W Marston Linehan

Summary

Affiliation: National Institutes of Health
Country: USA

Publications

  1. pmc Tumor-specific hypermethylation of epigenetic biomarkers, including SFRP1, predicts for poorer survival in patients from the TCGA Kidney Renal Clear Cell Carcinoma (KIRC) project
    Christopher J Ricketts
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, United States of America
    PLoS ONE 9:e85621. 2014
  2. pmc Targeting HIF2α translation with Tempol in VHL-deficient clear cell renal cell carcinoma
    Carole Sourbier
    Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA
    Oncotarget 3:1472-82. 2012
  3. pmc Metabolic reprogramming for producing energy and reducing power in fumarate hydratase null cells from hereditary leiomyomatosis renal cell carcinoma
    Youfeng Yang
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, United States of America
    PLoS ONE 8:e72179. 2013
  4. doi request reprint A novel germline mutation in BAP1 predisposes to familial clear-cell renal cell carcinoma
    Megan N Farley
    Urologic Oncology Branch, National Cancer Institute, Bldg 10 CRC 1W5940, Bethesda, MD 20892
    Mol Cancer Res 11:1061-71. 2013
  5. doi request reprint Molecular pathways: Fumarate hydratase-deficient kidney cancer--targeting the Warburg effect in cancer
    W Marston Linehan
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD 20892, USA
    Clin Cancer Res 19:3345-52. 2013
  6. doi request reprint Non-clear cell renal cancer: disease-based management and opportunities for targeted therapeutic approaches
    W Marston Linehan
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD, USA
    Semin Oncol 40:511-20. 2013
  7. pmc The metabolic basis of kidney cancer
    W Marston Linehan
    Urologic Oncology Branch, National Cancer Institute, Bethesda, MD, United States
    Semin Cancer Biol 23:46-55. 2013
  8. ncbi request reprint Identification of the genes for kidney cancer: opportunity for disease-specific targeted therapeutics
    W Marston Linehan
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD 20892, USA
    Clin Cancer Res 13:671s-679s. 2007
  9. ncbi request reprint Identification of the genes for kidney cancer
    W Marston Linehan
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, Maryland 20892 1107, USA
    Cancer Biol Ther 5:696-9. 2006
  10. pmc Hereditary kidney cancer: unique opportunity for disease-based therapy
    W Marston Linehan
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Building 10 CRC, Room 1 5940, Bethesda, MD 20892 1107, USA
    Cancer 115:2252-61. 2009

Detail Information

Publications129 found, 100 shown here

  1. pmc Tumor-specific hypermethylation of epigenetic biomarkers, including SFRP1, predicts for poorer survival in patients from the TCGA Kidney Renal Clear Cell Carcinoma (KIRC) project
    Christopher J Ricketts
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, United States of America
    PLoS ONE 9:e85621. 2014
    ....
  2. pmc Targeting HIF2α translation with Tempol in VHL-deficient clear cell renal cell carcinoma
    Carole Sourbier
    Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA
    Oncotarget 3:1472-82. 2012
    ....
  3. pmc Metabolic reprogramming for producing energy and reducing power in fumarate hydratase null cells from hereditary leiomyomatosis renal cell carcinoma
    Youfeng Yang
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, United States of America
    PLoS ONE 8:e72179. 2013
    ..This increased NADPH producing PPP activity was shown to be a strong consistent feature in both fumarate hydratase deficient tumors and cell line models. ..
  4. doi request reprint A novel germline mutation in BAP1 predisposes to familial clear-cell renal cell carcinoma
    Megan N Farley
    Urologic Oncology Branch, National Cancer Institute, Bldg 10 CRC 1W5940, Bethesda, MD 20892
    Mol Cancer Res 11:1061-71. 2013
    ..These findings suggest that BAP1 is an early-onset familial RCC predisposing gene...
  5. doi request reprint Molecular pathways: Fumarate hydratase-deficient kidney cancer--targeting the Warburg effect in cancer
    W Marston Linehan
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD 20892, USA
    Clin Cancer Res 19:3345-52. 2013
    ..Targeting the metabolic basis of a rare cancer such as FH-deficient kidney cancer will hopefully provide insights into the development of effective forms of therapies for other, more common forms of cancer...
  6. doi request reprint Non-clear cell renal cancer: disease-based management and opportunities for targeted therapeutic approaches
    W Marston Linehan
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD, USA
    Semin Oncol 40:511-20. 2013
    ..In this review, we discuss molecular and clinical characteristics of each of the non-clear cell RCC subtypes and describe ongoing efforts to develop novel agents for this subset of patients. ..
  7. pmc The metabolic basis of kidney cancer
    W Marston Linehan
    Urologic Oncology Branch, National Cancer Institute, Bethesda, MD, United States
    Semin Cancer Biol 23:46-55. 2013
    ..Understanding the metabolic basis of kidney cancer will hopefully provide the foundation for the development of effective forms of therapy for this disease...
  8. ncbi request reprint Identification of the genes for kidney cancer: opportunity for disease-specific targeted therapeutics
    W Marston Linehan
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD 20892, USA
    Clin Cancer Res 13:671s-679s. 2007
    ..These Mendelian single-gene syndromes provide a unique opportunity to evaluate the effectiveness of agents that target the VHL, c-Met, BHD, and fumarate hydratase pathways...
  9. ncbi request reprint Identification of the genes for kidney cancer
    W Marston Linehan
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, Maryland 20892 1107, USA
    Cancer Biol Ther 5:696-9. 2006
  10. pmc Hereditary kidney cancer: unique opportunity for disease-based therapy
    W Marston Linehan
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Building 10 CRC, Room 1 5940, Bethesda, MD 20892 1107, USA
    Cancer 115:2252-61. 2009
    ..Studies of the tricarboxylic acid cycle and the VHL-HIF pathways have provided the foundation for therapeutic approaches in patients with HLRCC-associated kidney cancer as well as other hereditary and sporadic forms of RCC...
  11. pmc Molecular diagnosis and therapy of kidney cancer
    W Marston Linehan
    Urologic Oncology Branch, National Cancer Institute, Bethesda, Maryland 20892, USA
    Annu Rev Med 61:329-43. 2010
    ..Knowledge of these kidney cancer gene pathways has enabled new approaches in the management of this disease and has provided the foundation for the development of targeted therapeutics...
  12. pmc The genetic basis of kidney cancer: a metabolic disease
    W Marston Linehan
    Urologic Oncology Branch, National Cancer Institute, Bethesda, MD 20892 1107, USA
    Nat Rev Urol 7:277-85. 2010
    ..Targeting the fundamental metabolic abnormalities in kidney cancer provides a unique opportunity for the development of more-effective forms of therapy for this disease...
  13. pmc Germline BHD-mutation spectrum and phenotype analysis of a large cohort of families with Birt-Hogg-Dubé syndrome
    Laura S Schmidt
    Basic Research Program, Science Applications International Corporation Frederick Inc, Frederick, MD, USA
    Am J Hum Genet 76:1023-33. 2005
    ..This study expands the BHD-mutation spectrum and evaluates genotype-phenotype correlations among families with BHD...
  14. ncbi request reprint Early onset hereditary papillary renal carcinoma: germline missense mutations in the tyrosine kinase domain of the met proto-oncogene
    Laura S Schmidt
    Basic Research Program, SAIC Frederick, Inc, Frederick, Maryland, USA
    J Urol 172:1256-61. 2004
    ..In the current study we evaluated the clinical phenotype and germline MET mutation of 3 new HPRC families. We describe the early onset clinical features of HPRC...
  15. pmc Homozygous loss of BHD causes early embryonic lethality and kidney tumor development with activation of mTORC1 and mTORC2
    Yukiko Hasumi
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA
    Proc Natl Acad Sci U S A 106:18722-7. 2009
    ....
  16. pmc The UOK 257 cell line: a novel model for studies of the human Birt-Hogg-Dubé gene pathway
    Youfeng Yang
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Building 10, Room 1W 5888, Bethesda, MD 20892, USA
    Cancer Genet Cytogenet 180:100-9. 2008
    ..The result demonstrates that the established tumor cells consist of two cell populations, one containing four and one containing five copies of the MYC oncogene...
  17. ncbi request reprint High frequency of somatic frameshift BHD gene mutations in Birt-Hogg-Dubé-associated renal tumors
    Cathy D Vocke
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD 20892, USA
    J Natl Cancer Inst 97:931-5. 2005
    ..These results support a role for BHD as a tumor suppressor gene that predisposes to the development of renal tumors when both copies are inactivated...
  18. ncbi request reprint Hereditary leiomyomatosis and renal cell cancer: a syndrome associated with an aggressive form of inherited renal cancer
    Robert L Grubb
    Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA
    J Urol 177:2074-9; discussion 2079-80. 2007
    ..We report the clinical characteristics and urological treatment of patients with hereditary leiomyomatosis and renal cell cancer associated renal tumors...
  19. pmc Identification and characterization of a novel folliculin-interacting protein FNIP2
    Hisashi Hasumi
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20894, United States
    Gene 415:60-7. 2008
    ....
  20. pmc Molecular alterations in primary prostate cancer after androgen ablation therapy
    Carolyn J M Best
    Pathogenetics Unit, Laboratory of Pathology, National Cancer Institute, NIH, Bethesda, Maryland 20892, USA
    Clin Cancer Res 11:6823-34. 2005
    ..The molecular mechanisms underlying progression are not well known in part due to the rarity of androgen-independent samples from primary and metastatic sites...
  21. ncbi request reprint Differential expression of erythropoietin and its receptor in von hippel-lindau-associated and multiple endocrine neoplasia type 2-associated pheochromocytomas
    Timothy W A Vogel
    Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Building 10, Room 5D 37, Bethesda, Maryland 20892 1414, USA
    J Clin Endocrinol Metab 90:3747-51. 2005
    ..These findings suggest an alternative process of tumorigenesis in VHL- and MEN 2-associated pheochromocytomas and implicate Epo as a clinical biomarker to differentiate these tumors...
  22. pmc UOK 262 cell line, fumarate hydratase deficient (FH-/FH-) hereditary leiomyomatosis renal cell carcinoma: in vitro and in vivo model of an aberrant energy metabolic pathway in human cancer
    Youfeng Yang
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, 10 Center Dr, MSC 1107, Bldg 10 CRC, Room 1 5942, Bethesda, MD 20892 1107
    Cancer Genet Cytogenet 196:45-55. 2010
    ..This tumor model is the embodiment of the Warburg effect. UOK 262 provides a unique in vitro and in vivo preclinical model for studying the bioenergetics of the Warburg effect in human cancer...
  23. pmc Lung cysts, spontaneous pneumothorax, and genetic associations in 89 families with Birt-Hogg-Dubé syndrome
    Jorge R Toro
    Genetic Epidemiology Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, Rockville, MD 20892 7231, USA
    Am J Respir Crit Care Med 175:1044-53. 2007
    ..Birt-Hogg-Dubé syndrome (BHDS) is an autosomal, dominantly inherited genodermatosis that predisposes to fibrofolliculomas, kidney neoplasms, lung cysts, and spontaneous pneumothorax...
  24. ncbi request reprint Patterns of aneuploidy in stage IV clear cell renal cell carcinoma revealed by comparative genomic hybridization and spectral karyotyping
    Christian P Pavlovich
    Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892, USA
    Genes Chromosomes Cancer 37:252-60. 2003
    ..e., whole chromosomal gains and losses (7.8/case)] or chromosomal rearrangements (10.7/case), of which the majority were unbalanced translocations...
  25. pmc Defining early-onset kidney cancer: implications for germline and somatic mutation testing and clinical management
    Brian Shuch
    All authors Center for Cancer Research, National Cancer Institute, Bethesda, MD
    J Clin Oncol 32:431-7. 2014
    ..No guidelines exist for patient selection for RCC germline mutation testing. We evaluate how age of onset could indicate the need for germline mutation testing for detection of inherited forms of kidney cancer...
  26. ncbi request reprint HIF overexpression correlates with biallelic loss of fumarate hydratase in renal cancer: novel role of fumarate in regulation of HIF stability
    Jennifer S Isaacs
    Urologic Oncology Branch, National Cancer Institute, Bethesda, Maryland 20892, USA
    Cancer Cell 8:143-53. 2005
    ..Further, we show that fumarate acts as a competitive inhibitor of HPH. These data delineate a novel fumarate-dependent pathway for regulating HPH activity and HIF protein levels...
  27. ncbi request reprint The genetic basis of cancer of the kidney
    W Marston Linehan
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, Maryland 20892, USA
    J Urol 170:2163-72. 2003
    ..We identified the genetic basis of these different types of kidney cancer to provide better methods for early diagnosis of this disease as well as provide the foundation for the development of molecular therapeutic approaches...
  28. doi request reprint Adrenal nodular hyperplasia in hereditary leiomyomatosis and renal cell cancer
    Brian Shuch
    Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892 1107, USA
    J Urol 189:430-5. 2013
    ....
  29. pmc Pathway biomarker profiling of localized and metastatic human prostate cancer reveal metastatic and prognostic signatures
    Robert L Grubb
    Urologic Oncology Branch, National Cancer Institute, Bethesda, Maryland, USA
    J Proteome Res 8:3044-54. 2009
    ....
  30. pmc Genetic basis for kidney cancer: opportunity for disease-specific approaches to therapy
    Elizabeth Cartwright Pfaffenroth
    National Cancer Institute, Medical Oncology, Urologic Oncology Branch, 10 Center Drive MSC 1107, Building 10 CRC Room 1 5942, Bethesda, Maryland 20892 1107, USA
    Expert Opin Biol Ther 8:779-90. 2008
    ..Kidney cancer is not a homogenous entity; it is comprised of many different tumor types, with different biologies and molecular mechanisms leading to disease and therefore different treatment approaches...
  31. ncbi request reprint Metastases to hemangioblastomas in von Hippel-Lindau disease
    S Taylor Jarrell
    Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892 1414, USA
    J Neurosurg 105:256-63. 2006
    ....
  32. ncbi request reprint Genetic basis of cancer of the kidney: disease-specific approaches to therapy
    W Marston Linehan
    Urologic Oncology Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, Bethesda, Maryland 20892 1501, USA
    Clin Cancer Res 10:6282S-9S. 2004
    ..The HLRC gene has been found to be the Krebs cycle enzyme, fumarate hydratase. Studies are under way to understand the downstream pathway of this cancer gene...
  33. pmc Comparison of snap freezing versus ethanol fixation for gene expression profiling of tissue specimens
    Mark A Perlmutter
    Pathogenetics Unit, Laboratory of Pathology and Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA
    J Mol Diagn 6:371-7. 2004
    ..However, ethanol fixation and paraffin embedding of tissue specimens is not optimal for high-throughput mRNA expression analysis. Improved methods for transcript profiling of archival samples, and/or tissue processing are still required...
  34. ncbi request reprint Gene promoter methylation in prostate tumor-associated stromal cells
    Jeffrey A Hanson
    Genetics Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA
    J Natl Cancer Inst 98:255-61. 2006
    ..Although tumor-associated stromal cells also exhibit changes in gene expression, promoter methylation has not been described in these cells...
  35. ncbi request reprint Molecular differentiation of high- and moderate-grade human prostate cancer by cDNA microarray analysis
    Carolyn J M Best
    Pathogenetics Unit, Laboratory of Pathology and Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA
    Diagn Mol Pathol 12:63-70. 2003
    ..We suggest that these data provide insight into the molecular nature of clinically aggressive prostate cancer...
  36. ncbi request reprint Assessment of normalization strategies for quantitative RT-PCR using microdissected tissue samples
    Heidi S Erickson
    Pathogenetics Unit, Laboratory of Pathology and Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA
    Lab Invest 87:951-62. 2007
    ..Taken together, these results suggest that precise gene expression measurements can be made from MD samples if the appropriate normalization strategy is employed...
  37. pmc Kidney-targeted Birt-Hogg-Dube gene inactivation in a mouse model: Erk1/2 and Akt-mTOR activation, cell hyperproliferation, and polycystic kidneys
    Masaya Baba
    Urologic Oncology Branch, National Cancer Institute Frederick, Frederick, MD 21702, USA
    J Natl Cancer Inst 100:140-54. 2008
    ..BHD encodes folliculin, a protein that may interact with the energy- and nutrient-sensing 5'-AMP-activated protein kinase-mammalian target of rapamycin (AMPK-mTOR) signaling pathways...
  38. pmc Low suspicion lesions on multiparametric magnetic resonance imaging predict for the absence of high-risk prostate cancer
    Nitin K Yerram
    Urologic Oncology Branch, National Cancer Institute, Bethesda, MD 20892 1210, USA
    BJU Int 110:E783-8. 2012
    ..Lesions with a low suspician level at mpMRI of the prostate have low risk of including high risk prostate cancer...
  39. pmc Regulation of mitochondrial oxidative metabolism by tumor suppressor FLCN
    Hisashi Hasumi
    Urologic Oncology Branch, National Cancer Institute, 10 Center Dr, MSC 1107, CRC Rm 1 5940W, Bethesda, MD 20892, USA
    J Natl Cancer Inst 104:1750-64. 2012
    ..Genetic studies of BHD patients have uncovered the causative gene, FLCN, but its function is incompletely understood...
  40. pmc Succinate dehydrogenase kidney cancer: an aggressive example of the Warburg effect in cancer
    Christopher J Ricketts
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA
    J Urol 188:2063-71. 2012
    ..We report our experience with the diagnosis, evaluation and treatment of this novel form of hereditary kidney cancer...
  41. pmc Association of germline mutations in the fumarate hydratase gene and uterine fibroids in women with hereditary leiomyomatosis and renal cell cancer
    Laveta Stewart
    Division of Cancer Epidemiology and Genetics, Genetic Epidemiology Branch, National Cancer Institute, National Institutes of Health, Rockville, MD 20892 7231, USA
    Arch Dermatol 144:1584-92. 2008
    ..To investigate the risk of uterine fibroids and other reproductive risk factors in women with hereditary leiomyomatosis and renal cell cancer (HLRCC)...
  42. ncbi request reprint Expression of Birt-Hogg-Dubé gene mRNA in normal and neoplastic human tissues
    Michelle B Warren
    Laboratory of Immunobiology, Center for Cancer Research, NCI Frederick, Frederick, MD, USA
    Mod Pathol 17:998-1011. 2004
    ..These results indicate a wide expression pattern for BHD mRNA in many tissues, including skin, lung and kidney, which are involved in the BHD phenotype, and support a tumor suppressor role for BHD in renal cancer...
  43. ncbi request reprint Surgical management of multi-organ visceral tumors in patients with von Hippel-Lindau disease: a single stage approach
    Jonathan J Hwang
    Urological Oncology Branch, National Cancer Institute, National Institute of Health, Bethesda, Maryland, USA
    J Urol 169:895-8. 2003
    ..We assessed surgical feasibility of a 1-stage multi-organ approach for multiple visceral tumors in patients with von Hippel-Lindau disease...
  44. pmc Mutations in the fumarate hydratase gene cause hereditary leiomyomatosis and renal cell cancer in families in North America
    Jorge R Toro
    Genetic Epidemiology Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, 6120 Executive Boulevard, Executive Plaza South, Rockville, MD 20892, USA
    Am J Hum Genet 73:95-106. 2003
    ..HLRCC is associated with clinically significant uterine fibroids and aggressive renal tumors. The present study also expands the histologic spectrum of renal tumors and FH mutations associated with HLRCC...
  45. pmc Von Hippel-Lindau (VHL) inactivation in sporadic clear cell renal cancer: associations with germline VHL polymorphisms and etiologic risk factors
    Lee E Moore
    Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, United States of America
    PLoS Genet 7:e1002312. 2011
    ..A proportion of tumors from current smokers lacked VHL alterations and may represent a biologically distinct clinical entity from inactivated cases...
  46. ncbi request reprint The contribution of VHL substrate binding and HIF1-alpha to the phenotype of VHL loss in renal cell carcinoma
    Jodi K Maranchie
    Urologic Oncology Branch, National Institutes of Health, Bethesda, MD 20892, USA
    Cancer Cell 1:247-55. 2002
    ..We further demonstrate that normoxic stabilization of HIF1alpha alone, while capable of mimicking some aspects of VHL loss, is not sufficient to reproduce tumorigenesis, indicating that it is not the critical oncogenic substrate of VHL...
  47. pmc Genotype and tumor locus determine expression profile of pseudohypoxic pheochromocytomas and paragangliomas
    Uma Shankavaram
    Radiation Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892 1109, USA
    Neoplasia 15:435-47. 2013
    ..The presented data suggest novel subclassification of pseudohypoxic PHEOs/PGLs and implies cluster-specific pathogenic mechanisms and treatment strategies...
  48. ncbi request reprint Evaluation of ethanol-fixed, paraffin-embedded tissues for proteomic applications
    Mamoun Ahram
    Pathogenetics Unit, National Cancer Institute, NIH, Bethesda, MD 20892, USA
    Proteomics 3:413-21. 2003
    ..In light of these results, we conclude that ethanol-fixed tissues can be successfully utilized for proteomic analyses...
  49. pmc Robot-assisted laparoscopic partial nephrectomy for tumors greater than 4 cm and high nephrometry score: feasibility, renal functional, and oncological outcomes with minimum 1 year follow-up
    Gopal N Gupta
    Urologic Oncology Branch, National Cancer Institute, Bethesda, MD 20892, USA
    Urol Oncol 31:51-6. 2013
    ..We report on the technical feasibility and renal functional and oncologic outcomes with minimum 1 year follow-up of robot-assisted laparoscopic partial nephrectomy (RALPN) for tumors greater than 4 cm...
  50. pmc Tumor suppressor FLCN inhibits tumorigenesis of a FLCN-null renal cancer cell line and regulates expression of key molecules in TGF-beta signaling
    Seung Beom Hong
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, 10 Center Drive MSC1107, 10 CRC 1W 5940, Bethesda, MD 20892, USA
    Mol Cancer 9:160. 2010
    ..Somatic mutations or loss of heterozygosity in the remaining wild type copy of the FLCN gene have been found in renal tumors from BHD patients suggesting that FLCN is a classic tumor suppressor gene...
  51. ncbi request reprint The role of 6-[18F]fluorodopamine positron emission tomography in the localization of adrenal pheochromocytoma associated with von Hippel-Lindau syndrome
    Priya Kaji
    Reproductive Biology and Medicine Branch, Section on Medical Neuroendocrinology, National Institute of Child Health and Human Development, Building 10, CRC, 1 East, Room 1 3140, 10 Center Drive, MSC 1109, Bethesda, Maryland 20892, USA
    Eur J Endocrinol 156:483-7. 2007
    ..Therefore, we investigated whether [(18)F]DA PET is more effective than [(123/131)I]MIBG scintigraphy in the diagnostic localization of VHL-related adrenal pheochromocytoma...
  52. pmc Inactivation of the FLCN tumor suppressor gene induces TFE3 transcriptional activity by increasing its nuclear localization
    Seung Beom Hong
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, Maryland, United States of America
    PLoS ONE 5:e15793. 2010
    ..Since TFE3 is implicated in RCC, we hypothesized that elevated GPNMB expression was due to increased TFE3 activity resulting from the inactivation of FLCN...
  53. pmc Fumarate hydratase deficiency in renal cancer induces glycolytic addiction and hypoxia-inducible transcription factor 1alpha stabilization by glucose-dependent generation of reactive oxygen species
    Sunil Sudarshan
    Urologic Oncology Branch, National Cancer Institute NIH, Bethesda, MD 20892, USA
    Mol Cell Biol 29:4080-90. 2009
    ..These data reveal that the obligate glycolytic switch present in HLRCC is critical to HIF stabilization via ROS generation...
  54. ncbi request reprint Familial renal carcinoma: clinical evaluation, clinical subtypes and risk of renal carcinoma development
    Berton Zbar
    Urologic Oncology Branch, National Cancer Institute, Bethesda, Maryland 20892 1107, USA
    J Urol 177:461-5; discussion 465. 2007
    ..We also determined the risk of renal carcinoma in first-degree relatives of affected family members...
  55. pmc Prostate cancer: can multiparametric MR imaging help identify patients who are candidates for active surveillance?
    Baris Turkbey
    Molecular Imaging Program, Laboratory of Pathology, Radiation Oncology Branch, and Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, 10 Center Dr, MSC 1182 Bldg 10, Room B3B69, Bethesda, MD 20892 1088, USA
    Radiology 268:144-52. 2013
    ....
  56. doi request reprint Very distal apical prostate tumours: identification on multiparametric MRI at 3 Tesla
    Jeffrey W Nix
    National Institute of Health, National Cancer Institute, Urologic Oncology Branch, Bethesda, MD 20892, USA
    BJU Int 110:E694-700. 2012
    ..g. high intensity focused ultrasound therapy that might spare the distal apex...
  57. ncbi request reprint The in vitro and in vivo effects of re-expressing methylated von Hippel-Lindau tumor suppressor gene in clear cell renal carcinoma with 5-aza-2'-deoxycytidine
    Wade G Alleman
    Howard Hughes Medical Institute, Chevy Chase, MD, USA
    Clin Cancer Res 10:7011-21. 2004
    ..We test the ability of two hypo-methylating agents to re-express VHL in cell culture and in mice bearing human ccRCC and evaluate the effects of re-expressed VHL in these models...
  58. ncbi request reprint Regulation of HIF prolyl hydroxylases by hypoxia-inducible factors
    Olga Aprelikova
    Laboratory of Biosystems and Cancer, Center for Cancer Research, National Cancer Institute, Bethesda, Maryland 20892, USA
    J Cell Biochem 92:491-501. 2004
    ..These data further advance our understanding of the differential role of HIF factors and putative feedback loop in HIF regulation...
  59. pmc Use of patient-specific MRI-based prostate mold for validation of multiparametric MRI in localization of prostate cancer
    Hari Trivedi
    Center for Interventional Oncology, Radiology and Imaging Sciences, Clinical Center, Molecular Imaging Program, Urologic Oncology Branch, Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892 1088, USA
    Urology 79:233-9. 2012
    ..A significant difficulty to date has been accurate correlation between the magnetic resonance images and histopathologic specimens...
  60. pmc Genotype-phenotype correlation in von Hippel-Lindau disease with retinal angiomatosis
    Wai T Wong
    Division of Epidemiology and Clinical Research, Office of the Scientific Director, National Eye Institute, National Institutes of Health, Bethesda, MD 20892, USA
    Arch Ophthalmol 125:239-45. 2007
    ....
  61. pmc Repeat partial nephrectomy on the solitary kidney: surgical, functional and oncological outcomes
    Nick W Liu
    Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892 1414, USA
    J Urol 183:1719-24. 2010
    ..We examined outcomes in patients with recurrent or de novo renal lesions treated with repeat partial nephrectomy on a solitary kidney...
  62. ncbi request reprint Usefulness of standardized uptake values for distinguishing adrenal glands with pheochromocytoma from normal adrenal glands by use of 6-18F-fluorodopamine PET
    Henri J L M Timmers
    Reproductive and Adult Endocrinology Program, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892 1109, USA
    J Nucl Med 48:1940-4. 2007
    ..6-(18)F-Fluorodopamine ((18)F-FDA) PET is a highly sensitive tool for the localization of pheochromocytoma (PHEO). The aim of this study was to establish cutoff values for pathologic and physiologic adrenal gland tracer uptake...
  63. ncbi request reprint Transcriptional regulation of phenylethanolamine N-methyltransferase in pheochromocytomas from patients with von Hippel-Lindau syndrome and multiple endocrine neoplasia type 2
    Thanh Truc Huynh
    Clinical Neurocardiology Section, National Institute of Neurological Disorders and Stroke, Building 10, Room 6N252, National Institutes of Health, 10 Center Drive, MSC 1620, Bethesda, MD 20892 1620, USA
    Ann N Y Acad Sci 1073:241-52. 2006
    ..Microarray analysis, however, did indicate differences in expression of genes involved in neural crest cell lineage and chromaffin cell development, consistent with differential survival of PNMT-expressing cells in the two syndromes...
  64. ncbi request reprint Evaluation and management of renal tumors in the Birt-Hogg-Dubé syndrome
    Christian P Pavlovich
    Urologic Oncology Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute and Basic Research Program, SAIC Frederick, Inc, Frederick, Maryland, USA
    J Urol 173:1482-6. 2005
    ..Herein we describe the evaluation and management of renal tumors in Birt-Hogg-Dubé (BHD), an autosomal dominant disorder predisposing to cutaneous fibrofolliculomas, pulmonary cysts, spontaneous pneumothorax and renal tumors...
  65. pmc Oncological outcomes of partial nephrectomy for multifocal renal cell carcinoma greater than 4 cm
    Gopal N Gupta
    Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA
    J Urol 184:59-63. 2010
    ..We present oncological outcomes in patients with hereditary renal cell carcinoma treated with partial nephrectomy for multifocal solid tumors with the largest lesion greater than 4 cm...
  66. pmc Inhibition of tumor cell motility by the interferon-inducible GTPase MxA
    J Frederic Mushinski
    Laboratory of Cancer Biology and Genetics, Medical Oncology Branch, Pediatric Oncology Branch, and Urologic Oncology Branch, Center for Cancer Research, NCI, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Biol Chem 284:15206-14. 2009
    ..These data demonstrate that MxA inhibits tumor cell motility and invasion, and that MxA expression can be induced by small molecules, potentially offering a new approach to the prevention and treatment of metastasis...
  67. pmc Predicting survival in patients with metastatic kidney cancer by gene-expression profiling in the primary tumor
    James R Vasselli
    Urologic Oncology Branch, National Cancer Institute, Bethesda, MD 20892, USA
    Proc Natl Acad Sci U S A 100:6958-63. 2003
    ..We conclude that survival in patients with metastatic renal cell cancer can be correlated with the expression of various genes based solely on the expression profile in the primary kidney tumor...
  68. ncbi request reprint Coexpression of erythropoietin and erythropoietin receptor in von Hippel-Lindau disease-associated renal cysts and renal cell carcinoma
    Youn Soo Lee
    Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Cancer Institute NIH, 10 Center Drive, Bethesda, MD 20892, USA
    Clin Cancer Res 11:1059-64. 2005
    ..Such arrest may lead to autocrine stimulation, cell proliferation, and renal tumor development, similar to tumorigenesis of VHL disease-associated hemangioblastomas...
  69. pmc Impact of ischemia and procurement conditions on gene expression in renal cell carcinoma
    Nick W Liu
    Urologic Oncology Branch, National Cancer Institute, NIH, Bethesda, MD, USA
    Clin Cancer Res 19:42-9. 2013
    ..There are no studies that evaluated effects of ischemia in renal tumors. This study examines the impact of ischemia and tissue procurement conditions on RNA integrity and gene expression in renal cell carcinoma...
  70. pmc Outcomes of patients with surgically treated bilateral renal masses and a minimum of 10 years of followup
    Eric A Singer
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA
    J Urol 188:2084-8. 2012
    ..We report the outcomes of patients with bilateral renal masses and a minimum 10-year followup...
  71. ncbi request reprint Impact of pathological tumour characteristics in patients with sarcomatoid renal cell carcinoma
    Brian Shuch
    Urologic Oncology Branch, The National Cancer Institute, Bethesda, MD, USA
    BJU Int 109:1600-6. 2012
    ..This series helps define the prognostic influence of histological subtype, type of sarcomatoid morphology, the percentage necrosis and sarcomatoid features, and the presence of microvascular invasion...
  72. ncbi request reprint Histomathematical analysis of clinical specimens: challenges and progress
    Gallya Gannot
    Laboratory of Pathology and Urologic Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892 4605, USA
    J Histochem Cytochem 53:177-85. 2005
    ..This strategy likely will be a useful method for extending the number of proteins that can be analyzed in clinical cancer specimens using currently available laboratory techniques...
  73. ncbi request reprint Renal tumors in the Birt-Hogg-Dubé syndrome
    Christian P Pavlovich
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute NIH, Bldg 10 Rm 2N212, Bethesda, MD 20892, USA
    Am J Surg Pathol 26:1542-52. 2002
    ..Recognition by the pathologist of the unusual renal tumors associated with BHD may assist in the clinical diagnosis of the syndrome...
  74. ncbi request reprint The genetic basis of cancer of kidney cancer: implications for gene-specific clinical management
    W Marston Linehan
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, Maryland, USA
    BJU Int 95:2-7. 2005
  75. ncbi request reprint Expression microdissection: operator-independent retrieval of cells for molecular profiling
    Michael A Tangrea
    Pathogenetics Unit, Laboratory of Pathology and Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, Maryland, USA
    Diagn Mol Pathol 13:207-12. 2004
    ..xMD may become a valuable tool for investigators studying cancer or other disease states in patient specimens and animal models...
  76. ncbi request reprint Expression of hypoxia inducible factor-1alpha and 2alpha in genetically distinct early renal cortical tumors
    Chong M Kim
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892 1107, USA
    J Urol 175:1908-14. 2006
    ..To assess if HIF over expression is a prominent feature of other renal cell carcinoma histological subtypes we characterized the expression of HIF-1alpha and HIF-2alpha in genetically distinct early renal cortical tumors...
  77. ncbi request reprint Genetic disorders and renal cell carcinoma
    Jodi K Maranchie
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, 9000 Rockville Pike, Building 10 2B47, Bethesda, MD 20892, USA
    Urol Clin North Am 30:133-41. 2003
    ..Recognition of familial syndromes will facilitate the institution of parenchymal sparing measures, as well as appropriate screening and intervention for associated nonrenal manifestations...
  78. ncbi request reprint Hereditary leiomyomatosis associated with bilateral, massive, macronodular adrenocortical disease and atypical cushing syndrome: a clinical and molecular genetic investigation
    Ludmila Matyakhina
    Developmental Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Clinical Research Center, 10 Center Drive, MSC 1103, Bethesda, Maryland 20892, USA
    J Clin Endocrinol Metab 90:3773-9. 2005
    ....
  79. doi request reprint Correlation of magnetic resonance imaging tumor volume with histopathology
    Baris Turkbey
    Molecular Imaging Program, National Cancer Institute, Bethesda, Maryland 20892 1210, USA
    J Urol 188:1157-63. 2012
    ..We determined the accuracy of magnetic resonance imaging for determining index tumor volume compared with volumes derived from histopathology...
  80. doi request reprint Association of type O blood with pancreatic neuroendocrine tumors in Von Hippel-Lindau syndrome
    Allison B Weisbrod
    Endocrine Oncology Section, Surgery Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA
    Ann Surg Oncol 19:2054-9. 2012
    ..We analyzed the association of ABO blood type with pancreatic neuroendocrine tumors (PNETs) in a high-risk cohort of patients with Von Hippel-Lindau (VHL) syndrome...
  81. ncbi request reprint Post-analysis follow-up and validation of microarray experiments
    Rodrigo F Chuaqui
    Pathogenetics Unit, Laboratory of Pathology and Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, Maryland 20892, USA
    Nat Genet 32:509-14. 2002
    ....
  82. ncbi request reprint Linking laboratory and clinical research: the development of molecularly targeted therapeutics inside the national cancer institute center for cancer research
    J Carl Barrett
    National Cancer Institute Center for Cancer Research, National Institutes of Health, Bethesda, MD 20892, USA
    Clin Adv Hematol Oncol 1:302-6. 2003
    ..Its infrastructure supports the iterative flow of information from the bench to the bedside and from the bedside to the bench, expediting the delivery of molecularly based therapeutics to cancer patients...
  83. pmc Robot-assisted laparoscopic partial adrenalectomy: initial experience
    Ronald S Boris
    Urologic Oncology Branch, National Cancer Institute, Building 10 Room 1 5940, Bethesda, MD 20892 1107, USA
    Urology 77:775-80. 2011
    ..To evaluate the feasibility of performing robot-assisted laparoscopic partial adrenalectomy (RALPA) in patients seen at the National Cancer Institute and report the results of our initial experience...
  84. pmc Molecular sub-classification of renal epithelial tumors using meta-analysis of gene expression microarrays
    Thomas Sanford
    Urologic Oncology Branch, National Cancer Institute, Bethesda, Maryland, United States of America
    PLoS ONE 6:e21260. 2011
    ..To evaluate the accuracy of the sub-classification of renal cortical neoplasms using molecular signatures...
  85. pmc Increasing reactive oxygen species as a therapeutic approach to treat hereditary leiomyomatosis and renal cell carcinoma
    Carole Sourbier
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA
    Cell Cycle 9:4183-9. 2010
    ..Increasing tumor ROS with bortezomib in combination with cisplatin represents a novel targeted therapeutic approach to treat advanced HLRCC-associated renal tumors...
  86. ncbi request reprint Gene expression profiling of benign and malignant pheochromocytoma
    Frederieke M Brouwers
    Reproductive Biology and Medicine Branch, National Institute of Child Health and Human Development, National Institutes of Health, Building 10, CRC, Room 1E 1 3140, 10 Center Drive, MSC 1109, Bethesda, MD 20892 1109, USA
    Ann N Y Acad Sci 1073:541-56. 2006
    ....
  87. ncbi request reprint Risk factors for skin breakdown after renal and adrenal surgery
    Jennifer Stevens
    Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892 1502, USA
    Urology 64:246-9. 2004
    ..We have noted skin breakdown in patients placed on stabilizing devices and in patients with germline von Hippel-Lindau (VHL) gene mutations (a gene important in angiogenesis)...
  88. pmc Robot-assisted laparoscopic partial adrenalectomy for pheochromocytoma: the National Cancer Institute technique
    Kevin P Asher
    Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892 1107, USA
    Eur Urol 60:118-24. 2011
    ..Partial adrenalectomy has recently been advocated to preserve unaffected adrenal tissue during resection of pheochromocytoma...
  89. pmc Von Hippel-Lindau tumor suppressor gene loss in renal cell carcinoma promotes oncogenic epidermal growth factor receptor signaling via Akt-1 and MEK-1
    S Justin Lee
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD 20892, USA
    Eur Urol 54:845-53. 2008
    ..To determine the functional impact of EGFR activation on RCC, we suppressed critical components of this pathway: EGFR, Akt-1, and MEK-1...
  90. ncbi request reprint Mutations in a novel gene lead to kidney tumors, lung wall defects, and benign tumors of the hair follicle in patients with the Birt-Hogg-Dubé syndrome
    Michael L Nickerson
    Laboratory of Immunobiology, Center for Cancer Research, SAIC Frederick, Inc, National Center for Cancer Research, Frederick, MD 21702, USA
    Cancer Cell 2:157-64. 2002
    ....
  91. ncbi request reprint Genetic basis of cancer of the kidney
    Sunil Sudarshan
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD 20892 1107, USA
    Semin Oncol 33:544-51. 2006
    ..The elucidation of molecular pathogenesis in these familial forms of kidney cancer should provide the opportunity to determine successful approaches for novel therapeutic agents...
  92. pmc Folliculin encoded by the BHD gene interacts with a binding protein, FNIP1, and AMPK, and is involved in AMPK and mTOR signaling
    Masaya Baba
    Laboratories of Immunobiology, Center for Cancer Research, National Cancer Institute Frederick, Frederick, MD 21702, USA
    Proc Natl Acad Sci U S A 103:15552-7. 2006
    ..Our data suggest that FLCN, mutated in Birt-Hogg-Dubé syndrome, and its interacting partner FNIP1 may be involved in energy and/or nutrient sensing through the AMPK and mTOR signaling pathways...
  93. ncbi request reprint Studying cancer families to identify kidney cancer genes
    Berton Zbar
    Laboratory of Immunobiology, Center for Cancer Research, National Cancer Institute at Frederick, Frederick, Maryland 21702, USA
    Annu Rev Med 54:217-33. 2003
    ....
  94. ncbi request reprint Topotecan blocks hypoxia-inducible factor-1alpha and vascular endothelial growth factor expression induced by insulin-like growth factor-I in neuroblastoma cells
    Kiichiro Beppu
    Pediatric Oncology Branch, Center for Cancer Research, National Cancer Institute, NIH, Bethesda, Maryland 20892, USA
    Cancer Res 65:4775-81. 2005
    ....
  95. ncbi request reprint Solid renal tumor severity in von Hippel Lindau disease is related to germline deletion length and location
    Jodi K Maranchie
    Urologic Oncology Branch, National Cancer Institute, Bethesda, Maryland 20892, USA
    Hum Mutat 23:40-6. 2004
    ..Careful correlation of genotypic data with objective phenotypic measures will provide further insight into the mechanisms of tumor formation...
  96. ncbi request reprint Heterogeneous gene methylation patterns among pre-invasive and cancerous lesions of the prostate: a histopathologic study of whole mount prostate specimens
    Karen Woodson
    Cancer Prevention Studies Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892, USA
    Prostate 60:25-31. 2004
    ..This is the first study to evaluate gene methylation patterns across multiple pre-cancerous and invasive cancer foci from the same prostate gland...
  97. ncbi request reprint VHL-mediated hypoxia regulation of cyclin D1 in renal carcinoma cells
    Ranjit S Bindra
    Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, NIH, Bethesda, MD 20892, USA
    Cancer Res 62:3014-9. 2002
    ....
  98. ncbi request reprint Focus on kidney cancer
    W Marston Linehan
    Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD 20892, USA
    Cancer Cell 6:223-8. 2004
  99. ncbi request reprint Highlights from the Society of Urologic Oncology 4th annual meeting
    Jonathan Coleman
    Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Urol 173:938-41. 2005
    ..The meeting was attended by urologists, medical and radiation oncologists, and researchers whose focus is genitourinary (GU) malignancies. More than 500 participants registered for the meeting...
  100. ncbi request reprint Compensatory alterations in energy homeostasis characterized in uterine tumors from hereditary leiomyomatosis and renal cell cancer
    William H Catherino
    Department of Obstetrics and Gynecology, Uniformed Services University of the Health Sciences, Bethesda, Maryland 20814 4799, USA
    Fertil Steril 88:1039-48. 2007
    ..To determine the molecular alterations that maintain energy homeostasis in hereditary leiomyomatosis and renal cell cancer (HLRCC) uterine tumors with disrupted fumarate hydratase, compared with nonsyndromic uterine tumors...
  101. pmc Mistaken identifiers: gene name errors can be introduced inadvertently when using Excel in bioinformatics
    Barry R Zeeberg
    Genomics and Bioinformatics Group, Laboratory of Molecular Pharmacology, Center for Cancer Research CCR, National Cancer Institute NCI, National Institutes of Health NIH, Bethesda, MD 20892 USA
    BMC Bioinformatics 5:80. 2004
    ..When processing microarray data sets, we recently noticed that some gene names were being changed inadvertently to non-gene names...