Hereditary kidney tumors are a rare entity of kidney tumors, which represents 2-3% or 1 in 36000 births.
Currently a decade of diseases have been described, six of which were detailed in our thesis: Von Hippel-Lindau disease, tuberous sclerosis, Birt-Hogg-Dubé syndrome, hereditary leiomyomatosis and cell carcinoma, hereditary papillary renal carcinoma, renal cell carcinoma associated with chromosome 3 translocations.
Epidemiologically, these tumors are characterized by their occurrence in early age to the 2nd and 3rd decades, and even in childhood. They have no particular gender predominance.
Despite the great similarities in clinical and radiology with the sporadics forms, hereditary kidney tumors are characterized by the presence of similar cases in the family, association with other systemic manifestations, and they are bilateral, multifocal and present a high risk of recurrence. All these elements make their diagnostic suspicion before conducting anatomopathological examination or genetic test. Several histological types have been associated with these entities; they can be malignant or benign tumors, with a clear predominance of clear cell carcinomas. Because of genetic progress in the last decade, most of the genes responsible for these tumors were identified. However, the function of some of them remains misunderstood, and other studies are necessary in the future to understand their mechanisms.
The main purpose of the management of familial forms of renal tumors is to prevent the occurrence of metastasis and preserve the maximum nephron capital (because they are bilateral, multifocal and recurrent tumors) to keep proper kidney function and avoid as long as possible the use of renal dialysis. Other feature of the management of these tumors is close monitoring of patients, because the high risk of occurrence and involvement of other organs. We must not forget also the genetic counseling of patients and their families to sensitized to early detection of other similar cases in their family and in future generations.
The aim of our study is to report the latest bibliographic data on hereditary renal tumors to better know these entities, and to draw practical conclusions. And also, encourage close collaboration between urologists and geneticists in the CHU Hassan II of Fez, to better diagnose these diseases, in order to provide optimal care to our patients.