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NDT Advance Access originally published online on October 26, 2004
Nephrology Dialysis Transplantation 2004 19(12):2954-2958; doi:10.1093/ndt/gfh535
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Nephrol Dial Transplant Vol. 19 No. 12 © ERA-EDTA 2004; all rights reserved

Editorial Comment

The growing family of hereditary renal cell carcinoma

Stéphane Richard1,2, Rosette Lidereau3 and Sophie Giraud1,4 on behalf of the French inherited kidney tumours consortium

1 Laboratoire de Génétique Oncologique EPHE Faculté de Médecine Paris-Sud-UMR 8125 (Institut Gustave Roussy) and Service d’Urologie, CHU de Bicêtre, Le Kremlin-Bicêtre, 2 Service de Néphrologie, Hôpital Necker, 75743 Paris, 3 Laboratoire de Génétique, INSERM E0017, Centre René Huguenin, Saint-Cloud and 4 Laboratoire de Génétique, Hôpital Edouard Herriot, Lyon, France

Correspondence and offprint requests to: Professor Stéphane Richard, Génétique Oncologique EPHE, Faculté de Médecine Paris-Sud, 94276 Le Kremlin-Bicêtre, France. Email: stephane.richard@kb.u-psud.fr

Keywords: Birt–Hogg–Dubé (BHD); chromosome 3 translocations; fumarate hydratase; hereditary renal cell carcinoma; MET proto-oncogene; von Hippel–Lindau (VHL)

The first 150 words of the full text of this article appear below.



   Introduction
 
Renal cell carcinoma (RCC) accounts for ~3% of human malignancies and its incidence appears to be rising [1]. RCC is diagnosed in ~150 000 people each year worldwide and 78 000 die from the disease [1]. The main histological subtypes of renal epithelial tumours include clear cell RCC (75%), papillary RCC (10–15%), chromophobe RCC (5%) and oncocytomas (5%) [2]. Complete surgical resection is considered to be curative for localized RCC, but there is no effective treatment for metastatic disease that is already present in 30% of cases at diagnosis [1]. Most cases of RCC are thought to be sporadic; smoking, obesity and occupational exposures are the main known risk factors [1]. Hereditary RCC syndromes are estimated at <3% but have major clinical and scientific implications [3,4]. First, the identification of predisposing genes offers the possibility of . . . [Full Text of this Article]



   Familial clear cell RCC
 
Von Hippel–Lindau (VHL) disease (OMIM 193300)
Constitutional chromosome 3 translocations
Familial clear cell renal cell cancer (FCRC)
SDHB-associated heritable paraganglioma (OMIM 185470 and 605373)
Tuberous sclerosis (OMIM 191100)


   Familial papillary RCC
 
Hereditary papillary RCC (HPRC) (OMIM 605074)
Hereditary leiomyomatosis renal cell cancer (HLRCC) (OMIM 605839)
Hyperparathyroidism–jaw tumour (HPT-JT) (OMIM 145001)
Papillary thyroid carcinoma with associated renal neoplasia (FPTC-PRN) (OMIM 605642)


   Familial chromophobe RCC and oncocytomas
 
Birt–Hogg–Dubé syndrome (BHD) (OMIM 135150)


   Diagnostic and therapeutic recommendations
 


   Future directions
 

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