Clinical, surgical, pathological and follow-up features of kidney cancer patients with Von Hippel-Lindau syndrome: novel insights from a large consortium.
World J Urol
; 39(8): 2969-2975, 2021 Aug.
Article
em En
| MEDLINE
| ID: mdl-33416974
ABSTRACT
PURPOSE:
To investigate the natural history and follow-up after kidney tumor treatment of Von Hippel-Lindau (VHL) patients. MATERIALS ANDMETHODS:
A multi-institutional European consortium of patients with VHL syndrome included 96 non-metastatic patients treated at 9 urological departments (1987-2018). Descriptive and survival analyses were performed. RESULTS ANDLIMITATIONS:
Median age at VHL diagnosis was 34 years (IQR 25-43). Two patients (2.1%) showed only renal manifestations at VHL diagnosis. Concomitant involvement of Central Nervous System (CNS) vs. pancreas vs. eyes vs. adrenal gland vs. others were present in 60.4 vs. 68.7 vs. 30.2 vs. 15.6 vs. 15.6% of patients, respectively. 45% of patients had both CNS and pancreatic diseases alongside kidney. The median interval between VHL diagnosis and renal cancer treatment resulted 79 months (IQR 0-132), and median index tumor size leading to treatment was 35.5 mm (IQR 28-60). Of resected malignant tumours, 73% were low grade. Of high-grade tumors, 61.1% were large > 4 cm. With a median follow-up of 8 years, clinical renal progression rate was 11.7% and 29.3% at 5 and 10 years, respectively. Overall mortality was 4% and 7.5% at 5 and 10 years, respectively. During the follow-up, 50% of patients did not receive a second active renal treatment. Finally, 25.3% of patients had CKD at last follow-up.CONCLUSIONS:
Mean period between VHL diagnosis and renal cancer detection is roughly three years, with significant variability. Although, most renal tumors are small low-grade, clinical progression and mortality are not negligible. Moreover, kidney function represents a key issue in VHL patients.Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Pancreatopatias
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Doenças do Sistema Nervoso Central
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Proteína Supressora de Tumor Von Hippel-Lindau
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Oftalmopatias
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Doença de von Hippel-Lindau
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Neoplasias Renais
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Nefrectomia
Tipo de estudo:
Etiology_studies
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Observational_studies
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Prognostic_studies
/
Risk_factors_studies
País/Região como assunto:
Europa
Idioma:
En
Revista:
World J Urol
Ano de publicação:
2021
Tipo de documento:
Article
País de afiliação:
Itália