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. 2014 Jul;140(7):1171-7.
doi: 10.1007/s00432-014-1654-y. Epub 2014 Mar 27.

Renal tumor size is an independent prognostic factor for overall survival in von Hippel-Lindau disease

Taekmin Kwon  1 , In Gab Jeong, Sahyun Pak, Dalsan You, Cheryn Song, Jun Hyuk Hong, Hanjong Ahn, Choung-Soo Kim
Affiliations

Renal tumor size is an independent prognostic factor for overall survival in von Hippel-Lindau disease

Taekmin Kwon et al. J Cancer Res Clin Oncol. 2014 Jul.

Abstract

Objectives: To evaluate the effect of renal cell carcinoma (RCC) on survival in von Hippel-Lindau (VHL) disease and to assess the relationship between tumor size and survival.

Materials and methods: In this retrospective cohort study, the medical records of 72 patients who presented with VHL disease between 1994 and 2012 were reviewed. Clinical VHL-related characteristics were analyzed, and the prognostic value of renal tumor size for overall survival was assessed by using Cox regression models.

Results: Of the 72 VHL patients, 42 (58.3 %) and 30 (41.7 %) were male and female, respectively. The mean age was 37.9 years, and the median follow-up period was 61.5 months. In terms of VHL-related manifestations, 40 (55.6 %) had RCC, 46 (63.8 %) had hemangioblastoma in the cerebellum, 10 (13.9 %) had hemangioblastoma in the spinal cord, 34 (47.2 %) had a pancreatic mass, 18 (25.0 %) had pheochromocytoma, and 14 (19.4 %) had retinal capillary hemangioma. RCC was a major cause of mortality: Of the 11 patients who died, nine (12.5 %) died due to RCC progression. The 5-year overall survival rate was 85.6 % for all patients, 96.9 % for patients without RCC, 83.6 % for patients with RCC < 3 cm, and 75.8 % for patients with RCC ≥ 3 cm. Multivariable analysis showed that RCC ≥ 3 cm was an independent predictor of overall survival (HR 9.87, 95 % CI 1.17-83.00, p = 0.035) along with age (HR 1.05, 95 % CI 1.01-1.10, p = 0.027).

Conclusions: Renal tumor size was an independent prognostic factor for overall survival in VHL disease. This observation will be helpful for planning RCC treatment in VHL disease.

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Figures

Fig. 1
Fig. 1
Overall disease-specific survival rate of patients with von Hippel–Lindau disease with and without renal cell carcinoma
See this image and copyright information in PMC

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