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neo_renal

renal malignancies

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
    • 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%)
    • night sweats
    • malaise
    • varicocele
  • paraneoplastic syndromes are common including:
  • 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 17:17 (external edit)