Renal cell carcinoma Flashcards
Why has there been an apparent increase in RCC cases?
Due to the increased use of USS and CT leading to accidental findings of early tumours.
Why does RCC normally present late?
Signs and symptoms are not experienced until the tumour is large.
What is the most common form of RCC?
Adenocarcinoma of the glandular epithelium e.g. tubule parenchyma
What is paediatric RCC known as?
Nephroblastoma or Wilm’s
What is the two main types of RCC?
Clear cell carcinoma
Sporadic (most common)
How does RCCC present?
Diagnostic triad:
Haematuria
Flank pain
Abdominal mass
Weight loss, anaemia, fever, metastatic symptoms
How does RCCC appear histologically?
Empty cytoplasm as the lipids and glycogen dissolve during tissue processing
What is the familial link of RCCC?
Von Hippel Lindau syndrome - Germline mutation in VHL TSG that is involved in the HIF pathway. Affects both kidneys.
How early does VHL syndrome present?
Early due to germline dominant mutation.
What type of cancers are associated with VHL syndrome?
Blastoma of CNS, retina, pancreas, renal cysts, renal tumours. Multiple tumours within one kidney.
How does a mutation in the HIF pathway lead to development of cancer?
VHL mutation or hypoxia leads to the accumulation of HIFalpha (hypoxia induced factor) that upregulates VEGF, PDGF, TGFalpha. The factors then promote tumourgenesis through angiogenesis, glucose transport, autocrine growth stimulation.
When does sporadic RCC present?
Late due to non germline, aberrant VHL gene mutation from loss of expression of loss of function due to a base substitution.
How does sporadic RCC present?
Single tumour in kideney
How is sporadic RCC treated?
Radical nephrectomy including adrenal and lymph.
What characteristics will increase the severity of staging?
Necrosis and invasion outside of renal capsule.
Spread to adrenal or vascular invasion =T
Metastasis = M
