renal

Question 1

What are the commonest histologies of renal cell carcinoma

Answer 1

Clear cell

Papillary

Chromophobe

Question 2

What mutation is typically seen in clear cell renal carcinoma & on what chromosome

Answer 2

VHL alterations (chr 3)

Question 3

What tumour is associated with a VHL mutation

Answer 3

Phaeochromocytoma

Question 4

What is associated with VHL disease
What is the inheritance

Answer 4

AD
Haemangioblastoma of brain, spine and retina Pancreatic and renal cysts
Neuroendocrine tumours
Endolympatic sac tumours
Phaeochromocytoma

Question 5

How are renal cell carcinomas graded and for which histologies

Answer 5

Fuhrman grading 1-4
Clear cell and papillary only

Question 6

What must be excluded in renal cell cancers <40yrs

Answer 6

Micro-ophthalmia associated transcription factor (MiT) alterations

Question 7

How much is the risk of renal cell carcinoma increased by due to dialysis

Answer 7

x30

Question 8

What genetic syndromes increase the risk of renal cell carcinoma

Answer 8

VHL disease (AD)
Tuberous sclerosis (AD)
Adult PKD (AD)
Hereditary leiomyomatosis and renal cell cancer (HLRCC)
Hereditary papillary renal cell carcinoma (AD)

Question 9

What paraneoplastic syndromes occur with renal cell carcinoma

Answer 9

Secretion of:
PTHrP -> hyper-Ca
Renin -> htn
EPO -> polycythaemia
IL6 -> hepatic dysfunction with raised ALP (cholestasis) -> stauffer syndrome

Question 10

What scoring system predicts the risk of metastatic disease following nephrectomy
What factors are included

Answer 10

Leibovitch scoring system
Includes: stage, nodal involvement, tumour size, grade, histological necrosis (Y/N)

Question 11

How is a T1 renal tumour defined

Answer 11

Tumour confined to the kidney and is <7cm
T1a - <4cm
T1b - 4-7cm

Question 12

How is a T2 renal tumour defined

Answer 12

Tumour confined to the kidney but >7cm
T2a - 7-10cm
T2b - >10cm

Question 13

How is a T3 renal tumour defined

Answer 13

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

Question 14

How is a T4 renal tumour defined

Answer 14

Spread beyond Gerota’s fascia, incl invasion into adrenal gland

Question 15

How is stage 3 renal cell carcinoma defined

Answer 15

T3 disease (invasion into perinephric fat or renal vein) or node positive

Question 16

How is stage 4 renal cell carcinoma defined

Answer 16

T4 disease (invasion into adrenal or beyond Gerota’s fascia) or distant mets

Question 17

How is a stage 1 (T1N0) renal carcinoma treated

Answer 17

Partial nephrectomy
Or RFA if not suitable for surgery

Question 18

How is a stage 2 (T2 N0) renal cell carcinoma treated

Answer 18

Lap radical nephrectomy

Question 19

How is a stage 3 (T1-3 N1, T3N0) treated

Answer 19

Open radical nephrectomy +/- lymphadenectomy

Radical nephrectomy - Remove kidney, adrenal, peri-renal fat, Gerota’s fascia +/- LN dissection.

Question 20

When is adjuvant treatment indicated for renal cell carcinoma

Answer 20

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

Question 21

What is a cytoreductive nephrectomy considered for the treatment for metastatic renal cell carcinoma

Answer 21

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

Question 22

When is a secondary cytoreductive radical nephrectomy indicated

Answer 22

Pts with local symptoms due to the primary tumour or near-complete responses to systemic therapy

Question 23

When is local metastasis-directed treatment indicated

Answer 23

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

Question 24

What scoring system quantifies prognosis of metastatic renal cell carcinoma

What is included

What are the categories

Answer 24

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

Question 25

How is the first line management of clear cell renal cell carcinoma determined
What are they

Answer 25

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

Question 26

What is the second line management of ccRCC

Answer 26

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

Question 27

What is the preferred first line treatment for non-clear cell RCC

Answer 27

Cabozantinib
Or axitinib or avelumab

Question 28

when can pazopanib and tivozanib be used in metastatic RCC

Answer 28

first line only

Question 29

what is the risk of renal cysts being malignant, and based on what score

Answer 29

Bosniak scoring system (based on Hounsfield units)
2F = minimally complex -> repeat imaging 6/12
3 = indeterminate -> partial nephrectomy
4 = 90% malignant -> partial/total nephrectomy