in the 7MM is expected to change during the study period 2018–2030. The therapeutic market of Von Hippel-Lindau (VHL) Disease in the seven major markets is expected to increase during the study period (2018–2030) with a CAGR of 8.3%. According to the estimates, the highest market size of Von Hippel-Lindau (VHL) Disease is found in the United States followed by Japan.


The United States Market Outlook

The total market size of Von Hippel-Lindau (VHL) Disease therapies in the United States is expected to increase with a CAGR of 9.0% in the study period (2018–2030).


EU-5 Countries: Market Outlook

The total market size of Von Hippel-Lindau (VHL) Disease therapies in EU-5 countries is expected to increase with a CAGR of 7.4% in the study period (2018–2030).


Japan Market Outlook

The total market size of Von Hippel-Lindau (VHL) Disease therapies in Japan is expected to increase with a CAGR of 5.2% in the study period (2018–2030).

Von Hippel-Lindau (VHL) Disease Pipeline Development Activities

The drugs which are in pipeline include:

  • Belzutifan/MK 6482 (Merck)
  • DFF332 (Novartis)


Note: Detailed emerging therapies assessment will be provided in the final report.

Analyst Commentary

  • Due to lack of approved treatment options in VHL, currently surgery is the preferred choice of treatment. Entry of belzutifan (MK-6482) is expected to impact the VHL market dynamics significantly.
  • Limited pipeline activity in the VHL space can be observed and the possible hurdles might be rarity of disorder, data scarcity on the latest epidemiology studies across the geographies and limited understanding about the VHL pathophysiology.
  • Among the emerging therapies, Merck’s belzutifan (MK-6482) appears to be the only drug which has the potential to transform the VHL on market owing to impressive clinical data. Merck has filed the New Drug Application (NDA) for the belzutifan (MK-6482) for the potential treatment of patients with VHL disease-associated renal cell carcinoma (RCC), not requiring immediate surgery. Owing to impressive clinical data, expected monopoly in near future, drug is expected to generate a huge revenue in the 7MM by 2030 from the VHL associated RCC. Company is also trying to expand the belzutifan (MK-6482) in other tumor types as well and this might provide additional opportunities.
  • Novartis with DFF332 is trying to enter into the VHL space and planned a phase I trial for the same. Due to lack of clinical evidences, it is too early to predict the drug’s potential in VHL market.

Access and Reimbursement Scenario in Von Hippel-Lindau (VHL) Disease Therapies

Since there are no approved pharmacological treatment options for VHL disease, surgical removal of the tumor from the organ site is the only way available for the patients. But when surgery is no longer an option and the tumors have advanced or become metastatic, some tumor inhibitors may be given for any relief. Therefore, treatment of VHL associated tumors such as VHL-RCC (Renal Cell Carcinoma) can be similar or comparable.


The French National Authority for Health, HAS recommends some drugs for the RCC, such as Sutent (Sunitinib) for having substantial/significant clinical benefit for the treatment of advanced and/or metastatic kidney cancer. Also, Avastin has been given an opinion of significant clinical interest without demonstrated clinical benefit in combination with interferon in the first-line management of advanced and/or metastatic renal cell carcinoma, in the absence of data versus clinically relevant comparators (sunitinib and pazotinib). HAS also has a favorable opinion for reimbursement of Keytruda (Pembrolizumab) in combination with Axitinib in the first-line treatment of advanced purely clear-cell renal cell carcinoma (RCC) or with a clear-cell component. The clinical benefit of Keytruda in combination with Axitinib was also found to be substantial in the first-line treatment of advanced purely clear-cell renal cell carcinoma (RCC) or with a clear-cell component.


As shown previously, there is a similarity in RCC and VHL associated RCC treatment areas, therefore the reimbursement scenario of drugs approved in RCC (such as bevacizumab, axitinib, etc.) can be used as comparison to assume that any new drug when approved will receive a similar reimbursement depending on the HTA assessments it can possibly attain.


Bevacizumab was assessed by the National Institute for Health and Care Excellence (NICE) in England. On the basis of unproven cost-effectiveness of the treatment, NICE gave a negative reimbursement decision. Similarly, Sunitinib is a multitargeted TKI with antitumour and antiangiogenic activities. It received a positive reimbursement decision in England, Germany and some other European countries, albeit with some restrictions in some cases. Axitinib is a more recent addition to treatments for mRCC and positive reimbursement decisions were awarded to the drug in England, France, and Germany. Nivolumab received positive reimbursement decisions in England, France and Germany. Moreover, in June 2017, cabozantinib had only been assessed by the HAS and IQWiG in France and Germany, respectively. Both HTA bodies had granted positive reimbursement decisions.


As per the study by, Hrishikesh Kale (2018) which included 10,392 cases each of RCC and control patients. The average annual THC associated with RCC was USD 7,419 million for all phases, USD 22,752 million for initial phase, USD 4,860 million for continuing phase and USD 13,232 million for the late phase of care. The average Total Healthcare Cost (THC) was USD 4,584 million for patients diagnosed at stage-I, USD 4,727 million for stage-II, USD 9,331 million for stage-III, and USD 31,637 million for stage-IV. The annual economic burden of RCC on Medicare was estimated to be USD 1.51 billion.


As per the study conducted in France by Maroun et al. (2017), estimated per patient per month (PPPM) costs for mRCC patients receiving targeted therapies as first-line treatment and reported that costs of targeted oral agents accounted for more than 50% of the estimated total PPPM cost of Euro 5,546 million (USD 6,944 million). The estimated lifetime costs with IFNα was Euro 32,951 million (USD 41,253 million) and the addition of sunitinib was projected to raise the 5-year national budget from Euro 26 million (USD 33 million) to Euro 41M (USD 51 million).

Note: Detailed HTA assessment will be provided in the final report.

KOL-Views

To keep up with current market trends, we take KOLs and SME’s opinion working in the Von Hippel-Lindau (VHL) Disease domain through primary research to fill the data gaps and validate our secondary research. Their opinion helps to understand and validate current and emerging therapies treatment patterns or Von Hippel-Lindau (VHL) Disease market trends. This will support the clients in potential upcoming novel treatment by identifying the overall scenario of the market and the unmet needs.

Competitive Intelligence Analysis

We perform Competitive and Market Intelligence analysis of the Von Hippel-Lindau (VHL) Disease Market by using various Competitive Intelligence tools that includes – SWOT analysis, PESTLE analysis, Porter’s five forces, BCG Matrix, Market entry strategies, etc. The inclusion of the analysis entirely depends upon the data availability.

Scope of the Report

  • The report covers the descriptive overview of Von Hippel-Lindau (VHL) Disease, explaining its causes, signs and symptoms, pathophysiology, diagnosis, and currently available therapies.
  • Comprehensive insight has been provided into the Von Hippel-Lindau (VHL) Disease epidemiology and treatment in the 7MM.
  • Additionally, an all-inclusive account of both the current and emerging therapies for Von Hippel-Lindau (VHL) Disease is provided, along with the assessment of new therapies, which will have an impact on the current treatment landscape.
  • A detailed review of the historical and forecasted Von Hippel-Lindau (VHL) Disease market is included in the report, covering drug outreach in the 7MM.
  • The report provides an edge while developing business strategies, by understanding trends shaping and driving the global Von Hippel-Lindau (VHL) Disease market.

Report Highlights

  • In the coming years, the Von Hippel-Lindau (VHL) Disease market is set to change due to the upcoming therapies with a novel mechanism of action which are under investigation; which would expand the size of the market to enable the drug manufacturers to penetrate more into the market.
  • The companies and academics are working to assess challenges and seek opportunities that could influence Von Hippel-Lindau (VHL) Disease R&D. The therapies under development are focused on novel approaches to treat/improve the disease condition.
  • Major players are involved in developing therapies for Von Hippel-Lindau (VHL) Disease. The launch of emerging therapies will significantly impact the Von Hippel-Lindau (VHL) Disease market.
  • A better understanding of disease pathogenesis will also contribute to the development of novel therapeutics for Von Hippel-Lindau (VHL) Disease.
  • Our in-depth analysis of the pipeline assets across different stages of development (Phase III to Phase I), different emerging trends, and comparative analysis of pipeline products with detailed clinical profiles, key cross-competition, launch date along with product development activities will support the clients in the decision-making process regarding their therapeutic portfolio by identifying the overall scenario of the research and development activities.

Von Hippel-Lindau (VHL) Disease Report Insights

  • Patient Population
  • Therapeutic Approaches
  • Von Hippel-Lindau (VHL) Disease Pipeline Analysis
  • Von Hippel-Lindau (VHL) Disease Market Size and Trends
  • Market Opportunities
  • Impact of upcoming Therapies

Von Hippel-Lindau (VHL) Disease Report Key Strengths

  • 10 Years Forecast
  • 7MM Coverage
  • Von Hippel-Lindau (VHL) Disease Epidemiology Segmentation
  • Key Cross Competition
  • Highly Analyzed Market
  • Drugs Uptake

Von Hippel-Lindau (VHL) Disease Report Assessment

  • SWOT Analysis
  • Current Treatment Practices
  • Unmet Needs
  • Pipeline Product Profiles
  • Conjoint Analysis
  • Market Attractiveness
  • Market Drivers and Barriers

Key Questions

Market Insights:

  • What was the Von Hippel-Lindau (VHL) Disease Market share (%) distribution in 2018 and how it would look like in 2030?
  • What would be the Von Hippel-Lindau (VHL) Disease total market size as well as market size by therapies across the 7MM during the study period (2018–2030)?
  • What are the key findings of the market across the 7MM and which country will have the largest Von Hippel-Lindau (VHL) Disease market size during the study period (2018–2030)?
  • At what CAGR, the Von Hippel-Lindau (VHL) Disease market is expected to grow in the 7MM during the study period (2018–2030)?
  • What would be the Von Hippel-Lindau (VHL) Disease market outlook across the 7MM during the study period (2018–2030)?
  • What would be the Von Hippel-Lindau (VHL) Disease market growth till 2030 and what will be the resultant market size in the year 2030?
  • How would the market drivers, barriers, and future opportunities affect the market dynamics and subsequent analysis of the associated trends?
  • Von Hippel-Lindau (VHL) Disease patient types/pool where unmet need is more and whether emerging therapies will be able to address the residual unmet need?
  • How emerging therapies are performing on the parameters like efficacy, safety, route of administration (RoA), treatment duration, and frequencies based on their clinical trial results?
  • Among the emerging therapies, what are the potential therapies which are expected to disrupt the Von Hippel-Lindau (VHL) Disease market?


Epidemiology Insights:

  • What are the disease risks, burdens, and unmet needs of the Von Hippel-Lindau (VHL) Disease?
  • What is the historical Von Hippel-Lindau (VHL) Disease patient pool in the 7MM covering the United States, EU5 (Germany, France, Italy, Spain, and the United Kingdom), and Japan?
  • What would be the forecasted patient pool of Von Hippel-Lindau (VHL) Disease in the 7MM covering the United States, EU5 (Germany, France, Italy, Spain, and the United Kingdom), and Japan?
  • What will be the growth opportunities in the 7MM concerning the patient population about Von Hippel-Lindau (VHL) Disease?
  • Out of all the 7MM countries, which country would have the highest prevalent population of Von Hippel-Lindau (VHL) Disease during the study period (2018–2030)?
  • At what CAGR the population is expected to grow in the 7MM during the study period (2018–2030)?
  • What are the various recent and upcoming events which are expected to improve the diagnosis of Von Hippel-Lindau (VHL) Disease?


Current Treatment Scenario and Emerging Therapies:

  • What are the current options for the treatment of Von Hippel-Lindau (VHL) Disease?
  • What are the current treatment guidelines for the treatment of Von Hippel-Lindau (VHL) Disease in the US, Europe, and Japan?
  • How many companies are developing therapies for the treatment of Von Hippel-Lindau (VHL) Disease?
  • How many therapies are developed by each company for the treatment of Von Hippel-Lindau (VHL) Disease?
  • How many emerging therapies are in the mid-stage and late stages of development for the treatment of Von Hippel-Lindau (VHL) Disease?
  • What are the key collaborations (Industry–Industry, Industry-Academia), Mergers and acquisitions, licensing activities related to the Von Hippel-Lindau (VHL) Disease therapies?
  • What are the recent novel therapies, targets, mechanisms of action, and technologies developed to overcome the limitation of existing therapies?
  • What are the clinical studies going on for Von Hippel-Lindau (VHL) Disease and their status?
  • What are the key designations that have been granted for the emerging therapies for Von Hippel-Lindau (VHL) Disease?
  • What is the global historical and forecasted market of Von Hippel-Lindau (VHL) Disease?

Reasons to buy

  • The report will help in developing business strategies by understanding trends shaping and driving the Von Hippel-Lindau (VHL) Disease market.
  • To understand the future market competition in the Von Hippel-Lindau (VHL) Disease market and Insightful review of the key market drivers and barriers.
  • Organize sales and marketing efforts by identifying the best opportunities for Von Hippel-Lindau (VHL) Disease in the US, Europe (Germany, France, Italy, Spain, and the United Kingdom), and Japan.
  • Identification of strong upcoming players in the market will help in devising strategies that will help in getting ahead of competitors.
  • Organize sales and marketing efforts by identifying the best opportunities for the Von Hippel-Lindau (VHL) Disease market.
  • To understand the future market competition in the Von Hippel-Lindau (VHL) Disease market.

1. Key Insights

2. Report Introduction

3. Von Hippel-Lindau (VHL) disease Market Overview at a Glance

3.1. Market Share (%) Distribution of VHL disease in 2018

3.2. Market Share (%) Distribution of VHL disease in 2030

4. Executive Summary of Von Hippel-Lindau (VHL) disease

4.1. Key Events

5. Epidemiology and Market Methodology

6. Disease Background and Overview

6.1. Introduction

6.2. Clinical Manifestations of VHL

6.2.1. Central Nervous System Hemangioblastomas

6.2.2. Renal Cell Carcinomas and Renal Cysts

6.2.3. Pheochromocytoma

6.2.4. Retinal Hemangioblastomas

6.2.5. Papillary Cystadenomas

6.2.6. Epididymal Cystadenomas

6.2.7. Pancreatic Neuroendocrine Tumors and Cysts

6.2.8. Endolymphatic Sac Tumors

6.3. Signs and Symptoms of VHL

6.4. Risk Factors and Causes of VHL

6.4.1. Genetics of VHL

6.5. Classification of VHL

6.6. Pathophysiology of VHL

6.7. Complications of VHL

7. Diagnosis of VHL

7.1. Screening of VHL

7.2. Genetic Testing

7.3. Imaging Tests

7.4. Laboratory Tests

7.5. Differential diagnosis

8. Diagnostic Guidelines

8.1. VHL Alliance Active Surveillance Guidelines

8.2. VHL disease Clinical practice guidelines 2017 edition

9. Current Treatment Practices of VHL

9.1. Surgical Treatment

9.1.1. Central Nervous System Hemangioblastomas

9.1.2. Retinal Capillary Hemangioblastomas

9.1.3. Renal Cell Carcinoma

9.1.4. Pancreatic Neuroendocrine Tumors and Cysts

9.1.5. Pheochromocytoma

9.1.6. Endolymphatic Sac Tumors

10. Treatment Guidelines

10.1. The Japanese VHL Study Group Treatment Guidelines

10.2. VHL disease Clinical practice guidelines 2017 edition

11. Epidemiology and Patient Population

11.1. Key Findings

11.2. Epidemiology of Von Hippel-Lindau (VHL) disease

11.3. Epidemiology Scenario

11.3.1. Total Prevalent Cases of VHL disease

11.3.2. Total Symptomatic/ Diagnosed Cases of VHL disease

11.3.3. Total Clinical Manifestation-specific Cases of VHL disease

11.3.4. Total Treated Cases of VHL disease

12. Patient Journey

13. Key Endpoints in Von Hippel–Lindau (VHL) Disease Clinical Trials

14. Emerging Therapies

14.1. Belzutifan (MK 6482; previously known as PT-2977): Peloton Therapeutics (acquired by Merck)

14.1.1. Product Description

14.1.2. Other Developmental Activities

14.1.3. Clinical Development

14.1.4. Safety and Efficacy

14.2. DFF332: Novartis

14.2.1. Product Description

14.2.2. Clinical Development

15. Von Hippel–Lindau Disease: 7 Major Market Analysis

15.1. Key Findings

15.2. Market Outlook

15.3. Market Size of VHL

15.3.1. Total Market Size of VHL

15.3.2. Market Size of VHL by Therapies

16. Market Access and Reimbursement

16.1. Reimbursement

17. KOL Views

18. Market Drivers

19. Market Barriers

20. SWOT Analysis

21. Unmet Needs

22. Appendix

22.1. Bibliography

22.2. Report Methodology

23. DelveInsight Capabilities

24. Disclaimer

25. About DelveInsight

List of Table

Table 1: Summary of Von Hippel-Lindau (VHL) disease, Market, Epidemiology, and Key Events (2018–2030)

Table 2: Key Classifications of VHL

Table 3: Cambridge Protocol for Screening Patients With VHL Disease

Table 4: Imaging Features of VHL-related Benign and Malignant Tumors

Table 5: VHL Alliance Active Surveillance Guidelines

Table 6: Management of Major Manifestations of VHL Disease

Table 7: Total Prevalent Cases of VHL disease (2018–2030)

Table 8: Total Symptomatic/ Diagnosed Cases of VHL disease (2018–2030)

Table 9: Total Clinical manifestation-specific Cases of VHL disease (2018–2030)

Table 10: Total Treated Cases of VHL disease (2018–2030)

Table 11: Belzutifan (MK 6482; previously known as PT-2977), Clinical Trial Description, 2021

Table 12: DFF332, Clinical Trial Description, 2021

Table 13: 7MM Market Size of VHL in USD Million (2018–2030)

Table 14: 7MM Market Size of VHL by Therapies in USD Million (2018–2030)

List of Figures

Figure 1: Epidemiology and Market Methodology

Figure 2: Tumors in VHL Disease

Figure 3: Inheritance Pattern of VHL

Figure 4: Mechanism of Action of VHL Protein

Figure 5: Total Prevalent Cases of VHL disease in the 7MM (2018–2030)

Figure 6: Total Symptomatic/ Diagnosed Cases of VHL disease in the 7MM (2018–2030)

Figure 7: Total Clinical Manifestation-specific Cases of VHL disease by country (2018)

Figure 8: Total Treated Cases of VHL disease in the 7MM (2018–2030)

Figure 9: Market Size of VHL in the 7MM, USD Million (2018–2030)

Figure 10: 7MM Market Size of VHL by Therapies in USD Million (2018–2030)

• Peloton Therapeutics (acquired by Merck)

• Novartis

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