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Belzutifan for Renal Cell Carcinoma in von Hippel–Lindau Disease
Open accessAbstract
BACKGROUND: gene inactivation and constitutive activation of the transcription factor hypoxia-inducible factor 2α (HIF-2α). METHODS: In this phase 2, open-label, single-group trial, we investigated the efficacy and safety of the HIF-2α inhibitor belzutifan (MK-6482, previously called PT2977), administered orally at a dose of 120 mg daily, in patients with renal cell carcinoma associated with VHL disease. The primary end point was objective response (complete or partial response) as measured according to the Response Evaluation Criteria in Solid Tumors, version 1.1, by an independent central radiology review committee. We also assessed responses to belzutifan in patients with non-renal cell carcinoma neoplasms and the safety of belzutifan. RESULTS: After a median follow-up of 21.8 months (range, 20.2 to 30.1), the percentage of patients with renal cell carcinoma who had an objective response was 49% (95% confidence interval, 36 to 62). Responses were also observed in patients with pancreatic lesions (47 of 61 patients [77%]) and central nervous system hemangioblastomas (15 of 50 patients [30%]). Among the 16 eyes that could be evaluated in 12 patients with retinal hemangioblastomas at baseline, all (100%) were graded as showing improvement. The most common adverse events were anemia (in 90% of the patients) and fatigue (in 66%). Seven patients discontinued treatment: four patients voluntarily discontinued, one discontinued owing to a treatment-related adverse event (grade 1 dizziness), one discontinued because of disease progression as assessed by the investigator, and one patient died (of acute toxic effects of fentanyl). CONCLUSIONS: Belzutifan was associated with predominantly grade 1 and 2 adverse events and showed activity in patients with renal cell carcinomas and non-renal cell carcinoma neoplasms associated with VHL disease. (Funded by Merck Sharp and Dohme and others; MK-6482-004 ClinicalTrials.gov number, NCT03401788.).
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“PT2977/belzutifan was recently approved by the FDA for the treatment of RCC in patients with von Hippel-Lindau syndrome (47, 48).”
From HIF inhibitor 32-134D eradicates murine hepatocellular carcinoma in combination with anti-PD1 therapy (2022) · cites this paper as [48]
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“The most frequent on-target toxicity with HIF-2 inhibitors (PT2385; belzutifan) is anemia from suppression of renal/hepatic erythropoietin.”
From HIF inhibition: Current strategies and clinical challenges (2026) · cites this paper as [421]
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“Anemia occurred commonly in early trials and, during prolonged belzutifan therapy, occasionally required transfusion or erythropoiesis-stimulating agents, hence the need for routine hematological monitoring [37,412,421].”
From HIF inhibition: Current strategies and clinical challenges (2026) · cites this paper as [421]
In the citing paper
“VHL gene mutations lead to the stability of HIF subunits and the constitutive activation of HIF‐mediated transcriptional pathways in patients with VHL, causing the progression of renal cell carcinoma.”
From Hypoxia signaling in cancer: Implications for therapeutic interventions (2023) · cites this paper as [24]
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Citations
Cites
von Hippel–Lindau disease: A clinical and scientific review
Maher, E. R., Neumann, H. P. H., & Richard, S. (2011) · European Journal of Human Genetics · doi:10.1038/ejhg.2010.175 · Cited by 744
Targeting renal cell carcinoma with a HIF-2 antagonist
Chen, W., Hill, H., Christie, A., Kim, M. S., Holloman, E., Pavia-Jimenez, A., Homayoun, F., Ma, Y., Patel, N., Yell, P., et al. (2016) · Nature · doi:10.1038/nature19796 · Cited by 709
Hypoxia-inducible factor-2 (HIF-2) regulates hepatic erythropoietin in vivo
Rankin, E. B., Biju, M. P., Liu, Q., Unger, T. L., Rha, J., Johnson, R. S., Simon, M. C., Keith, B., & Haase, V. H. (2007) · Journal of Clinical Investigation · doi:10.1172/jci30117 · Cited by 596
Targeting the HIF2–VEGF axis in renal cell carcinoma
Choueiri, T. K., & Kaelin, W. G. (2020) · Nature Medicine · doi:10.1038/s41591-020-1093-z · Cited by 477 · cited 1×
“Upper respiratory tract infection 11 (18) 4 (7) 7 (11) 0”
On-target efficacy of a HIF-2α antagonist in preclinical kidney cancer models
Cho, H., Du, X., Rizzi, J. P., Liberzon, E., Chakraborty, A. A., Gao, W., Carvo, I., Signoretti, S., Bruick, R. K., Josey, J. A., et al. (2016) · Nature · doi:10.1038/nature19795 · Cited by 451 · cited 1×
“Alanine aminotransferase level increase 10 (16) 10 (16) 0 0”
BackgroundCourtney, K. D., Infante, J. R., Lam, E. T., Figlin, R. A., Rini, B. I., Brugarolas, J., Zojwalla, N. J., Lowe, A. M., Wang, K., Wallace, E. M., et al. (2018) · Journal of Clinical Oncology · doi:10.1200/jco.2017.74.2627 · Cited by 393
Choueiri, T. K., Bauer, T. M., Papadopoulos, K. P., Plimack, E. R., Merchan, J. R., McDermott, D. F., Michaelson, M. D., Appleman, L. J., Thamake, S., Perini, R. F., et al. (2021) · Nature Medicine · doi:10.1038/s41591-021-01324-7 · Cited by 305 · cited 1×
“Upper respiratory tract infection 11 (18) 4 (7) 7 (11) 0”
Self-citationPharmacologic Targeting of Hypoxia-Inducible Factors
Semenza, G. L. (2019) · Annual Review of Pharmacology and Toxicology · doi:10.1146/annurev-pharmtox-010818-021637 · Cited by 288
HIF-2α regulates murine hematopoietic development in an erythropoietin-dependent manner
Scortegagna, M., Ding, K., Zhang, Q., Oktay, Y., Bennett, M. J., Bennett, M., Shelton, J. M., Richardson, J. A., Moe, O. W., & Garcia, J. A. (2004) · Blood · doi:10.1182/blood-2004-05-1695 · Cited by 237
Courtney, K. D., Ma, Y., Diaz de Leon, A., Christie, A., Xie, Z., Woolford, L., Singla, N., Joyce, A., Hill, H., Madhuranthakam, A. J., et al. (2020) · Clinical Cancer Research · doi:10.1158/1078-0432.ccr-19-1459 · Cited by 188
Flagged first, then by citations
Cited by
Sorted by citations
Items 501–600 of 717
Structure-guided optimization of SLC1A1/EAAT3-selective inhibitors targeting renal cancer metabolism
Koochaki, P., Qiu, B., Coker, J. A., Earsley, A., Wang, N. S., Romigh, T., Goins, C. M., Salem, C., Mi, D., Days, E., et al. (2026) · The EMBO Journal · doi:10.1038/s44318-026-00776-2 · Cited by 1
“Pooneh Koochaki et al functions as a critical oncogenic driver in ccRCC (Gordan et al, 2008; Kondo et al, 2003), and pharmacological approaches to target this protein are now FDA approved for use in ccRCC patients (Chen et al, 2016; Cho et al, 2016; Jonasch et al, 2021; Wallace et al, 2016).”
Renal Cell Carcinoma: Histopathology, Molecular Features, and the Emerging Role of Organoid Models
Coppola, A. K., Shahini, A., Mehrjoo, Z., Tarikere, S., Tanguay, S., & Riazalhosseini, Y. (2026) · Gene · doi:10.1016/j.gene.2026.150216 · Cited by 1
He, H., Liu, X.-D., & Jonasch, E. W. (2026) · MedScience · doi:10.1007/s11684-025-1136-4 · Cited by 1
Self-citationVon Hippel-Lindau disease: pathophysiological and clinical advances
van Alfen, S. J., van der Tuin, K., van de Kooij, B., Links, T. P., & Zandee, W. T. (2026) · Familial Cancer · doi:10.1007/s10689-026-00579-8 · Cited by 1
Hypoxia-driven tumor immune escape: mechanisms and therapeutic opportunities
Qin, H., 杨书强, He, J., Zhao, M., Zhao, L., Wang, J., Jiang, X., Chen, X., & Xu, X. (2026) · Frontiers in Immunology · doi:10.3389/fimmu.2026.1893099 · Cited by 1
“HIF-2 a inhibitors, such as belzutifan, have demonstrated clinical activity in HIF-2 a -driven tumors and provide proof-of-concept that hypoxia-response pathways can be therapeutically targeted in patients (72).”
So, E., Morizane, C., & Okusaka, T. (2026) · Therapeutic Advances in Medical Oncology · doi:10.1177/17588359261471127 · Cited by 1
“107,108 Belzutifan is a therapeutic option for VHL-associated pNETs (Figure 2).”
Jilg, C. A., Astheimer, S., Kotsis, F., Ganner, A., Schultze-Seemann, W., Neumann, H. P., Grabbert, M. T., Spohn, S. K. B., Kirste, S., Gratzke, C., et al. (2026) · European Urology Focus · doi:10.1016/j.euf.2026.07.002 · Cited by 1
Mathews, P., Neiditz, H., Hollenbeck, P., Rajkumar, J., Schoen, M. W., Pachynski, R. K., & Knoche, E. M. (2026) · Clinical Genitourinary Cancer · doi:10.1016/j.clgc.2026.102645 · Cited by 1
Non-canonical approaches to targeting hypoxic tumors.
Zhao, S., & El‐Deiry, W. S. (2022) · PubMed · Cited by 1
Updates in neuroendocrine neoplasms: From mechanisms to the clinic
Fodil-Cherif, S., Desailloud, R., & Brue, T. C. (2023) · Annales d Endocrinologie · doi:10.1016/j.ando.2022.12.424 · Cited by 1
Von Hippel-Lindau; A 47-year-old woman with renal cell carcinoma and multiorgan involvement
Tarokhian, A., Dehghan, A., Kulivand, S., & Borzouei, S. (2022) · Journal of Renal Endocrinology · doi:10.34172/jre.2022.25065 · Cited by 1
Promising targetable biomarkers in pancreatic neuroendocrine tumours
Borghesani, M., Gervaso, L., Cella, C. A., Benini, L., Ciardiello, D., Algeri, L., Ferrero, A., Valenza, C., Guidi, L., Zampino, M. G., et al. (2023) · Expert Review of Endocrinology & Metabolism · doi:10.1080/17446651.2023.2248239 · Cited by 1
“%) and the others VHL-associated solid tumors, in detail 77% of PanNET achieved objective response; duration of response was sustained as well, as all responders hadn’t progressed at date of data cutoff, with a median follow up of almost 22 months [72].”
The emerging genetic landscape of renal cell carcinoma
Lee, G., Shinder, B. M., Sadimin, E. T., Stephenson, R. D., & Singer, E. A. (2023) · Elsevier eBooks · doi:10.1016/b978-0-12-822824-1.00035-3 · Cited by 1
CADTH (2023) · Canadian Journal of Health Technologies · doi:10.51731/cjht.2023.782 · Cited by 1
Schwartz, A. M., Fakhre, W., Jin, K., Bollich, V., Ahmadzadeh, S., Shekoohi, S., & Kaye, A. D. (2023) · Drugs & Therapy Perspectives · doi:10.1007/s40267-023-01031-y · Cited by 1
“Seven patients discontinued treatment, with the median duration of treat-ment being 8.1 months in those who discontinued [24].”
BackgroundInfluentialSomatostatin receptor 2A expression in von Hippel‐Lindau–related hemangioblastomas
Ahmad, S., Mühlebner, A., Snijders, T. J., de Leng, W. W. J., Seute, T., & van Leeuwaarde, R. S. (2024) · Cancer · doi:10.1002/cncr.35418 · Cited by 1
“22 These results seem promising and should be further investigated in prospective cohort studies.”
Meng, H., Cao, S., Tian, S., Huo, J., Li, X., Xu, D., & Liu, L. (2024) · Cancer Cell International · doi:10.1186/s12935-024-03442-x · Cited by 1
“An inhibitor of HIF has recently been approved for the treatment of renal cell carcinoma [27].”
BackgroundTan, D. T. H., Fujiwara, R. J. T., Tan, C., Isaacson, B. B., & Hunter, J. B. (2024) · Otology & Neurotology · doi:10.1097/mao.0000000000004307 · Cited by 1
Von Hippel Lindau syndrome: A systematic review of pharmaceutical treatments
Fortuna, G. G., Özay, Z. İ., Chehade, C. H., Gebrael, G., Li, H., & Maughan, B. L. (2024) · Kidney Cancer · doi:10.1177/24684570241292245 · Cited by 1
“12 Given that surgical interventions are a signi fi cant source of morbidity and mortality in patients with VHL, delaying surgical interventions is of utmost importance in this population.”
Self-citationShi, C., Lin, J.-J., Xiang, X., Bao, W., & Yang, J. (2025) · Frontiers in Oncology · doi:10.3389/fonc.2025.1546217 · Cited by 1
“…cancer and kidney diseases such as MN, MCD, and IgA nephropathy (7, 8), but few studies have reported the association between pNETs and glomerular diseases or the related pathological mechanisms, it may manifest with RCC and pNETs in patients with von Hippel - Lindau (VHL) disease (11, 12).”
Larcher, A., Umberto Capitanio, A., Necchi, A., Montorsi, F., & Salonia, A. (2025) · The Lancet Oncology · doi:10.1016/s1470-2045(25)00295-5 · Cited by 1
Is HIF activation Janus faced? The therapeutic effects of the HIF-2α inhibitor
Ishii, T., Tanaka, T., & Nangaku, M. (2022) · Kidney International · doi:10.1016/j.kint.2022.01.023 · Cited by 1
Kaelin, W. G. (2022) · PubMed Central · Cited by 1
Update on systemic therapy for clear cell renal cell carcinoma
Mühlmann, J. (2023) · memo - Magazine of European Medical Oncology · doi:10.1007/s12254-022-00867-w · Cited by 1
“The most advanced is belzutifan, which achieved response rates of 49% and 25%, respectively, in RCCs in autosomal dominant inherited Hippel-Lindau disease and in sporadic RCCs pretreated with a median of three treatment lines [16, 17].”
BackgroundManagement of Toxicity and Side Effects of Systemic Therapy for Renal Cell Carcinoma
Young, K., Schmitt, A. M., Mukherji, D., Spain, L. A., Schmidinger, M., & Pickering, L. M. (2022) · Société Internationale d’Urologie Journal · doi:10.48083/syab9165 · Cited by 1
Hereditary Renal Cell Carcinoma Syndromes
Maranchie, J. K., Schuch, B., Bratslavsky, G., & Maher, E. R. (2022) · Société Internationale d’Urologie Journal · doi:10.48083/cbto5325 · Cited by 1
Self-citationNon-Functioning Pancreatic Neuroendocrine Tumors
Fazio, N., & Koumarianou, A. A. (2024) · Springer eBooks · doi:10.1007/978-3-031-56968-5_18 · Cited by 1
Re: Belzutifan for Renal Cell Carcinoma in von Hippel–Lindau Disease
Bensalah, K., Khene, Z., & Massard, C. (2022) · European Urology · doi:10.1016/j.eururo.2022.01.014 · Cited by 1
Yuan, G., Peng, Y., Reddy, V. D., Makarenko, V. V., Nanduri, J., Khan, S. A., Garcia, J. A., Kumar, G. K., Semenza, G. L., & Prabhakar, N. R. (2022) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2022.07.11.499380 · Cited by 1
Gardi, M., Basso, U., Ferrara, A. M., Zovato, S., Feletti, A., Watutantrige‐Fernando, S., Moro, F. D., Opocher, G., & the VHL–Padova Network (2022) · Cancer · doi:10.1002/cncr.34460 · Cited by 1
Strowd, R. E., III (2023) · Neuro-Oncology · doi:10.1093/neuonc/noad049 · Cited by 1
Zhang, X., Li, S., He, J., Jin, Y., Zhang, R., Dong, W., Lin, M., Yang, Y., Tian, T., Zhou, Y., et al. (2023) · doi:10.1158/0008-5472.c.6513850.v1 · Cited by 1
Chan, L., Tsang, S. H., Sharma, T., & Diaconita, V. (2025) · Advances in experimental medicine and biology · doi:10.1007/978-3-031-72230-1_44 · Cited by 1
External control arms can validate treatment efficacy in single-arm studies
Miller, E. J., Atiq, S., Anker, J. F., & Tsao, C.-K. (2026) · JNCI Journal of the National Cancer Institute · doi:10.1093/jnci/djag120 · Cited by 1
Rescuing the Function of Missense-Mutated Tumor Suppressor VHL using Stabilizing Small MoleculesPreprint
Fouad, M. A., Parry, C. S., Miller, S. A., Malhotra, S., Doyle, G. A. R., Shimabenga, E., Nurilloeva, M., Bondarenko, E., Andrianov, G. V., Childers, W. E., et al. (2026) · eLife · doi:10.7554/elife.110305.2 · Cited by 1
Changing the Course of an Orphan Disease
Schmidinger, M. (2021) · New England Journal of Medicine · doi:10.1056/nejme2114846
Mandal, S. (2022) · Indian Journal of Urology · doi:10.4103/iju.iju_460_21
“Belzutifan is an oral HIF‐2‐alpha inhibitor studied at a dose of 120 mg daily in a phase‐2, open‐label, single‐group, multicentric trial, enrolling 61 patients.[5] Patients were 18 years and older with germline VHL mutation and at least one measurable RCC 1–3 cm with no metastatic disease with ECOG of 0 or 1.”
BackgroundKidney Cancer Research Highlights from ASCO GU 2022 Meeting
Ged, Y. M. A., & Singla, N. (2022) · Kidney Cancer Journal · doi:10.52733/kcj20n1-gu1
Practice-changing publications: Kidney cancer
Lavallée, L. T., & Kapoor, A. (2022) · Canadian Urological Association Journal · doi:10.5489/cuaj.7862
Hereditary Renal Cancer Predisposition Syndromes
Shepherd, S. T. C., & Turajlic, S. (2022) · Springer eBooks · doi:10.1007/978-3-030-84756-2_3
Advances in Targeted Therapy for Patients with Neuroendocrine Tumours
Katiyar, V., & Das, S. (2022) · touchREVIEWS in Oncology & Haematology · doi:10.17925/ohr.2022.18.1.66
“46,47 In this study, among the 22 patients with concomitant non-metastatic pNETs, 90.9% achieved a response.”
BackgroundInfluentialMajor heritable renal cell carcinoma syndromes: novel treatment options and future perspectives
Naidoo, V., Perera, M., & Adam, A. (2022) · Current Opinion in Urology · doi:10.1097/mou.0000000000001030
Efficacy of HIF-PH inhibitors in the treatment for renal anemia
坂下, 碧., & 南学, 正. (2022) · Nippon Ronen Igakkai Zasshi Japanese Journal of Geriatrics · doi:10.3143/geriatrics.59.263
Kidney Cancer Research Highlights from ASCO 2022 Annual Meeting.
Ged, Y. M. A., & Singla, N. (2022) · PubMed
PERIPAPILLARY MASS IN AN ADULT HEALTHY WOMAN
Tekin, K., Celik, S., Teke, M. Y., Johnson, M. W., Vempuluru, V. S., Konstantinou, E. K., & Shields, C. L. (2023) · Retina · doi:10.1097/iae.0000000000003793
García, B. E. C., Santos, C. C. C., Freire, E. B. O., Sánchez, E. A. H., González, A. K. A., Rodas, D. G. C., Yauli, D. L. M., & Gómez, J. C. C. (2023) · EPRA International Journal of Multidisciplinary Research (IJMR) · doi:10.36713/epra12759
Hypoxia and the Metastatic Cascade
Gandhi, S., Khanna, S., Kaushik, S., Chaudhry, D., Ghorawat, P., Chongtham, J., & Srivastava, T. (2023) · Springer eBooks · doi:10.1007/978-981-99-0313-9_9
Data from A View on Drug Development for Cancer Prevention
Reynolds, A. R., Moschetta, M., Yohannes, A. R., Walcott, F. L., Ashford, M. B., Szűcs, Z., Sarbajna, T., Hadfield, J., Harrison, E., Challis, B., et al. (2023) · doi:10.1158/2159-8290.c.6602824.v1
Data from A View on Drug Development for Cancer Prevention
Reynolds, A. R., Moschetta, M., Yohannes, A. R., Walcott, F. L., Ashford, M. B., Szűcs, Z., Sarbajna, T., Hadfield, J., Harrison, E., Challis, B., et al. (2023) · doi:10.1158/2159-8290.c.6602824
On either side of homeostasis: EPAS1 gain- and loss-offunction mutations
Prchal, J. T., & Semenza, G. L. (2023) · Haematologica · doi:10.3324/haematol.2023.283285
“highlight the remarkably rapid progress that has been made since the discovery of HIF-1 three decades ago (2), with development of the HIF stabilizer daprodustat for the treatment of anemia (6) and the HIF-2α inhibitor belzutifan for the treatment of renal cell carcinoma (7).”
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(2023) · Journal of Clinical Review & Case Reports · doi:10.33140/jcrc.08.06.05
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Jain, R., Sarfraz, H. R., Farooq, M. Z., & Sonpavde, G. (2023) · Springer eBooks · doi:10.1007/978-981-99-2341-0_4
van Overdam, K. A., Hajjaj, A., Smid, L. M., Kılıç, E., & de Jong, J. H. (2023) · Case Reports in Ophthalmology · doi:10.1159/000530687
“However, belzutifan also resulted in signi fi cant adverse events [28].”
BackgroundSurgical Management of Hereditary Kidney Cancer Syndromes
Gopal, N., Webster, B. R., Antony, M. B., & Ball, M. W. (2023) · Springer eBooks · doi:10.1007/978-3-031-40901-1_6
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Shinder, B. M., Kronstedt, S., & Hakimi, A. A. (2023) · Springer eBooks · doi:10.1007/978-3-031-40901-1_11
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Basin, M. F., Sager, R., Bhat, K. R. S., & Bratslavsky, G. (2023) · Springer eBooks · doi:10.1007/978-3-031-40901-1_3
Chen, R., Wu, J. T., Che, Y., Jiao, Y., Sun, H., Zhao, Y., Chen, P., Meng, L., & Zhao, T. (2023) · Research Square · doi:10.21203/rs.3.rs-3582005/v1
Jonasch, E. W., Balijepalli, C., Yan, K., Gullapalli, L., Li, J., & Sundaram, M. (2024) · Kidney Cancer · doi:10.3233/kca-230021
Self-citationGhorbani, M., Wang, Z., Chen, X., Tiwari, P. B., Klauda, J. B., & Brelidze, T. I. (2024) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2024.02.23.581826
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Khan, T. M., Gupta, S., & Nilubol, N. (2024) · Springer eBooks · doi:10.1007/978-3-031-53858-2_12
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Ristau, B. T. (2024) · The Journal of Urology · doi:10.1097/ju.0000000000004087
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“2 ; [23]).”
Predominantly multiple focal non-cystic renal lesions: an imaging approach
Santamarina, M. G., Raffo, J. A., Contreras, G. L., Jaime Recasens, T., & Volpacchio, M. M. (2024) · Abdominal Radiology · doi:10.1007/s00261-024-04440-3
(2024) · Elsevier eBooks · doi:10.1016/b978-0-443-13575-0.00225-1
Vickers, E. (2024) · Wiley eBooks · doi:10.1002/9781119834090.ch4
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Konstantakou, E., Bear, L. M., & Iliopoulos, O. (2024) · Elsevier eBooks · doi:10.1016/b978-0-443-19176-3.00045-5
Self-citationMultimodal Treatment Strategy for Central Nervous System Hemangioblastomas
Watanabe, T., Yokogami, K., & Takeshima, H. (2023) · Japanese Journal of Neurosurgery · doi:10.7887/jcns.32.728
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“12 Pathogenic VHL variants reduce VHL protein activity, resulting in stabilization of HIF subunits and subsequent constitutive activation of HIF-mediated transcriptional pathways, independent of oxygen concentrations.”
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Krivosic, V. (2026) · Encyclopédie médico-chirurgicale. Ophtalmologie · doi:10.1016/s0246-0343(25)48285-9
Hurtado, V. H., Mañay, C. E. C., & Aguilar, P. T. (2025) · Oncología (Ecuador) · doi:10.33821/765
Mori, G. H., Fagundes, G. F. C., de Santana, L. S., Freitas-Castro, F., Afonso, A. C. F., Lourenço, D. M., Pereira, M. A. A., Tanno, F. Y., Srougi, V., Chambo, J. L., et al. (2025) · Archives of Endocrinology and Metabolism · doi:10.20945/2359-4292-2024-0354
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Black, P. C., Mumtaz, F., & Tanguay, S. (2025) · Société Internationale d’Urologie Journal · doi:10.3390/siuj6010020
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Hypoxia-Inducible Factors: Regulation and Therapeutic Potential
Lin, I., Chang, K., Cheng, F., Kanwar, R. K., Kamplikar, P., Kanwar, J. R., & Cheung, C. H. A. (2025) · Springer eBooks · doi:10.1007/978-981-96-1016-7_11
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Hämatologische Neoplasien und solide Tumore
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Weber, S., & Bergmann, L. (2025) · Elsevier eBooks · doi:10.1016/b978-3-437-21183-6.00015-5
Hussein, M. J. H., Lundsgaard, M., Madsen, M. G., & Lund, L. C. (2025) · Ugeskrift for Læger · doi:10.61409/v11240800
Smith, C. C., Caleshu, C. A., Bonadies, D. C., & Denton, J. J. (2025) · Journal of Genetic Counseling · doi:10.1002/jgc4.70047
Closson, J. D., Xu, X., Zhang, M., Tiyani, T. T., Marcelino, L. P., Isiorho, E. A., Nagati, J. S., Garcia, J. A., & Gardner, K. H. (2026) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.2514042123
Richard, S., Benoudiba, F., Adam, C., Corréas, J.-M., Krivosic, V., & Aghakhani, N. (2025) · Encyclopédie médico-chirurgicale. Neurologie · doi:10.1016/s0246-0378(25)46750-5
Banerjee, S., Thirunavukkarasu, S., Ojha, R., & Kumar, S. (2025) · Elsevier eBooks · doi:10.1016/b978-0-443-22300-6.00002-4
Coenzyme A homeostasis regulates hypoxic signalling via UBFD1 in triple-negative breast cancerPreprint
Grevitt, P., Maiques, Ó., Shah, K., Morales, V., Gadaleta, E., Dodel, M., Bianchi, K., Gout, I. T., Jones, J. L., Chelala, C., et al. (2025) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2025.06.18.660436
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Gerste, R. D. (2025) · Deutsches Ärzteblatt · doi:10.3238/persasco.2025.07.11.18
Matsumura, N., & Mandai, M. (2025) · International Journal of Clinical Oncology · doi:10.1007/s10147-025-02830-1
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Maquet, C., Crampon, C., Tendron, A., Giraudet, F., Ferrand, F. R., Horion, J., Nebbache, R., Crampon, F., & Deneuve, S. (2025) · Bulletin du Cancer · doi:10.1016/j.bulcan.2025.03.006
Diagnosis and treatment of Von Hippel‑Lindau syndrome: A case series
Dong, S., Chu, H., Duan, R., Bu, Y., Jia, D., Pan, T., & Wang, Y. (2025) · Experimental and Therapeutic Medicine · doi:10.3892/etm.2025.12940
“Insights into VHL gene mutations affecting the VEGF signaling pathway have spurred progress in targeted drug therapies, such as belzutifan (HIF‑2 inhibitor) (36), tivozanib (VEGF receptor tyrosine kinase inhibitor) (37) and bevacizumab (anti‑VEGF monoclonal antibody) (38).”
Li, P.-H., Tsai, J.-Y., & Yeh, T. (2025) · Asian Journal of Surgery · doi:10.1016/j.asjsur.2025.07.127
Zhou, Z., Yu, Y., Chen, C., & Lin, J. (2025) · Genes Chromosomes and Cancer · doi:10.1002/gcc.70071
Nethala, D., Millan, B., Hyman, J., Rathi, N., Hsueh, J. Y., Koller, C. R., Hesswani, C., Kenigsberg, A. P., Mendhiratta, N., Lawson, K. A., et al. (2025) · Urologic Oncology Seminars and Original Investigations · doi:10.1016/j.urolonc.2025.06.015
Self-citationWang, X., Calucho, M., Estrada-Zuniga, C. M., Noureen, M., Gonzalez‐Cantu, H., Mossuto, S., Levi, J. N., Landry, B. N., Guo, Q., Rutherford, K. A., et al. (2025) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2025.08.22.671868
Park, N., & Sung, M. J. (2025) · Journal of Digestive Cancer Research · doi:10.52927/jdcr.2025.13.2.131
Neurogenetic tumor syndromes: The current landscape of workup and treatment
Lukas, R. V., Cannon, A. D., Chittiboina, P., Vasudevan, H. N., Blakeley, J. O., & Hirbe, A. C. (2025) · Neuro-Oncology Practice · doi:10.1093/nop/npaf089
Metadata
- Authors
- Jonasch, E., Donskov, F., Iliopoulos, O., Rathmell, W. K., Narayan, V. K., Maughan, B. L., Oudard, S., Else, T., Maranchie, J. K., Welsh, S. J., Thamake, S., Park, E. K., Perini, R. F., Linehan, W. M., & Srinivasan, R.
- Affiliations
- The University of Texas MD Anderson Cancer Center (US)
- From the University of Texas M.D. Anderson Cancer Center, Houston (E.J.); Aarhus University Hospital, Aarhus, Denmark (F.D.); Massachusetts General Hospital Cancer Center and Harvard Medical School, Boston (O.I.); Vanderbilt University Medical Center, Nashville (W.K.R.); University of Pennsylvania, Philadelphia (V.K.N.); the University of Utah, Salt Lake City (B.L.M.); Hôpital Européen Georges-Pompidou, University of Paris, Paris (S.O.); the University of Michigan, Ann Arbor (T.E.); the University of Pittsburgh, Pittsburgh (J.K.M.); Cambridge University Hospitals NHS Foundation Trust, Cambridge, United Kingdom (S.J.W.); Merck, Kenilworth, NJ (S.T., E.K.P., R.F.P.); and the Center for Cancer Research, National Cancer Institute, Bethesda, MD (W.M.L., R.S.).
- ORCID
- Venue
- New England Journal of Medicine
- Publisher
- Massachusetts Medical Society
- Published
- 2021-11-25
- Volume
- 385
- Issue
- 22
- Pages
- 2036-2046
- ISSN
- 0028-4793 (ISSN-L)
- 1533-4406
- PMC
- PMC9275515
- OpenAlex
- W3215189579
- Cited by
- 714
- References
- 22
- Citations by year
- 2026: 140 · 2025: 167 · 2024: 135 · 2023: 140 · 2022: 128 · 2021: 4
- FWCI
- 66.14
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- New England Journal of Medicine (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- Funders
- Merck Sharp & Dohme Corp., a subsidiary of Merck & Co., Inc., Kenilworth, NJ, USA
- Topics
- Health Sciences › Medicine › Oncology › Renal cell carcinoma treatment
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Cancer Research › Cancer, Hypoxia, and Metabolism
- Health Sciences › Medicine › Oncology › Ear and Head Tumors
- Keywords
- belzutifan, HIF-2α inhibition, von Hippel-Lindau disease, renal cell carcinoma, hemangioblastoma, retinal hemangioblastoma, pancreatic lesions, anemia, drug safety, objective response
- MeSH terms
- Adult, Aged, Anemia, Antineoplastic Agents, Carcinoma, Renal Cell, Fatigue, Female, Follow-Up Studies, von Hippel-Lindau Disease, Humans, Indenes, Kidney Neoplasms, Male, Middle Aged, Neoplasms, Multiple Primary, Pancreatic Neoplasms, Age of Onset, Hemangioblastoma, Neuroendocrine Tumors, Disease Progression, Basic Helix-Loop-Helix Proteins
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