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Distinctive clinicopathological features of Von Hippel‑Lindau‑associated hereditary renal cell carcinoma: A single‑institution study

  • Authors:
    • Baoan Hong
    • Zhongyuan Zhang
    • Jingcheng Zhou
    • Kaifang Ma
    • Jiufeng Zhang
    • Lin Cai
    • Ning Zhang
    • Kan Gong
  • View Affiliations / Copyright

    Affiliations: Department of Urology, Peking University First Hospital, Beijing 100034, P.R. China, Department of Urology, Beijing Cancer Hospital, Beijing 100142, P.R. China
    Copyright: © Hong et al. This is an open access article distributed under the terms of Creative Commons Attribution License.
  • Pages: 4600-4606
    |
    Published online on: March 1, 2019
       https://doi.org/10.3892/ol.2019.10091
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Abstract

Von Hippel‑Lindau (VHL) disease is a genetic syndrome that involves the development of tumors in numerous organs. The kidney is one of the most frequently affected organs, and patients with VHL and renal tumors require repeated nephrectomy. The present study aimed to further determine the clinicopathological characteristics of patients with VHL‑associated renal cell carcinoma (RCC), which may allow more rational clinical treatment decisions. This study included 27 patients with VHL who underwent radical or partial nephrectomy at the Peking University First Hospital between January 2010 and April 2018. The clinicopathological characteristics and prognosis of the patients were retrospectively reviewed. The expression of RCC‑associated molecular markers was evaluated by immunohistochemistry. The mean size of the renal tumors was 4.3±2.0 cm (range 1.3‑9.5 cm). The pathological type in 26 cases (96.3%) was clear cell RCC (CCRCC), whereas only one patient was diagnosed with CCRCC and clear cell papillary RCC. Renal cysts with a clear cell lining were observed, and RCC cell clusters were scattered in renal cyst cavities. Among the 27 patients, 21 (77.8%) were diagnosed with stage IA/T1N0M0, according to Tumor‑Node‑Metastasis staging, and 16 (59.3%) had grade 1 tumors. The mean postoperative follow‑up duration was 39.0±24.0 months (range, 1.7‑96.5 months). No metastasis or VHL‑associated mortality was observed. VHL‑associated RCC is a relatively low‑risk disease, and a tumor size of 4 cm was determined as a threshold for nephron‑sparing surgery. In addition, to prevent tumor cell dispersion, renal cysts should be carefully treated. A comprehensive understanding of the clinicopathological characteristics and underlying mechanisms of RCC associated with VHL syndrome may improve patient prognosis.
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1 

Gossage L, Eisen T and Maher ER: VHL, the story of a tumour suppressor gene. Nat Rev Cancer. 15:55–64. 2015. View Article : Google Scholar : PubMed/NCBI

2 

Neumann HP and Wiestler OD: Clustering of features of von Hippel-Lindau syndrome: Evidence for a complex genetic locus. Lancet. 337:1052–1054. 1991. View Article : Google Scholar : PubMed/NCBI

3 

Maher ER, Yates JR, Harries R, Benjamin C, Harris R, Moore AT and Ferguson-Smith MA: Clinical features and natural history of von Hippel-Lindau disease. Q J Med. 77:1151–1163. 1990. View Article : Google Scholar : PubMed/NCBI

4 

Maher ER, Neumann HP and Richard S: von Hippel-Lindau disease: A clinical and scientific review. Eur J Hum Genet. 19:617–623. 2011. View Article : Google Scholar : PubMed/NCBI

5 

Bader HL and Hsu T: Systemic VHL gene functions and the VHL disease. FEBS Lett. 586:1562–1569. 2012. View Article : Google Scholar : PubMed/NCBI

6 

Nielsen SM, Rhodes L, Blanco I, Chung WK, Eng C, Maher ER, Richard S and Giles RH: Von Hippel-Lindau disease: Genetics and role of genetic counseling in a multiple neoplasia syndrome. J Clin Oncol. 34:2172–2181. 2016. View Article : Google Scholar : PubMed/NCBI

7 

Nordstrom-O'Brien M, van der Luijt RB, van Rooijen E, van den Ouweland AM, Majoor-Krakauer DF, Lolkema MP, van Brussel A, Voest EE and Giles RH: Genetic analysis of von Hippel-Lindau disease. Hum Mutat. 31:521–537. 2010.PubMed/NCBI

8 

Chen F, Kishida T, Yao M, Hustad T, Glavac D, Dean M, Gnarra JR, Orcutt ML, Duh FM, Glenn G, et al: Germline mutations in the von Hippel-Lindau disease tumor suppressor gene: Correlations with phenotype. Hum Mutat. 5:66–75. 1995. View Article : Google Scholar : PubMed/NCBI

9 

Hoffman MA, Ohh M, Yang H, Klco JM, Ivan M and Kaelin WG Jr: von Hippel-Lindau protein mutants linked to type 2C VHL disease preserve the ability to downregulate HIF. Hum Mol Genet. 10:1019–1027. 2001. View Article : Google Scholar : PubMed/NCBI

10 

Richard S, David P, Marsot-Dupuch K, Giraud S, Béroud C and Resche F: Central nervous system hemangioblastomas, endolymphatic sac tumors, and von Hippel-Lindau disease. Neurosurg Rev. 23:1–24. 2000. View Article : Google Scholar : PubMed/NCBI

11 

Wang JY, Peng SH, Li T, Ning XH, Liu SJ, Hong BA, Liu JY, Wu PJ, Zhou BW, Zhou JC, et al: Risk factors for survival in patients with von Hippel-Lindau disease. J Med Genet. 55:322–328. 2018. View Article : Google Scholar : PubMed/NCBI

12 

Wu P, Zhang N, Wang X, Ning X, Li T, Bu D and Gong K: Family history of von Hippel-Lindau disease was uncommon in Chinese patients: Suggesting the higher frequency of de novo mutations in VHL gene in these patients. J Hum Genet. 57:238–243. 2012. View Article : Google Scholar : PubMed/NCBI

13 

Wang JY, Peng SH, Ning XH, Li T, Liu SJ, Liu JY, Hong BA, Qi NN, Peng X, Zhou BW, et al: Shorter telomere length increases age-related tumor risks in von Hippel-Lindau disease patients. Cancer Med. 6:2131–2141. 2017. View Article : Google Scholar : PubMed/NCBI

14 

Moch H, Cubilla AL, Humphrey PA, Reuter VE and Ulbright TM: The 2016 WHO classification of tumours of the urinary system and male genital organs-part a: Renal, penile, and testicular tumours. Eur Urol. 70:93–105. 2016. View Article : Google Scholar : PubMed/NCBI

15 

Kim SP, Alt AL, Weight CJ, Costello BA, Cheville JC, Lohse C, Allmer C and Leibovich BC: Independent validation of the 2010 American Joint Committee on Cancer TNM classification for renal cell carcinoma: Results from a large, single institution cohort. J Urol. 185:2035–2039. 2011. View Article : Google Scholar : PubMed/NCBI

16 

Ning XH, Li T, Gong YQ, He Q, Shen QI, Peng SH, Wang JY, Chen JC, Guo YL and Gong K: Association between FBP1 and hypoxia-related gene expression in clear cell renal cell carcinoma. Oncol Lett. 11:4095–4098. 2016. View Article : Google Scholar : PubMed/NCBI

17 

Neumann HP, Bender BU, Berger DP, Laubenberger J, Schultze-Seemann W, Wetterauer U, Ferstl FJ, Herbst EW, Schwarzkopf G, Hes FJ, et al: Prevalence, morphology and biology of renal cell carcinoma in von Hippel-Lindau disease compared to sporadic renal cell carcinoma. J Urol. 160:1248–1254. 1998. View Article : Google Scholar : PubMed/NCBI

18 

Choyke PL, Glenn GM, Walther MM, Patronas NJ, Linehan WM and Zbar B: Von Hippel-Lindau disease: Genetic, clinical, and imaging features. Radiology. 194:629–642. 1995. View Article : Google Scholar : PubMed/NCBI

19 

Ning XH, Zhang N, Li T, Wu PJ, Wang X, Li XY, Peng SH, Wang JY, Chen JC and Gong K: Telomere shortening is associated with genetic anticipation in Chinese Von Hippel-Lindau disease families. Cancer Res. 74:3802–3809. 2014. View Article : Google Scholar : PubMed/NCBI

20 

Walther MM, Lubensky IA, Venzon D, Zbar B and Linehan WM: Prevalence of microscopic lesions in grossly normal renal parenchyma from patients with von Hippel-Lindau disease, sporadic renal cell carcinoma and no renal disease: Clinical implications. J Urol. 154:2010–2015. 1995. View Article : Google Scholar : PubMed/NCBI

21 

Binderup ML, Jensen AM, Budtz-Jørgensen E and Bisgaard ML: Survival and causes of death in patients with von Hippel-Lindau disease. J Med Genet. 54:11–18. 2017. View Article : Google Scholar : PubMed/NCBI

22 

Duffey BG, Choyke PL, Glenn G, Grubb RL, Venzon D, Linehan WM and Walther MM: The relationship between renal tumor size and metastases in patients with von Hippel-Lindau disease. J Urol. 172:63–65. 2004. View Article : Google Scholar : PubMed/NCBI

23 

Gupta GN, Peterson J, Thakore KN, Pinto PA, Linehan WM and Bratslavsky G: Oncological outcomes of partial nephrectomy for multifocal renal cell carcinoma greater than 4 cm. J Urol. 184:59–63. 2010. View Article : Google Scholar : PubMed/NCBI

24 

Lonser RR, Glenn GM, Walther M, Chew EY, Libutti SK, Linehan WM and Oldfield EH: Von Hippel-Lindau disease. Lancet. 361:2059–2067. 2003. View Article : Google Scholar : PubMed/NCBI

25 

Crespigio J, Berbel L, Dias MA, Berbel RF, Pereira SS, Pignatelli D and Mazzuco TL: Von Hippel-Lindau disease: A single gene, several hereditary tumors. J Endocrinol Invest. 41:21–31. 2018. View Article : Google Scholar : PubMed/NCBI

26 

Rao P, Monzon F, Jonasch E, Matin SF and Tamboli P: Clear cell papillary renal cell carcinoma in patients with von Hippel-Lindau syndrome-clinicopathological features and comparative genomic analysis of 3 cases. Hum Pathol. 45:1966–1972. 2014. View Article : Google Scholar : PubMed/NCBI

27 

Diolombi ML, Cheng L, Argani P and Epstein JI: Do clear cell papillary renal cell carcinomas have malignant potential. Am J Surg Pathol. 39:1621–1634. 2015. View Article : Google Scholar : PubMed/NCBI

28 

Kim E and Zschiedrich S: Renal cell carcinoma in von Hippel-Lindau disease-from tumor genetics to novel therapeutic strategies. Front Pediatr. 6:162018. View Article : Google Scholar : PubMed/NCBI

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Copy and paste a formatted citation
Spandidos Publications style
Hong B, Zhang Z, Zhou J, Ma K, Zhang J, Cai L, Zhang N and Gong K: Distinctive clinicopathological features of Von Hippel‑Lindau‑associated hereditary renal cell carcinoma: A single‑institution study. Oncol Lett 17: 4600-4606, 2019.
APA
Hong, B., Zhang, Z., Zhou, J., Ma, K., Zhang, J., Cai, L. ... Gong, K. (2019). Distinctive clinicopathological features of Von Hippel‑Lindau‑associated hereditary renal cell carcinoma: A single‑institution study. Oncology Letters, 17, 4600-4606. https://doi.org/10.3892/ol.2019.10091
MLA
Hong, B., Zhang, Z., Zhou, J., Ma, K., Zhang, J., Cai, L., Zhang, N., Gong, K."Distinctive clinicopathological features of Von Hippel‑Lindau‑associated hereditary renal cell carcinoma: A single‑institution study". Oncology Letters 17.5 (2019): 4600-4606.
Chicago
Hong, B., Zhang, Z., Zhou, J., Ma, K., Zhang, J., Cai, L., Zhang, N., Gong, K."Distinctive clinicopathological features of Von Hippel‑Lindau‑associated hereditary renal cell carcinoma: A single‑institution study". Oncology Letters 17, no. 5 (2019): 4600-4606. https://doi.org/10.3892/ol.2019.10091
Copy and paste a formatted citation
x
Spandidos Publications style
Hong B, Zhang Z, Zhou J, Ma K, Zhang J, Cai L, Zhang N and Gong K: Distinctive clinicopathological features of Von Hippel‑Lindau‑associated hereditary renal cell carcinoma: A single‑institution study. Oncol Lett 17: 4600-4606, 2019.
APA
Hong, B., Zhang, Z., Zhou, J., Ma, K., Zhang, J., Cai, L. ... Gong, K. (2019). Distinctive clinicopathological features of Von Hippel‑Lindau‑associated hereditary renal cell carcinoma: A single‑institution study. Oncology Letters, 17, 4600-4606. https://doi.org/10.3892/ol.2019.10091
MLA
Hong, B., Zhang, Z., Zhou, J., Ma, K., Zhang, J., Cai, L., Zhang, N., Gong, K."Distinctive clinicopathological features of Von Hippel‑Lindau‑associated hereditary renal cell carcinoma: A single‑institution study". Oncology Letters 17.5 (2019): 4600-4606.
Chicago
Hong, B., Zhang, Z., Zhou, J., Ma, K., Zhang, J., Cai, L., Zhang, N., Gong, K."Distinctive clinicopathological features of Von Hippel‑Lindau‑associated hereditary renal cell carcinoma: A single‑institution study". Oncology Letters 17, no. 5 (2019): 4600-4606. https://doi.org/10.3892/ol.2019.10091
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