RCC

Question 1

What does Von Hippel-Lindau syndrome cause?

Answer 1

RCC, ANgiomatosis, haemangiomatosis, pheo

Question 2

What mutation causes Von Hippel- Lindau syndrome?

Answer 2

vHL

Question 3

What is the most common type of RCC?

Answer 3

Clear cell - 70%

Question 4

Which 3 cancers cause recurrence after many years of remission?

Answer 4

Breast, Melanoma, RCC

Question 5

What is the pathogenesis of RCC?

Answer 5

vHL tumour suppressor gene is inactivated (can be sporadic) which controls the Hypoxia inducible factor and usually degrades it, causing accumulation of HIF. This cause accumulation in angiogenic growth factors especially VEGF.

Question 6

Management of RCC?

Answer 6

1. Nephrectomy
2. Sunitinib or Pasopanib
   if this fails Sorafenib or mTOR e.g Evorlimus