Tratamiento farmacológico de las neoplasias asociadas con la enfermedad de von Hippel-Lindau. Revisión Sistemática
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Copyright (c) 2025 Veronica Hurtado Hurtado, Cristina Cabrera Mañay, Patricia Tamayo Aguilar
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https://doi.org/10.33821/765Keywords:
Von Hippel- Lindau Disease, drug therapy, belzutifan, carcinoma, renal cell, hemangioblastoma, VHL protein, sunitinibAbstract
Background: Von Hippel-Lindau disease is an autosomal dominant syndrome characterized by the development of benign and malignant tumors throughout life. For many years, neoplasms associated with this disease were treated by surgical resection or ablation with the aim of reducing the risk of metastatic disease and controlling local or systemic sequelae. An effective systemic alternative could reduce the surgical burden and represents a new approach to oncological treatment.
Objective: To evaluate the efficacy and safety of different drugs used in the treatment of neoplasms associated with Von Hippel-Lindau disease. Search methods: An electronic search was carried out without language restriction, until July 31, 2024, in the Cochrane Central Register of Controlled Trials (CENTRAL), PUBMED, and SCIELO databases. Selection criteria: Clinical trials with patients with malignancies associated with Von Hippel-Lindau disease, and any targeted drug therapy as intervention were included. Data collection and analysis: Data from each clinical study were entered into a data table for qualitative analysis. Results: Five articles were selected, four of them are prospective studies and one is a retrospective study that evaluates the efficacy of treatment with Sunitinib, Dovitinib, Pazopanib, and Belzutifan. Conclusions: The inhibition of HIF-2? with Belzutifan presents a safer and more effective profile than the antiangiogenic agents Sunitinib and Pazopanib.
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