ArticleOrphanet journal of rare diseases2019
Growth characteristics and therapeutic decision markers in von Hippel-Lindau disease patients with renal cell carcinoma.
Article in Orphanet journal of rare diseases, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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Who cites it
11 citing papers in PubMed, 19 citations in OpenAlex.
- Belzutifan for von Hippel-Lindau Disease: Pancreatic Lesion Population of the Phase 2 LITESPARK-004 Study.Clinical cancer research : an official journal of the American Association for Cancer Research · 2024Trial
- Case Report: Diagnosis and treatment report and literature review of 2 cases of VHL-deficient renal cell carcinoma.Frontiers in oncology · 2026Article
- Longitudinal Evaluation of Clear-cell Renal Cell Carcinoma in von Hippel-Lindau Disease.European urology · 2025Article
- BOLA family genes are the drivers and potential biomarkers of survival in kidney renal clear cell carcinoma patients.Saudi medical journal · 2024Observational
- CT-derived radiomics predict the growth rate of renal tumours in von Hippel-Lindau syndrome.Clinical radiology · 2024Article
- Patient Sex and Origin Influence Distribution of Driver Genes and Clinical Presentation of Paraganglioma.Journal of the Endocrine Society · 2024Article
- Natural history of Von Hippel-Lindau disease-associated and sporadic clear cell renal cell carcinoma: a comparative study.Journal of cancer research and clinical oncology · 2022Article
- Advanced Kidney Volume Measurement Method Using Ultrasonography with Artificial Intelligence-Based Hybrid Learning in Children.Sensors (Basel, Switzerland) · 2021Article
- Evaluation, diagnosis and surveillance of renal masses in the setting of VHL disease.World journal of urology · 2021Review
- Pretreatment metastatic growth rate determines clinical outcome of advanced melanoma patients treated with anti-PD-1 antibodies: a multicenter cohort study.Journal for immunotherapy of cancer · 2021Article
- Hemangioblastoma and von Hippel-Lindau disease: genetic background, spectrum of disease, and neurosurgical treatment.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2020Article
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Authors and funding
10 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundVon Hippel-Lindau (VHL) disease is a multi-systemic hereditary disease associated with several benign and malignant tumor entities, including clear cell renal cell carcinoma (ccRCC). Since ccRCCs grow slowly, nephron sparing surgery is typically performed at a tumor diameter of 3-4 cm before the tumor metastasizes. However, in the case of recurrent disease, repeated surgical intervention can impair renal function. Therefore, it is crucial to optimize the timing for surgical interventions through a better understanding of the growth kinetics of ccRCCs in VHL. We investigated tumor growth kinetics and modern volumetric assessment to guide future therapeutic decisions.
resultsThe prevalence of ccRCC was 28% in a cohort of 510 VHL patients. Of 144 patients with ccRCC, 41 were followed with serial imaging which identified 102 renal tumors, which exhibited heterogeneous growth kinetics. ccRCCs grew at an average absolute growth rate of 0.287 cm/year, an average relative growth rate [(lnV
conclusionsTumor heterogeneity and differences in growth kinetics is suggestive of a state of transient tumor dormancy in ccRCCs of VHL patients. The relative growth rate has not been previously described in other studies. Volumetric assessment as an additional parameter for surgical intervention could be a useful clinical tool and needs further investigation.
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