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A Phase II Trial of Vandetanib in Children and Adults with Succinate Dehydrogenase-Deficient Gastrointestinal Stromal Tumor.

Glod J, Arnaldez FI, Wiener L, Spencer M, Killian JK, Meltzer P, Dombi E, Derse-Anthony C, Derdak J, Srinivasan R, Linehan WM, Miettinen M, Steinberg SM, Helman L, Widemann BC.

Clin Cancer Res. 2019 Aug 22. doi: 10.1158/1078-0432.CCR-19-0986. [Epub ahead of print]


Imaging of glucose metabolism by 13C-MRI distinguishes pancreatic cancer subtypes in mice.

Kishimoto S, Brender JR, Crooks DR, Matsumoto S, Seki T, Oshima N, Merkle H, Lin P, Reed G, Chen AP, Ardenkjaer-Larsen JH, Munasinghe J, Saito K, Yamamoto K, Choyke PL, Mitchell J, Lane AN, Fan TW, Linehan WM, Krishna MC.

Elife. 2019 Aug 13;8. pii: e46312. doi: 10.7554/eLife.46312.


Familial Kidney Cancer: Implications of New Syndromes and Molecular Insights.

Carlo MI, Hakimi AA, Stewart GD, Bratslavsky G, Brugarolas J, Chen YB, Linehan WM, Maher ER, Merino MJ, Offit K, Reuter VE, Shuch B, Coleman JA.

Eur Urol. 2019 Jul 17. pii: S0302-2838(19)30500-7. doi: 10.1016/j.eururo.2019.06.015. [Epub ahead of print] Review.


The Cancer Genome Atlas of renal cell carcinoma: findings and clinical implications.

Linehan WM, Ricketts CJ.

Nat Rev Urol. 2019 Sep;16(9):539-552. doi: 10.1038/s41585-019-0211-5. Epub 2019 Jul 5. Review.


The Metabolic Basis of Kidney Cancer.

Linehan WM, Schmidt LS, Crooks DR, Wei D, Srinivasan R, Lang M, Ricketts CJ.

Cancer Discov. 2019 Aug;9(8):1006-1021. doi: 10.1158/2159-8290.CD-18-1354. Epub 2019 May 14. Review.


TFE3 Xp11.2 Translocation Renal Cell Carcinoma Mouse Model Reveals Novel Therapeutic Targets and Identifies GPNMB as a Diagnostic Marker for Human Disease.

Baba M, Furuya M, Motoshima T, Lang M, Funasaki S, Ma W, Sun HW, Hasumi H, Huang Y, Kato I, Kadomatsu T, Satou Y, Morris N, Karim BO, Ileva L, Kalen JD, Wilan Krisna LA, Hasumi Y, Sugiyama A, Kurahashi R, Nishimoto K, Oyama M, Nagashima Y, Kuroda N, Araki K, Eto M, Yao M, Kamba T, Suda T, Oike Y, Schmidt LS, Linehan WM.

Mol Cancer Res. 2019 Aug;17(8):1613-1626. doi: 10.1158/1541-7786.MCR-18-1235. Epub 2019 May 1.


Birt-Hogg-Dubé syndrome initially diagnosed as tuberous sclerosis complex.

Pithadia DJ, Treichel AM, Lee CR, Cowen EW, Linehan WM, Moss J, Darling TN.

JAAD Case Rep. 2019 Apr 5;5(4):368-371. doi: 10.1016/j.jdcr.2019.02.009. eCollection 2019 Apr. No abstract available.


MicroRNA-204-5p: A novel candidate urinary biomarker of Xp11.2 translocation renal cell carcinoma.

Kurahashi R, Kadomatsu T, Baba M, Hara C, Itoh H, Miyata K, Endo M, Morinaga J, Terada K, Araki K, Eto M, Schmidt LS, Kamba T, Linehan WM, Oike Y.

Cancer Sci. 2019 Jun;110(6):1897-1908. doi: 10.1111/cas.14026. Epub 2019 May 24.


A comparison of outcomes for standard and multiplex partial nephrectomy in a solitary kidney: The National Cancer Institute experience.

Baiocco JA, Ball MW, Pappajohn AK, Rayn KN, Bratslavsky G, Boyle SL, Linehan WM, Metwalli AR.

Urol Oncol. 2019 Jun;37(6):356.e1-356.e7. doi: 10.1016/j.urolonc.2019.02.015. Epub 2019 Mar 20.


Salvage Surgery After Percutaneous Ablation of Renal Mass in Solitary Kidney in a Patient With Von Hippel-Lindau.

Gomella PT, Linehan WM, Ball MW.

Clin Genitourin Cancer. 2019 Jun;17(3):e482-e484. doi: 10.1016/j.clgc.2019.01.011. Epub 2019 Jan 26. No abstract available.


Metabolic Labeling of Cultured Mammalian Cells for Stable Isotope-Resolved Metabolomics: Practical Aspects of Tissue Culture and Sample Extraction.

Crooks DR, Fan TW, Linehan WM.

Methods Mol Biol. 2019;1928:1-27. doi: 10.1007/978-1-4939-9027-6_1.


A chemoproteomic portrait of the oncometabolite fumarate.

Kulkarni RA, Bak DW, Wei D, Bergholtz SE, Briney CA, Shrimp JH, Alpsoy A, Thorpe AL, Bavari AE, Crooks DR, Levy M, Florens L, Washburn MP, Frizzell N, Dykhuizen EC, Weerapana E, Linehan WM, Meier JL.

Nat Chem Biol. 2019 Apr;15(4):391-400. doi: 10.1038/s41589-018-0217-y. Epub 2019 Feb 4.


Post-translational Regulation of FNIP1 Creates a Rheostat for the Molecular Chaperone Hsp90.

Sager RA, Woodford MR, Backe SJ, Makedon AM, Baker-Williams AJ, DiGregorio BT, Loiselle DR, Haystead TA, Zachara NE, Prodromou C, Bourboulia D, Schmidt LS, Linehan WM, Bratslavsky G, Mollapour M.

Cell Rep. 2019 Jan 29;26(5):1344-1356.e5. doi: 10.1016/j.celrep.2019.01.018.


Differences in Tumor VHL Mutation and Hypoxia-inducible Factor 2α Expression Between African American and White Patients with Clear Cell Renal Cell Carcinoma.

Callahan CL, Moore L, Lenz P, Linehan WM, Nickerson ML, Rothman N, Purdue MP.

Eur Urol. 2019 May;75(5):882-884. doi: 10.1016/j.eururo.2019.01.001. Epub 2019 Jan 15. No abstract available.


Germline mutations of renal cancer predisposition genes and clinical relevance in Chinese patients with sporadic, early-onset disease.

Wu J, Wang H, Ricketts CJ, Yang Y, Merino MJ, Zhang H, Shi G, Gan H, Linehan WM, Zhu Y, Ye D.

Cancer. 2019 Apr 1;125(7):1060-1069. doi: 10.1002/cncr.31908. Epub 2018 Dec 12.


CDC73 Germline Mutation in a Family With Mixed Epithelial and Stromal Tumors.

Vocke CD, Ricketts CJ, Ball MW, Schmidt LS, Metwalli AR, Middelton LA, Killian JK, Khan J, Meltzer PS, Simonds WF, Merino MJ, Linehan WM.

Urology. 2019 Feb;124:91-97. doi: 10.1016/j.urology.2018.11.013. Epub 2018 Nov 16.


The Warburg effect in hominis: isotope-resolved metabolism in ccRCC.

Crooks DR, Linehan WM.

Nat Rev Urol. 2018 Dec;15(12):731-732. doi: 10.1038/s41585-018-0110-1. No abstract available.


Metabolic Pathways in Kidney Cancer: Current Therapies and Future Directions.

Rathmell WK, Rathmell JC, Linehan WM.

J Clin Oncol. 2018 Oct 29:JCO2018792309. doi: 10.1200/JCO.2018.79.2309. [Epub ahead of print]


Cumulative Radiation Exposures from CT Screening and Surveillance Strategies for von Hippel-Lindau-associated Solid Pancreatic Tumors.

Tirosh A, Journy N, Folio LR, Lee C, Leite C, Yao J, Kovacs W, Linehan WM, Malayeri A, Kebebew E, Berrington de González A.

Radiology. 2019 Jan;290(1):116-124. doi: 10.1148/radiol.2018180687. Epub 2018 Oct 9.


Pathologic Oxidation of PTPN12 Underlies ABL1 Phosphorylation in Hereditary Leiomyomatosis and Renal Cell Carcinoma.

Xu Y, Taylor P, Andrade J, Ueberheide B, Shuch B, Glazer PM, Bindra RS, Moran MF, Linehan WM, Neel BG.

Cancer Res. 2018 Dec 1;78(23):6539-6548. doi: 10.1158/0008-5472.CAN-18-0901. Epub 2018 Oct 8.


Updated Recommendations on the Diagnosis, Management, and Clinical Trial Eligibility Criteria for Patients With Renal Medullary Carcinoma.

Msaouel P, Hong AL, Mullen EA, Atkins MB, Walker CL, Lee CH, Carden MA, Genovese G, Linehan WM, Rao P, Merino MJ, Grodman H, Dome JS, Fernandez CV, Geller JI, Apolo AB, Daw NC, Hodges HC, Moxey-Mims M, Wei D, Bottaro DP, Staehler M, Karam JA, Rathmell WK, Tannir NM.

Clin Genitourin Cancer. 2019 Feb;17(1):1-6. doi: 10.1016/j.clgc.2018.09.005. Epub 2018 Sep 12.


Evaluation of Recipients of Positive and Negative Secondary Findings Evaluations in a Hybrid CLIA-Research Sequencing Pilot.

Sapp JC, Johnston JJ, Driscoll K, Heidlebaugh AR, Miren Sagardia A, Dogbe DN, Umstead KL, Turbitt E, Alevizos I, Baron J, Bönnemann C, Brooks B, Donkervoort S, Jee YH, Linehan WM, McMahon FJ, Moss J, Mullikin JC, Nielsen D, Pelayo E, Remaley AT, Siegel R, Su H, Zarate C; NISC Comparative Sequencing Program, Manolio TA, Biesecker BB, Biesecker LG.

Am J Hum Genet. 2018 Sep 6;103(3):358-366. doi: 10.1016/j.ajhg.2018.07.018. Epub 2018 Aug 16.


Therapeutic Targeting of TFE3/IRS-1/PI3K/mTOR Axis in Translocation Renal Cell Carcinoma.

Damayanti NP, Budka JA, Khella HWZ, Ferris MW, Ku SY, Kauffman E, Wood AC, Ahmed K, Chintala VN, Adelaiye-Ogala R, Elbanna M, Orillion A, Chintala S, Kao C, Linehan WM, Yousef GM, Hollenhorst PC, Pili R.

Clin Cancer Res. 2018 Dec 1;24(23):5977-5989. doi: 10.1158/1078-0432.CCR-18-0269. Epub 2018 Jul 30.


The Cancer Genome Atlas Comprehensive Molecular Characterization of Renal Cell Carcinoma.

Ricketts CJ, De Cubas AA, Fan H, Smith CC, Lang M, Reznik E, Bowlby R, Gibb EA, Akbani R, Beroukhim R, Bottaro DP, Choueiri TK, Gibbs RA, Godwin AK, Haake S, Hakimi AA, Henske EP, Hsieh JJ, Ho TH, Kanchi RS, Krishnan B, Kwiatkowski DJ, Lui W, Merino MJ, Mills GB, Myers J, Nickerson ML, Reuter VE, Schmidt LS, Shelley CS, Shen H, Shuch B, Signoretti S, Srinivasan R, Tamboli P, Thomas G, Vincent BG, Vocke CD, Wheeler DA, Yang L, Kim WY, Robertson AG; Cancer Genome Atlas Research Network, Spellman PT, Rathmell WK, Linehan WM.

Cell Rep. 2018 Jun 19;23(12):3698. doi: 10.1016/j.celrep.2018.06.032. No abstract available.


The origin, evolution and route to metastasis of clear cell RCC.

Ricketts CJ, Linehan WM.

Nat Rev Nephrol. 2018 Sep;14(9):538-540. doi: 10.1038/s41581-018-0031-5. No abstract available.


BHD-associated kidney cancer exhibits unique molecular characteristics and a wide variety of variants in chromatin remodeling genes.

Hasumi H, Furuya M, Tatsuno K, Yamamoto S, Baba M, Hasumi Y, Isono Y, Suzuki K, Jikuya R, Otake S, Muraoka K, Osaka K, Hayashi N, Makiyama K, Miyoshi Y, Kondo K, Nakaigawa N, Kawahara T, Izumi K, Teranishi J, Yumura Y, Uemura H, Nagashima Y, Metwalli AR, Schmidt LS, Aburatani H, Linehan WM, Yao M.

Hum Mol Genet. 2018 May 14. doi: 10.1093/hmg/ddy181. [Epub ahead of print]


ONC201 kills breast cancer cells in vitro by targeting mitochondria.

Greer YE, Porat-Shliom N, Nagashima K, Stuelten C, Crooks D, Koparde VN, Gilbert SF, Islam C, Ubaldini A, Ji Y, Gattinoni L, Soheilian F, Wang X, Hafner M, Shetty J, Tran B, Jailwala P, Cam M, Lang M, Voeller D, Reinhold WC, Rajapakse V, Pommier Y, Weigert R, Linehan WM, Lipkowitz S.

Oncotarget. 2018 Apr 6;9(26):18454-18479. doi: 10.18632/oncotarget.24862. eCollection 2018 Apr 6.


Multi-regional Sequencing Elucidates the Evolution of Clear Cell Renal Cell Carcinoma.

Ricketts CJ, Linehan WM.

Cell. 2018 Apr 19;173(3):540-542. doi: 10.1016/j.cell.2018.03.077.


The Cancer Genome Atlas Comprehensive Molecular Characterization of Renal Cell Carcinoma.

Ricketts CJ, De Cubas AA, Fan H, Smith CC, Lang M, Reznik E, Bowlby R, Gibb EA, Akbani R, Beroukhim R, Bottaro DP, Choueiri TK, Gibbs RA, Godwin AK, Haake S, Hakimi AA, Henske EP, Hsieh JJ, Ho TH, Kanchi RS, Krishnan B, Kwiatkowski DJ, Lui W, Merino MJ, Mills GB, Myers J, Nickerson ML, Reuter VE, Schmidt LS, Shelley CS, Shen H, Shuch B, Signoretti S, Srinivasan R, Tamboli P, Thomas G, Vincent BG, Vocke CD, Wheeler DA, Yang L, Kim WY, Robertson AG; Cancer Genome Atlas Research Network, Spellman PT, Rathmell WK, Linehan WM.

Cell Rep. 2018 Apr 3;23(1):313-326.e5. doi: 10.1016/j.celrep.2018.03.075. Erratum in: Cell Rep. 2018 Jun 19;23(12):3698.


Germline VHL gene variant in patients with von Hippel-Lindau disease does not predict renal tumor growth.

Farhadi F, Nikpanah M, Li X, Symons R, Pourmorteza A, Merino MJ, Linehan WM, Malayeri AA.

Abdom Radiol (NY). 2018 Oct;43(10):2743-2749. doi: 10.1007/s00261-018-1540-1.


Acute loss of iron-sulfur clusters results in metabolic reprogramming and generation of lipid droplets in mammalian cells.

Crooks DR, Maio N, Lane AN, Jarnik M, Higashi RM, Haller RG, Yang Y, Fan TW, Linehan WM, Rouault TA.

J Biol Chem. 2018 May 25;293(21):8297-8311. doi: 10.1074/jbc.RA118.001885. Epub 2018 Mar 9.


Differentiating papillary type I RCC from clear cell RCC and oncocytoma: application of whole-lesion volumetric ADC measurement.

Paschall AK, Mirmomen SM, Symons R, Pourmorteza A, Gautam R, Sahai A, Dwyer AJ, Merino MJ, Metwalli AR, Linehan WM, Malayeri AA.

Abdom Radiol (NY). 2018 Sep;43(9):2424-2430. doi: 10.1007/s00261-017-1453-4.


In silico VHL Gene Mutation Analysis and Prognosis of Pancreatic Neuroendocrine Tumors in von Hippel-Lindau Disease.

Tirosh A, El Lakis M, Green P, Nockel P, Patel D, Nilubol N, Gara SK, Keutgen XM, Linehan WM, Kebebew E.

J Clin Endocrinol Metab. 2018 Apr 1;103(4):1631-1638. doi: 10.1210/jc.2017-02434.


Superiority of 68Ga-DOTATATE over 18F-FDG and anatomic imaging in the detection of succinate dehydrogenase mutation (SDHx )-related pheochromocytoma and paraganglioma in the pediatric population.

Jha A, Ling A, Millo C, Gupta G, Viana B, Lin FI, Herscovitch P, Adams KT, Taïeb D, Metwalli AR, Linehan WM, Brofferio A, Stratakis CA, Kebebew E, Lodish M, Civelek AC, Pacak K.

Eur J Nucl Med Mol Imaging. 2018 May;45(5):787-797. doi: 10.1007/s00259-017-3896-9. Epub 2017 Dec 4.


Mithramycin A Enhances Tumor Sensitivity to Mitotic Catastrophe Resulting From DNA Damage.

Scroggins BT, Burkeen J, White AO, Chung EJ, Wei D, Chung SI, Valle LF, Patil SS, McKay-Corkum G, Hudak KE, Linehan WM, Citrin DE.

Int J Radiat Oncol Biol Phys. 2018 Feb 1;100(2):344-352. doi: 10.1016/j.ijrobp.2017.09.049. Epub 2017 Oct 12.


Insights into Epigenetic Remodeling in VHL-Deficient Clear Cell Renal Cell Carcinoma.

Ricketts CJ, Linehan WM.

Cancer Discov. 2017 Nov;7(11):1221-1223. doi: 10.1158/2159-8290.CD-17-0971.


Association of VHL Genotype With Pancreatic Neuroendocrine Tumor Phenotype in Patients With von Hippel-Lindau Disease.

Tirosh A, Sadowski SM, Linehan WM, Libutti SK, Patel D, Nilubol N, Kebebew E.

JAMA Oncol. 2018 Jan 1;4(1):124-126. doi: 10.1001/jamaoncol.2017.3428. No abstract available.


FLCN: The causative gene for Birt-Hogg-Dubé syndrome.

Schmidt LS, Linehan WM.

Gene. 2018 Jan 15;640:28-42. doi: 10.1016/j.gene.2017.09.044. Epub 2017 Sep 29. Review.


Genomic and metabolic characterization of a chromophobe renal cell carcinoma cell line model (UOK276).

Yang Y, Vocke CD, Ricketts CJ, Wei D, Padilla-Nash HM, Lang M, Sourbier C, Killian JK, Boyle SL, Worrell R, Meltzer PS, Ried T, Merino MJ, Metwalli AR, Linehan WM.

Genes Chromosomes Cancer. 2017 Oct;56(10):719-729. doi: 10.1002/gcc.22476. Epub 2017 Jul 24.


Recommendations for the Management of Rare Kidney Cancers.

Giles RH, Choueiri TK, Heng DY, Albiges L, Hsieh JJ, Linehan WM, Pal S, Maskens D, Paseman B, Jonasch E, Malouf G, Molina AM, Pickering L, Shuch B, Srinivas S, Srinivasan R, Tannir NM, Bex A.

Eur Urol. 2017 Dec;72(6):974-983. doi: 10.1016/j.eururo.2017.06.040. Epub 2017 Jul 16. Review.


A mouse model of renal cell carcinoma.

Schmidt LS, Linehan WM.

Nat Med. 2017 Jul 11;23(7):802-803. doi: 10.1038/nm.4366. No abstract available.


Multiple Recurrent Paraganglioma in a Pediatric Patient with Germline SDH-B Mutation.

McGowan A, An JY, Tanakchi S, Maruf M, Muthigi A, George A, Su D, Merino MJ, Linehan WM, Boyle SL, Metwalli AR.

Urol Case Rep. 2017 Apr 27;13:107-109. doi: 10.1016/j.eucr.2017.03.017. eCollection 2017 Jul.


Managing Renal Cell Carcinoma Associated Paraneoplastic Syndrome with Nephron-sparing Surgery in a Patient with von Hippel-Lindau.

DiBianco JM, An JY, Tanakchi S, Stanik Z, McGowan A, Maruf M, Sidana A, Jain AL, Muthigi A, George AK, Bayne C, Linehan WM, Boyle SL, Metwalli AR.

Urol Case Rep. 2017 Apr 27;13:101-103. doi: 10.1016/j.eucr.2017.02.015. eCollection 2017 Jul.


Comprehensive genomic and phenotypic characterization of germline FH deletion in hereditary leiomyomatosis and renal cell carcinoma.

Vocke CD, Ricketts CJ, Merino MJ, Srinivasan R, Metwalli AR, Middelton LA, Peterson J, Yang Y, Linehan WM.

Genes Chromosomes Cancer. 2017 Jun;56(6):484-492. doi: 10.1002/gcc.22452. Epub 2017 Mar 31.


Discovering Targets of Non-enzymatic Acylation by Thioester Reactivity Profiling.

Kulkarni RA, Worth AJ, Zengeya TT, Shrimp JH, Garlick JM, Roberts AM, Montgomery DC, Sourbier C, Gibbs BK, Mesaros C, Tsai YC, Das S, Chan KC, Zhou M, Andresson T, Weissman AM, Linehan WM, Blair IA, Snyder NW, Meier JL.

Cell Chem Biol. 2017 Feb 16;24(2):231-242. doi: 10.1016/j.chembiol.2017.01.002. Epub 2017 Feb 2.


Kidney cancer in 2016: RCC - advances in targeted therapeutics and genomics.

Linehan WM, Ricketts CJ.

Nat Rev Urol. 2017 Feb;14(2):76-78. doi: 10.1038/nrurol.2016.260. Epub 2016 Dec 29. Review. No abstract available.


Co-opting a Bioorthogonal Reaction for Oncometabolite Detection.

Zengeya TT, Garlick JM, Kulkarni RA, Miley M, Roberts AM, Yang Y, Crooks DR, Sourbier C, Linehan WM, Meier JL.

J Am Chem Soc. 2016 Dec 14;138(49):15813-15816. Epub 2016 Nov 30.


Genetic predisposition to kidney cancer.

Schmidt LS, Linehan WM.

Semin Oncol. 2016 Oct;43(5):566-574. doi: 10.1053/j.seminoncol.2016.09.001. Epub 2016 Sep 22. Review.


Targeting HIF2α in Clear-Cell Renal Cell Carcinoma.

Ricketts CJ, Crooks DR, Linehan WM.

Cancer Cell. 2016 Oct 10;30(4):515-517. doi: 10.1016/j.ccell.2016.09.016.


Haploinsufficiency in tumor predisposition syndromes: altered genomic transcription in morphologically normal cells heterozygous for VHL or TSC mutation.

Peri S, Caretti E, Tricarico R, Devarajan K, Cheung M, Sementino E, Menges CW, Nicolas E, Vanderveer LA, Howard S, Conrad P, Crowell JA, Campbell KS, Ross EA, Godwin AK, Yeung AT, Clapper ML, Uzzo RG, Henske EP, Ricketts CJ, Vocke CD, Linehan WM, Testa JR, Bellacosa A, Kopelovich L, Knudson AG.

Oncotarget. 2017 Mar 14;8(11):17628-17642. doi: 10.18632/oncotarget.12192.

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