title: Structured assessment and followup for patients with hereditary kidney tumour syndromes.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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-Dubé syndrome (BHD), familial paraganglioma-pheochromocytoma syndromes (PGL-PCC), and tuberous sclerosis (TSC) are proposed.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Division of Urology, Department of Surgery, University of Montreal Hospital Centre, Montreal, QC, Canada.
authors.forename: Jean-Baptiste
authors.initials: JB
authors.lastname: Lattouf
authors.affiliation: Divisions of Urology and Surgical Oncology, Departments of Surgery and Oncology, Western University, London, ON, Canada.
authors.forename: Stephen E
authors.initials: SE
authors.lastname: Pautler
authors.affiliation: Division of Medical Oncology, The Ottawa Hospital Cancer Centre, University of Ottawa, Ottawa, ON, Canada.
authors.forename: M Neil
authors.initials: MN
authors.lastname: Reaume
authors.affiliation: Division of Medical Oncology, Department of Medicine, Princess Margaret Cancer Centre, University Health Network, University of Toronto, Toronto, ON, Canada.
authors.forename: Raymond H
authors.initials: RH
authors.lastname: Kim
authors.affiliation: Fred A. Litwin Family Centre in Genetic Medicine, University Health Network & Mount Sinai Hospital, Toronto, ON, Canada.
authors.forename: Melanie
authors.initials: M
authors.lastname: Care
authors.affiliation: Disciplines of Genetics and Medicine, Faculty of Medicine, Memorial University of Newfoundland, St. John's, NL, Canada.
authors.forename: Jane
authors.initials: J
authors.lastname: Green
authors.affiliation: Department of Urologic Sciences, Faculty of Medicine, Vancouver General Hospital and University of British Columbia, Vancouver, BC, Canada.
authors.forename: Alan
authors.initials: A
authors.lastname: So
authors.affiliation: Division of Urology, Department of Surgery, Woodstock General Hospital, Woodstock, ON, Canada.
authors.forename: Philippe D
authors.initials: PD
authors.lastname: Violette
authors.affiliation: Division of ENT, Department of Surgery, University of Montreal Hospital Centre, Montreal, QC, Canada.
authors.forename: Issam
authors.initials: I
authors.lastname: Saliba
authors.affiliation: CHU Sainte-Justine, Department of Neurosciences, University of Montreal, Montreal, QC, Canada.
authors.forename: Philippe
authors.initials: P
authors.lastname: Major
authors.affiliation: Faculty of Medicine and the Division of Dermatology, University of Manitoba, Winnipeg, MB, Canada.
authors.forename: Shane
authors.initials: S
authors.lastname: Silver
authors.affiliation: Department of Ophthalmology, Faculty of Medicine, University of Manitoba, Winnipeg, MB, Canada.
authors.forename: Richard
authors.initials: R
authors.lastname: Leicht
authors.affiliation: Kidney Cancer Research Network of Canada, Toronto, ON, Canada.
authors.forename: Joan
authors.initials: J
authors.lastname: Basiuk
authors.affiliation: Division of Urology, McGill University, Montreal, QC, Canada.
authors.forename: Simon
authors.initials: S
authors.lastname: Tanguay
authors.affiliation: Division of Urology, Departments of Surgical Oncology and Surgery, Princess Margaret Cancer Centre and the University Health Network, University of Toronto, Toronto, ON, Canada.
authors.forename: Michael A S
authors.initials: MA
authors.lastname: Jewett
authors.affiliation: Section of Urology, Department of Surgery, University of Manitoba, Winnipeg, MB, Canada.
authors.forename: Darrel
authors.initials: D
authors.lastname: Drachenberg