Evaluation of Belzutifan in Patients with Tumors Associated with Von Hippel-Lindau Disease: A Retrospective Analysis from a Single Center - Takeaways - MDSpire

Evaluation of Belzutifan in Patients with Tumors Associated with Von Hippel-Lindau Disease: A Retrospective Analysis from a Single Center

  • By

  • Kilian Rhein

  • Fruzsina Kotsis

  • Athina Ganner

  • Sophie Astheimer

  • Christine Julia Gizaw

  • Yannik Laich

  • Cordula Annette Jilg

  • Jan-Helge Klingler

  • Elke Neumann-Haefelin

  • November 1, 2025

  • 0 min

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  • 1

    Von Hippel-Lindau disease is a tumor predisposition syndrome with a 100% lifetime penetrance by age 75, leading to various benign and malignant tumors.

  • 2

    Belzutifan is an oral HIF-2α inhibitor approved for treating VHL-associated tumors, demonstrating efficacy in renal cell carcinoma and other lesions.

  • 3

    The Freiburg VHL Center has extensive experience in managing VHL patients, providing comprehensive care and surveillance protocols since 1992.

  • 4

    Belzutifan was used off-label in advanced VHL cases where conventional treatments posed risks to organ function or were ineffective.

  • 5

    The decision to initiate belzutifan therapy involved a multidisciplinary team, focusing on organ involvement, tumor burden, and patient clinical status.

Original Source(s)

Evaluation of Belzutifan in Patients with Tumors Associated with Von Hippel-Lindau Disease: A Retrospective Analysis from a Single Center

  • by Kilian Rhein, Fruzsina Kotsis, Athina Ganner, Sophie Astheimer, Christine Julia Gizaw, Yannik Laich, Cordula Annette Jilg, Jan-Helge Klingler, Elke Neumann-Haefelin

  • November 1, 2025

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