Optimal clinical assessment and subsequent followup of patients with or suspected of having a hereditary renal cell carcinoma syndrome (hRCC) is not standardized and practice varies widely. We propose protocols to optimize these processes in patients with hRCC to encourage a more uniform approach to management that can then be evaluated.

A review of the literature, including existing guidelines, was carried out for the years 1985-2015. Expert consensus was used to define recommendations for initial assessment and followup.

Recommendations for newly diagnosed patients' assessment and optimal ages to initiate followup protocols for von Hippel Lindau disease (VHL), hereditary papillary renal cancer (HPRC), hereditary leiomyomatosis with renal cell carcinoma (HLRCC), Birt-Hogg-Dub syndrome (BHD), familial paraganglioma-pheochromocytoma syndromes (PGL-PCC), and tuberous sclerosis (TSC) are proposed.

Our proposed consensus for structured assessment and followup is intended as a roadmap for the care of patients with hRCC to guide healthcare providers. Although the list of syndromes included is not exhaustive, the document serves as a starting point for future updates.

Canadian Urological Association journal = Journal de l'Association des urologues du Canada. 2016 Jul 12 [Epub]

Jean-Baptiste Lattouf, Stephen E Pautler, M Neil Reaume, Raymond H Kim, Melanie Care, Jane Green, Alan So, Philippe D Violette, Issam Saliba, Philippe Major, Shane Silver, Richard Leicht, Joan Basiuk, Simon Tanguay, Michael A S Jewett, Darrel Drachenberg, Kidney Cancer Research Network of Canada

Division of Urology, Department of Surgery, University of Montreal Hospital Centre, Montreal, QC, Canada., Divisions of Urology and Surgical Oncology, Departments of Surgery and Oncology, Western University, London, ON, Canada., Division of Medical Oncology, The Ottawa Hospital Cancer Centre, University of Ottawa, Ottawa, ON, Canada., Division of Medical Oncology, Department of Medicine, Princess Margaret Cancer Centre, University Health Network, University of Toronto, Toronto, ON, Canada., Fred A. Litwin Family Centre in Genetic Medicine, University Health Network & Mount Sinai Hospital, Toronto, ON, Canada., Disciplines of Genetics and Medicine, Faculty of Medicine, Memorial University of Newfoundland, St. John's, NL, Canada., Department of Urologic Sciences, Faculty of Medicine, Vancouver General Hospital and University of British Columbia, Vancouver, BC, Canada., Division of Urology, Department of Surgery, Woodstock General Hospital, Woodstock, ON, Canada., Division of ENT, Department of Surgery, University of Montreal Hospital Centre, Montreal, QC, Canada., CHU Sainte-Justine, Department of Neurosciences, University of Montreal, Montreal, QC, Canada., Faculty of Medicine and the Division of Dermatology, University of Manitoba, Winnipeg, MB, Canada., Department of Ophthalmology, Faculty of Medicine, University of Manitoba, Winnipeg, MB, Canada., Kidney Cancer Research Network of Canada, Toronto, ON, Canada., Division of Urology, McGill University, Montreal, QC, Canada., Division of Urology, Departments of Surgical Oncology and Surgery, Princess Margaret Cancer Centre and the University Health Network, University of Toronto, Toronto, ON, Canada., Section of Urology, Department of Surgery, University of Manitoba, Winnipeg, MB, Canada.

PubMed http://www.ncbi.nlm.nih.gov/pubmed/28255411

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