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Cirugia y cirujanos · 2026

Analysis of survival in patients with sporadic hemangioblastoma versus that linked to von Hippel-Lindau. Experience of two reference centers.

СтатьяНаблюдательное исследованиеСравнительное исследованиеМногоцентровое исследованиеНазвание и аннотация: Перевод пока не подготовлен
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Torrecilla-Martínez Antonio, Osuna-Soto Julio, Sanz-Zorrilla Alicia, Sánchez-Ramírez Inmaculada, Haro-Yuste Sergio, Gálvez-Medina María J, Sánchez-Sánchez Rafael, Ortega-Salas Rosa, Leiva-Cepas Fernando

Аннотация

OBJECTIVE: To establish the overall survival (OS) and disease-free survival (DFS) profiles regarding the patients diagnosed with hemangioblastoma in the context of a von Hippel-Lindau (VHL) disease and those of sporadic presentation.

METHODS: An observational and analytical study was conducted using a retrospective historical cohort, followed prospectively. The inclusion criteria consisted of patients operated of hemangioblastoma in two reference hospitals, between 2000 and 2023. A total of 39 patients were included. The Kaplan-Meier estimator was used to summarize data, and the log-rank test was used in comparative analysis.

RESULTS: The OS at 5 years was around 94%. No differences were found between VHL patients compared to sporadic tumours (p = 0.683). DFS at 2 years was 59% in VHL patients and 96% in sporadic, with statistically significant differences (p < 0.0001). In addition to this, DFS at 5 years in patients with a predominant cystic component was 73% and 95% in those with a solid component (p = 0.029).

CONCLUSIONS: The DFS of hemangioblastomas in patients with VHL is significantly lower than in sporadic hemangioblastomas, but no difference in OS can be claimed. DFS in cystic-predominant hemangioblastomas is significantly lower than in solid-predominant hemangioblastomas.

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