Video interview: Dr W. Marston Linehan – National Cancer Institute, NIH, USA

Dr W. Marston Linehan is Chief of the Urologic Oncology Branch (UOB), which is part of the National Cancer Institute at the National Institutes of Health (NIH) in the United States. Dr Linehan has been at the NIH for 30 years, and is one of the world’s foremost experts in researching and treating genitourinary cancers. In recognition of this, Dr Linehan has received numerous honours, such as the Dr. Nathan Davis Award for Outstanding Government Service from the American Medical Association, and the Joseph H. Burchenal Memorial Award for Outstanding Achievement in Clinical Cancer Research from the American Association of Cancer Research.

During his distinguished career, Dr Linehan has played a major role in identifying the genes associated with Von Hippel–Lindau disease (VHL – Latif et al., 1993), hereditary papillary renal carcinoma (MET – Schmidt et al., 1997) and Birt-Hogg-Dubé syndrome (FLCN – Nickerson et al., 2002). His work has also focussed on hereditary leiomyomatosis and renal cell carcinoma (HLRCC), which is characterised by mutations in the FH gene. In particular, a new cellular model of HLRCC has recently been developed by the Linehan lab (Yang et al., 2012), data from which adds to the idea that kidney cancer is essentially a metabolic disease (Linehan & Ricketts, 2012).

With this knowledge and his surgical background, Dr Linehan has also helped inform approaches for the clinical and surgical management of such disorders (Barrisford et al., 2011Linehan 2012). Consequently, his contribution to the Fourth BHD Symposium regarding the treatment and management of BHD-associated kidney cancer was both greatly appreciated and invaluable. Moreover, his lab is conducting a clinical study entitled the “Genetic Analysis of Birt-Hogg-Dubé Syndrome and Characterization of Predisposition to Kidney Cancer”, which is currently recruiting participants.

For more information regarding Dr Linehan’s BHD research and the work of the UOB, please do look at the blog profiles of Dr Laura SchmidtDr Masaya Baba and Lindsay Middelton. In addition, learn more about his thoughts regarding BHD syndrome and its treatment by watching our video interview (with its associated audio-only file and transcript). The following publications will also be of interest:

  • Baba M, Keller JR, Sun HW, Resch W, Kuchen S, Suh HC, Hasumi H, Hasumi Y, Kieffer-Kwon KR, Gonzalez CG, Hughes RM, Klein ME, Oh HF, Bible P, Southon E, Tessarollo L, Schmidt LS, Linehan WM, & Casellas R (2012). The folliculin-FNIP1 pathway deleted in human Birt-Hogg-Dubé syndrome is required for murine B-cell development. Blood, 120 (6), 1254-61 PMID: 22709692
  • Barrisford GW, Singer EA, Rosner IL, Linehan WM, & Bratslavsky G (2011). Familial renal cancer: molecular genetics and surgical management. International journal of surgical oncology, 2011 PMID: 22312516
  • Latif F, Tory K, Gnarra J, Yao M, Duh FM, Orcutt ML, Stackhouse T, Kuzmin I, Modi W, & Geil L (1993). Identification of the von Hippel-Lindau disease tumor suppressor gene. Science (New York, N.Y.), 260 (5112), 1317-20 PMID:8493574
  • Linehan WM (2012). Genetic basis of kidney cancer: Role of genomics for the development of disease-based therapeutics. Genome research PMID: 23038766
  • Linehan WM, & Ricketts CJ (2012). The metabolic basis of kidney cancer.Seminars in cancer biology PMID: 22705279
  • Nickerson ML, Warren MB, Toro JR, Matrosova V, Glenn G, Turner ML, Duray P, Merino M, Choyke P, Pavlovich CP, Sharma N, Walther M, Munroe D, Hill R, Maher E, Greenberg C, Lerman MI, Linehan WM, Zbar B, & Schmidt LS (2002). 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. Cancer cell, 2 (2), 157-64 PMID: 12204536
  • Schmidt L, Duh FM, Chen F, Kishida T, Glenn G, Choyke P, Scherer SW, Zhuang Z, Lubensky I, Dean M, Allikmets R, Chidambaram A, Bergerheim UR, Feltis JT, Casadevall C, Zamarron A, Bernues M, Richard S, Lips CJ, Walther MM, Tsui LC, Geil L, Orcutt ML, Stackhouse T, Lipan J, Slife L, Brauch H, Decker J, Niehans G, Hughson MD, Moch H, Storkel S, Lerman MI, Linehan WM, & Zbar B (1997). Germline and somatic mutations in the tyrosine kinase domain of the MET proto-oncogene in papillary renal carcinomas. Nature genetics, 16 (1), 68-73 PMID:9140397
  • Yang Y, Valera V, Sourbier C, Vocke CD, Wei M, Pike L, Huang Y, Merino MA, Bratslavsky G, Wu M, Ricketts CJ, & Linehan WM (2012). A novel fumarate hydratase-deficient HLRCC kidney cancer cell line, UOK268: a model of the Warburg effect in cancer. Cancer genetics, 205 (7-8), 377-90 PMID: 22867999

www.bhdsyndrome.org – the primary online resource for anyone interested in BHD Syndrome.

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