Diagnosis and management of rare tumors – A structured approach

Diagnosis and management of rare tumors – A structured approach

Diagnosis and management of rare tumors – A structured approach (#2)

Derek Raghavan ¹

Levine Cancer Institute, Charlotte, NORTH CAROLINA, United States

Using a cut-off figure of 15 new cases/100,000 population/year as a definition for rare cancers, it appears that these tumors account for up to 25% of incident cases in the USA (1). With a more rigorous criterion of less than 6/100,000, the figure is much less, but it appears that outcomes are worse than for common cancers (2). There is also masking of some rare situations (viz. uncommon presentations of common cancers – e.g. small cell anaplastic cancers of bladder or prostate) (3,4). Key items in a structured approach include histological review by an expert tumor pathologist, clinical review of purported unusual imaging or other diagnostic results, careful literature review from expert sources, referral to a centre of excellence for a second opinion from a published expert, and development of a management partnership with that expert. In an evolving era of molecular diagnostics, gene expression analyses may add clarity to the precision of diagnosis and may provide therapeutic targets in uncommon cancers. Much caution should be exercised when relying upon isolated case reports and literature reviews, which may involve data dredging and recycling, unconfirmed pathological diagnoses, editorial bias against negative reporting and production by the most inexperienced clinicians. Examples of structured approaches to management of collecting duct cancer and medullary carcinoma of kidney (3-6), and small cell anaplastic bladder cancer are reviewed as illustrations of a structured approach.

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Gatta G et al, Eur. J. Cancer, 2011, 47: 2943-2511
Raghavan D et al (eds), Textbook of Uncommon Cancer, 2012, Wiley-Blackwell, Hoboken, Chichester.
Raghavan D, Ann. Oncol., 2013, 24: 2932-4
Ali K et al, Uncommon cancers of the kidney, in Raghavan D et al, ibid, pp. 1-21.
Pecuchet N et al, Ann. Oncol., 2013, 24: 2963-7

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Authors contributing to this presentation.

Raghavan, D

Dr. Raghavan, President of the Levine Cancer Institute, Charlotte, North Carolina, has served at senior level in oncology for more than 25 years, including roles as Director & Frank Rudy Distinguished Chair, Taussig Cancer Institute, Cleveland Clinic, Chair of Medical Oncology & Associate Director (Clinical Research), University of Southern California Norris Comprehensive Cancer Center, and Professor & Chair of Solid Tumor Oncology/Investigational Therapeutics, Roswell Park Cancer Institute. Trained initially in Australia, Dr. Raghavan completed a PhD at the University of London, and undertook post-doctoral research at the University of Minnesota. Upon his return to Australia, he developed a new paradigm of neoadjuvant therapy for bladder cancer, accompanied by the development of a series of preclinical models of heterogeneity and stem cell function in bladder cancer and prostate cancer. In the United States, he has served as Chair of the National Cancer Institute Cancer (NCI) Clinical Investigations Review Committee, Chair of the Veterans Administration Merit Review Board in Oncology, and has been a member of the FDA Oncology Drug Advisory Committee and the NCI Clinical Oncology Review Section. Dr. Raghavan has been Chair of the Cancer Communications Committee and the Health Disparities Advisory Group of the American Society of Clinical Oncology, a member of the ASCO Value Task Force, was a member of the Scientific Audit Committee of the EORTC for 7 years, has advised Cancer Research UK and the Irish Cancer Society, and the Italian Agency for Cancer Research, has been a member of the Scientific Advisory Board and the Board of Governors of Southwest Oncology Group, and has sat on the International Advisory Board of Deutsche Krebshilfe for more than 5 years. He has previously been the Chairman of the Medical Oncology Group of Australia and Chair of the Specialist Advisory Committee in Oncology of the Royal Australasian College of Physicians. Dr. Raghavan has published more than 250 papers and 12 books, with areas of focus including heterogeneity of tumor function, biology and management of cancers of the bladder, testis and prostate, new drug development in lung cancer, and geriatric oncology. He has been a member of editorial boards of Journal of Clinical Oncology, Clinical Cancer Research, European Journal of Cancer, Annals of Oncology, and many others, and has served on the external scientific advisory boards of 10 comprehensive cancer centers. He is the Editor-in-Chief (Medical Oncology) of HemOnc Today. He received the Frank Moran Award for Leadership in Cancer Research from the University of Michigan in 2003, was elected a Fellow of the American Association for the Advancement of Science in 2009, and a Fellow of the American Society of Clinical Oncology in 2010, and received a Statesman Award from ASCO in 2010. He was awarded a research doctorate in medicine (MD) from the University of Sydney in 2012.

Diagnosis and management of rare tumors – A structured approach (#2)

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