renal Flashcards
What are the commonest histologies of renal cell carcinoma
Clear cell
Papillary
Chromophobe
What mutation is typically seen in clear cell renal carcinoma & on what chromosome
VHL alterations (chr 3)
What tumour is associated with a VHL mutation
Phaeochromocytoma
What is associated with VHL disease
What is the inheritance
AD
Haemangioblastoma of brain, spine and retina Pancreatic and renal cysts
Neuroendocrine tumours
Endolympatic sac tumours
Phaeochromocytoma
How are renal cell carcinomas graded and for which histologies
Fuhrman grading 1-4
Clear cell and papillary only
What must be excluded in renal cell cancers <40yrs
Micro-ophthalmia associated transcription factor (MiT) alterations
How much is the risk of renal cell carcinoma increased by due to dialysis
x30
What genetic syndromes increase the risk of renal cell carcinoma
VHL disease (AD)
Tuberous sclerosis (AD)
Adult PKD (AD)
Hereditary leiomyomatosis and renal cell cancer (HLRCC)
Hereditary papillary renal cell carcinoma (AD)
What paraneoplastic syndromes occur with renal cell carcinoma
Secretion of:
PTHrP -> hyper-Ca
Renin -> htn
EPO -> polycythaemia
IL6 -> hepatic dysfunction with raised ALP (cholestasis) -> stauffer syndrome
What scoring system predicts the risk of metastatic disease following nephrectomy
What factors are included
Leibovitch scoring system
Includes: stage, nodal involvement, tumour size, grade, histological necrosis (Y/N)
How is a T1 renal tumour defined
Tumour confined to the kidney and is <7cm
T1a - <4cm
T1b - 4-7cm
How is a T2 renal tumour defined
Tumour confined to the kidney but >7cm
T2a - 7-10cm
T2b - >10cm
How is a T3 renal tumour defined
Tumour invades into perinephric tissues or renal vein, but not the adrenal or beyond Gerota’s fascia
T3a - into renal vein or perirenal fat
T3b - tumour extends into vena cava below diaphragm
T3c - tumour extends into vena cava above the diaphragm or invades the wall of the vena cava
How is a T4 renal tumour defined
Spread beyond Gerota’s fascia, incl invasion into adrenal gland
How is stage 3 renal cell carcinoma defined
T3 disease (invasion into perinephric fat or renal vein) or node positive
How is stage 4 renal cell carcinoma defined
T4 disease (invasion into adrenal or beyond Gerota’s fascia) or distant mets
How is a stage 1 (T1N0) renal carcinoma treated
Partial nephrectomy
Or RFA if not suitable for surgery
How is a stage 2 (T2 N0) renal cell carcinoma treated
Lap radical nephrectomy
How is a stage 3 (T1-3 N1, T3N0) treated
Open radical nephrectomy +/- lymphadenectomy
Radical nephrectomy - Remove kidney, adrenal, peri-renal fat, Gerota’s fascia +/- LN dissection.
When is adjuvant treatment indicated for renal cell carcinoma
Adjuvant pembrolizumab for 1yr following nephrectomy or nephrectomy and removal of all metastatic disease, for intermediate-high and high-risk clear cell RCC
pT2 & Fuhrman grade 4 or sarcomatoid histology (intermediate-high risk) or
pT3 or t4
What is a cytoreductive nephrectomy considered for the treatment for metastatic renal cell carcinoma
Indicated if: Good PS, large tumours (esp symptomatic), favourable/intermediate risk, limited metastatic disease
Not if: nephrectomy would delay systemic treatment for symptomatic pts, asymptomatic primary for int/poor risk pts, poor PS
When is a secondary cytoreductive radical nephrectomy indicated
Pts with local symptoms due to the primary tumour or near-complete responses to systemic therapy
When is local metastasis-directed treatment indicated
Good PS
Metachronous disease with disease free interval >2yrs
Solitary or oligomet
Absence of progression on systemic treatment
Low or intermediate grade and complete resection of primary tumour
What scoring system quantifies prognosis of metastatic renal cell carcinoma
What is included
What are the categories
IMDC / Heng criteria
PS >2 / KPS<80%
Hb <12
Diagnosis to development of mets <12mths
Corrected calcium > ULN
Platelets > ULN
Neutrophils > ULN
Categories:
Favourable risk - 0 factors
Intermediate risk 1-2 factors
Poor risk - ≥3 factors
How is the first line management of clear cell renal cell carcinoma determined
What are they
According to risk
Favourable risk:
- Single agent TKI - pazo, tivo
- Axitinib & avelumab
- 2nd line nivo or TKI or lenva/evero
Int / poor risk:
- IO - ipi/nivo (preferred 1st line if no immediate response needed
- TKI/IO combination - lenvatinib / pembro, avelumab/axitinib, cabo/nivo
- tki only - cabo
What is the second line management of ccRCC
If first line TKI -> nivolumab, different TKI (cabozantinib, axitinib), lenvatinib/everolimus or single agent everolimus
If first line ipi/nivo -> TKI, lenvatinib/everolimus
If first line lenvatinib/pembro -> TKI
What is the preferred first line treatment for non-clear cell RCC
Cabozantinib
Or axitinib or avelumab
when can pazopanib and tivozanib be used in metastatic RCC
first line only
what is the risk of renal cysts being malignant, and based on what score
Bosniak scoring system (based on Hounsfield units)
2F = minimally complex -> repeat imaging 6/12
3 = indeterminate -> partial nephrectomy
4 = 90% malignant -> partial/total nephrectomy
