congenital mesoblastic nephroma - usually found prenatal or before 3 months of age
see also wikipedia for other types of kidney cancer
while most renal cysts can just be observed, a major medical pitfall is ignoring a solid renal mass and failing to provide appropriate follow-up care
renal cell carcinoma (RCC)
90-95% of renal malignancies
3% of all adult malignancies
usually presents late
median age at diagnosis is 64yrs but may occur in much younger, particularly if familial clustering
after radical nephrectomy for stage I renal cell carcinoma, the 5-year survival rate is approximately 94%.
patients with stage II lesions have a survival rate of 79%.
aetiology
arises from the proximal renal tubular epithelium
more common in people of Northern European ancestry and North Americans than in those of Asian or African descent.
sporadic (nonhereditary) and a hereditary form, and both forms are associated with structural alterations of the short arm of chromosome 3 (3p)
at least 4 hereditary syndromes associated with renal cell carcinoma are recognized:
Von Hippel-Lindau (VHL) syndrome
AD with increased risk of phaeochromocytoma, pancreatic cysts and islet cell tumours, retinal angiomas, CNS haemangioblastomas, endolymphatic sac tumours, epididymal cystadenomas as well as 40% developing RCC
familial renal oncocytoma (FRO) associated with Birt-Hogg-Dube syndrome (BHDS)
hereditary renal carcinoma (HRC)
hereditary leiomyomatosis and renal cell cancer (HLRCC)
100 families worldwide
cutaneous leiomyomas typically develop in the third decade of life
most women develop (uterine fibroids (leiomyomas) - generally larger, more numerous and at an earlier age than usual
10-16% develop renal cell cancer
AD genetics due to fumarate hydratase FH gene
environmental risk factors:
smoking ⇒ 2x risk in a dose-dependent manner
obesity ⇒linear risk with body weight, esp. women
hypertension
long term dialysis ⇒ acquired cystic disease of kidney ⇒ 30x increased risk RCC
hysterectomy ⇒ 2x risk
?tuberous sclerosis
renal transplant recipients
PFOA contamination in blood over over 30ppb possibly doubles risk of kidney cancers
clinical features
usually presents late, with 30% having mets at diagnosis, and 30% will have spread into ipsilateral renal vein, while 5-10% this will have already spread to the IVC
classic triad of flank pain, hematuria, and flank mass only occurs in 10% at diagnosis
25-30% are found incidentally on radiologic examination
urothelial cell carcinoma (transitional cell carcinomas or TCCs)
the most common type of cancer of the urinary bladder, ureters, urethra and urachus in the developed world (SCC is more common in un-developed countries) but only accounts for 5-10% of all primary urologic cancers
TCCs are often multifocal with 30-40% having more than one tumour at diagnosis
they may be papillary or sessile
40-50% have invasive disease at diagnosis
risk factors
smoking contributes 50% of risk
chemical exposure - petroleum workers, aniline dyes, agrochemicals
medications eg. cyclophosphamide, phenacetin
long urine dwell time in the bladder
long haul truck drivers
inadequate daily fluid intake
chronic physical irritation such as chronic urinary stone disease, chronic IDC, chronic UTIs