Metastasis of Clear-Cell Renal Carcinoma to Cerebellar Hemangioblastoma in von Hippel Lindau Disease: Rare or not Investigated?

2001 ◽  
Vol 143 (10) ◽  
pp. 1059-1063 ◽  
Author(s):  
C. Mottolese ◽  
H. Stan ◽  
F. Giordano ◽  
D. Frappaz ◽  
D. Alexei ◽  
...  
Keyword(s):  
Renal Carcinoma ◽  
Clear Cell ◽  
Clear Cell Renal Carcinoma ◽  
Cerebellar Hemangioblastoma ◽  
Von Hippel Lindau ◽  
Cell Renal Carcinoma ◽  
Von Hippel Lindau Disease

Somatic mutations of the von Hippel — Lindau disease tumour suppressor gene in non-familial clear cell renal carcinoma

1994 ◽  
Vol 3 (12) ◽  
pp. 2169-2173 ◽  
Author(s):  
Keith Foster ◽  
Amanda Prowse ◽  
Anke van den Berg ◽  
Stewart Fleming ◽  
Miriam M.F. Hulsbeek ◽  
...  
Keyword(s):  
Somatic Mutations ◽  
Renal Carcinoma ◽  
Clear Cell ◽  
Suppressor Gene ◽  
Tumour Suppressor Gene ◽  
Tumour Suppressor ◽  
Clear Cell Renal Carcinoma ◽  
Von Hippel Lindau ◽  
Cell Renal Carcinoma ◽  
Von Hippel Lindau Disease

Clear cell renal carcinoma presenting as a bleeding cyst in pregnancy: inaugural manifestation of a von Hippel-Lindau disease

2008 ◽  
Vol 69 (03) ◽  
pp. 224-228 ◽  
Author(s):  
I. Simon ◽  
S. Rorive ◽  
C. Kirkpatrick ◽  
T. Roumeguere ◽  
J.L. Nortier
Keyword(s):  
Renal Carcinoma ◽  
Clear Cell ◽  
Clear Cell Renal Carcinoma ◽  
Von Hippel Lindau ◽  
Cell Renal Carcinoma ◽  
Von Hippel Lindau Disease ◽  
In Pregnancy

scholarly journals Emetine Promotes von Hippel-Lindau-Independent Degradation of Hypoxia-Inducible Factor-2α in Clear Cell Renal Carcinoma

2010 ◽  
Vol 78 (6) ◽  
pp. 1072-1078 ◽  
Author(s):  
Hye-Sik Kong ◽  
Sunmin Lee ◽  
Kristin Beebe ◽  
Bradley Scroggins ◽  
Gopal Gupta ◽  
...  
Keyword(s):  
Renal Carcinoma ◽  
Clear Cell ◽  
Hypoxia Inducible Factor ◽  
Clear Cell Renal Carcinoma ◽  
Von Hippel Lindau ◽  
Cell Renal Carcinoma

scholarly journals Molecular and Metabolic Subtypes in Sporadic and Inherited Clear Cell Renal Cell Carcinoma

Genes ◽  
2021 ◽  
Vol 12 (3) ◽  
pp. 388
Author(s):  
Maria F. Czyzyk-Krzeska ◽  
Julio A. Landero Figueroa ◽  
Shuchi Gulati ◽  
John T. Cunningham ◽  
Jarek Meller ◽  
...  
Keyword(s):  
Renal Carcinoma ◽  
Clear Cell ◽  
Hypoxia Inducible Factors ◽  
Clear Cell Renal Carcinoma ◽  
Cell Renal Cell Carcinoma ◽  
Biochemical Alterations ◽  
Von Hippel Lindau ◽  
Cell Renal Carcinoma ◽  
Altered Metabolism ◽  
High Level

The promise of personalized medicine is a therapeutic advance where tumor signatures obtained from different omics platforms, such as genomics, transcriptomics, proteomics, and metabolomics, in addition to environmental factors including metals and metalloids, are used to guide the treatments. Clear cell renal carcinoma (ccRCC), the most common type of kidney cancer, can be sporadic (frequently) or genetic (rare), both characterized by loss of the von Hippel-Lindau (VHL) gene that controls hypoxia inducible factors. Recently, several genomic subtypes were identified with different prognoses. Transcriptomics, proteomics, metabolomics and metallomic data converge on altered metabolism as the principal feature of the disease. However, in view of multiple biochemical alterations and high level of tumor heterogeneity, identification of clearly defined subtypes is necessary for further improvement of treatments. In the future, single-cell combined multi-omics approaches will be the next generation of analyses gaining deeper insights into ccRCC progression and allowing for design of specific signatures, with better prognostic/predictive clinical applications.

scholarly journals Implications of Von Hippel-Lindau Syndrome and Renal Cell Carcinoma

2015 ◽  
Vol 2 (4) ◽  
pp. 163-173 ◽  
Author(s):  
Kenan Ashouri ◽  
Sophia Mohseni ◽  
John Tourtelot ◽  
Pranav Sharma ◽  
Philippe E Spiess
Keyword(s):  
Renal Function ◽  
Targeted Therapies ◽  
Renal Carcinoma ◽  
Clear Cell ◽  
Renal Involvement ◽  
Clear Cell Renal Carcinoma ◽  
Von Hippel Lindau ◽  
Cell Renal Carcinoma ◽  
Von Hippel Lindau Syndrome

Von Hippel-Lindau syndrome (VHLS) is a rare hereditary neoplastic disorder caused by mutations in the vhl gene leading to the development of tumors in several organs including the central nervous system, pancreas, kidneys, and reproductive organs. Manifestations of VHLS can present at different ages based on the affected organ and subclass of disease. In the subclasses of VHLS that cause renal disease, renal involvement typically begins closer to the end of the second decade of life and can present in different ways ranging from simple cystic lesions to solid tumors. Mutations in vhl are most often associated with clear cell renal carcinoma, the most common type of renal cancer, and also play a major role in sporadic cases of clear cell renal carcinoma. The recurrent, multifocal nature of this disease presents difficult challenges in the long-term management of patients with VHLS. Optimization of renal function warrants the use of several different approaches common to the management of renal carcinoma such as nephron sparing surgery, enucleation, ablation, and targeted therapies. In VHLS, renal lesions of 3 cm or bigger are considered to have metastatic potential and even small lesions often harbor malignancy. Many of the aspects of management revolve around optimizing both oncologic outcome and long-term renal function. As new surgical strategies and targeted therapies develop, the management of this complex disease evolves.  This review will discuss the key aspects of the current management of VHLS.

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