Publications by authors named "E Jonasch"

Modern advances in systemic and localized therapies for patients with renal cell carcinoma (RCC) have significantly improved patients' outcomes. If disease progression occurs after initial treatment, clinicians often have multiple options for a first salvage therapy. Because salvage and initial treatments both may affect overall survival time, and they may interact in unanticipated ways, there is a growing need to determine sequences of initial therapy and first salvage therapy that maximize overall survival while maintaining quality of life.

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Background And Objective: Belzutifan, a hypoxia-inducible factor 2 alpha inhibitor, was approved initially for patients with von Hippel-Lindau disease and more recently for sporadic, metastatic clear cell renal cell carcinoma (ccRCC) based on the results of LITESPARK-005. There is a paucity of data regarding real-world experience with belzutifan in patients with sporadic, metastatic ccRCC. This study aims to describe clinical outcomes with belzutifan in patients with sporadic, metastatic ccRCC.

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Article Synopsis
  • - The LITESPARK-004 study is testing the hypoxia-inducible factor-2α inhibitor, belzutifan, for its effectiveness in treating tumors associated with von Hippel-Lindau disease, specifically focusing on patients with central nervous system (CNS) haemangioblastomas after additional follow-up.
  • - A total of 61 patients, with a majority having CNS haemangioblastomas, were enrolled in this phase 2 study across multiple cancer centers in four countries, and they received oral belzutifan until there were unacceptable side effects or disease progression.
  • - The study assesses the antitumor activity by evaluating patient responses using two different methods based on tumor measurements and is ongoing, with results still being
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Article Synopsis
  • The study evaluates the effectiveness of the oral drug belzutifan in treating retinal hemangioblastomas associated with von Hippel-Lindau disease during the LITESPARK-004 trial.
  • The analysis included 61 participants, with 12 having evaluable retinal lesions; all of the assessed eyes showed improvement after treatment, leading to a 100% response rate.
  • By the end of the follow-up, no new disease progression was reported, and significant reductions in the size of hemangioblastomas were observed in a subgroup of patients, indicating the drug's potential efficacy.
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Clear cell renal cell carcinoma (ccRCC) is characterized by dysregulated hypoxia signaling and a tumor microenvironment (TME) highly enriched in myeloid and lymphoid cells. Loss of the von Hippel Lindau (VHL) gene is a critical early event in ccRCC pathogenesis and promotes stabilization of HIF. Whether VHL loss in cancer cells affects immune cells in the TME remains unclear.

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