neo_renal
Table of Contents
renal malignancies
see also:
introduction
- most malignant tumours of the kidney in adults are renal cell carcinoma
- the other main primary cancer in adults is urothelial cell carcinoma (UCC) of the renal pelvis (transitional cell carcinoma or TCC)
- paediatric cancers of the kidney include:
- Wilm's tumour or nephroblastoma
- most occur in ages 3-3.5yrs with children of African descent having highest incidence
- see wikipedia
- 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
- hereditary papillary renal carcinoma (HPRC)
- AD, individuals develop bilateral, multifocal papillary renal carcinoma
- 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 leiomyomas (fibroids) - 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
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
- common presentations include:
- haematuria (40%)
- flank pain (40%)
- palpable mass (25%)
- LOW (33%)
- fever (20%)
- hypertension (20%)
- hypercalcaemia (5%)
- night sweats
- malaise
- varicocele
- paraneoplastic syndromes are common including:
- erythrocytosis
- liver dysfunction
- polyneuromyopathy
- fever, cachexia, LOW, dermatomyositis, raised erythrocyte sedimentation rate (ESR), hypertension
- metastases:
- lung (75%)
- soft tissues (36%)
- bone (20%)
- liver (18%)
- skin and CNS (8% each)
- supraclavicular LN's
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
- radiation exposure
- genetic factors
common sites of metastasis
- bone (35%) - 40% of these are in the spine
neo_renal.txt · Last modified: 2019/04/07 07:17 by 127.0.0.1