Renal Malignancy

Question 1

renal fibroma

Answer 1

• benign
• common
• present as white nodule in the medullary region
• no clinical signficance

Question 2

adenoma

Answer 2

• benign
• yellowish nodules in the cortical region
• <2cm

Question 3

Angiomyolipomas

Answer 3

• benign
• mixture of fat, muscle and blood vessels
• can be multiple and bilateral

Question 4

how are Angiomyolipomas diagnosed on CT

Answer 4

• demonstration of fat on CT is cornerstone of diagnosis
• the Hounsfield scale is used for describing radiodensity - fat has a value of less than ten (is dark)

Question 5

what can genetic disorder Angiomyolipomas be assoicated with

Answer 5

tuberous sclerosis

Question 6

tuberous sclerosis

Answer 6

• TSC1 (tuberin) or TSC2 (hamartin) gene
• Nails: Periungual fibromata and longitudinal ridging
• Cutaneous: ash-leaf macule (depigmented macule). These can be seen better under a Woods lamp.
• Facial angiofibromas
• Bone cyst
• Cortical tubers/calcification of falx cerebri – seizures, mental impairment
• Enamel pitting
• Shagreen patches

Question 7

how may Angiomyolipomas present

Answer 7

• may embolise
• suddenly as a haemorrhage - Wunderlich’s syndrome
  
  • this is a spontaenous, non traumatic haemorrhage that is confined to the subcapsular and peri renal space

Question 8

what are oncocytes

Answer 8

epithelical cells that have an excessive amount of mitochondria

Question 9

oncocytoma

Answer 9

• benign renal mass - 3-7% of all
• often asymptomatic

Question 10

describe the appearance of oncocytomas

Answer 10

large cells with eosinophilic granular pink cytoplasm

Question 11

are oncocytoma benign or malignant

Answer 11

benign, mayhave somemalignant potential

Question 12

diagnosis of oncocytoma

Answer 12

• there is often no definitive diagnosis except at nephrectomy
• often mistaken for RCC
• CT: sharp central stellate scar is characteristic

Question 13

JGCT

Answer 13

a rare tumour of the JG cells that produce renin. is a rare cause of 2y hypertension

Question 14

renal cysts

Answer 14

• comprise 70% o benign asympatomatic renal lesions
• can be single or multiple

Question 15

which type of CT is used for imaging

Answer 15

triple phase CT contrast enhanced

Question 16

Nephroblastoma (Wilm’s Tumour)

Answer 16

• embryonic malignant tumour
• of the primitive renal tubules and mesenchymal cells

Question 17

how does Nephroblastoma (Wilm’s Tumour) present

Answer 17

abodminal mass in children

Question 18

what is the most common abdominal malignancy in children

Answer 18

Nephroblastoma (Wilm’s Tumour)

Question 19

other names for RCC

Answer 19

Aka clear cell carcinoma, Grawtiz tumour, hypernephrom

Question 20

epidemiology of RCC

Answer 20

• males
• mean age 55
• 90% of renal cancer

Question 21

what percentage of dialysis patients develop carcinoma

Answer 21

15% - RCC

Question 22

classical triad of RCC presentation

Answer 22

• abdominal mass (may be lumpy), haematuria, flank pain

Question 23

presentation of RCC

Answer 23

• often asymptomatic
• classic triad of features
• hypertension
• systemic features eg anorexia, weight loss, malaise
• often an incidental finding on imaging

Question 24

how can RCC present in the testes

Answer 24

if invaded the left renal vein can compress the left testicular vein causing a varicocele

Question 25

name 3 paraneoplastic manifestations of RCC

Answer 25

* polcythaemia: increased production of erythropoietin from caricnoma cells - high haematocrit * anaemia: due to poor nutritional status and CKD causing a fall in erythropoietin (more common than the latter) * hypercalcaemia: due to release from bone metastases or increased calcium production

Question 26

how is polycythaemia treated

Answer 26

regular venesection

Question 27

describe the pathology of RCC

Answer 27

* large, well circumscribed mass centered on the cortex * yellow colour * solid cystic, necrotic and haemorrhagic areas

Question 28

what is the most common type of RCC

Answer 28

clear cell - tumour cells are rich in glycogen and lipid

Question 29

what genetic mutation occurs in clear cell RCC

Answer 29

3p deletion

Question 30

2nd most common type of RCC

Answer 30

papillary - elongated papilla with foamy cells

Question 31

chromophobe

Answer 31

* type of RCC * large cells with defined cell borders * atypical nuclei that resemble raisins - 'raisinoid'

Question 32

what is the most aggressive type of RCC

Answer 32

* collecting duct * high grade carcinoma of mixed cell type

Question 33

which genetic disorder often features multiple and bilateral RCC

Answer 33

von hippel-lindau

Question 34

VHL

Answer 34

* genetic AuD disorder characterised by cysts and benign tumours with potential for subsequent malignant transformation * caused by mutations in the VHL gene which is involved in hypoxia pathways

Question 35

which type of RCC is seen in VHL

Answer 35

clear cell

Question 36

diagnosis of RCC

Answer 36

* USS * CT * biopsy

Question 37

is biopsy good for RCC diagosis?

Answer 37

there is a high false negative rate

Question 38

spread of RCC

Answer 38

* direct renal vein extension, has a poor prognosis * can extend all the way from vena cava to RA * haematogenous - bone, liver, lung * lymphatic spread

Question 39

generally, how are RCC on presentation

Answer 39

often have metastases - they present late as they are deep seated in the retroperitoneum

Question 40

what is a classical sign in the lung of metastases from RCC

Answer 40

cannon ball metastases - caused by several types of tumour, but RCC is the most common

Question 41

what grading is used for RCC

Answer 41

Fuhrman nuclear grading, has less prognostic significance

Question 42

staging of RCC

Answer 42

Robson:

Question 43

surgery

Answer 43

* radical/partial nephrectomy * radiofrequency ablation, cryoablation

Question 44

radical nephrectomy

Answer 44

removes the whole kidney within the renal fascia

Question 45

partial nephrectomy

Answer 45

* nephron sparing - preserves renal function * good for low grade tumours

Question 46

what is an ECOG performance status

Answer 46

a scale used to assess the progression of disease and effect on patient's daily living activities

Question 47

when is an ECOG performance status used

Answer 47

metastatic kidney cancer will have one

Question 48

adjuvant therapy for RCC

Answer 48

* IL2 * Interferon alpha * Sunitinib * Inhibits VEGF/PDGF targeting angiogenesis and reducing neo-vascularization

Question 49

follow up of RCC

Answer 49

there is a risk of late recurrences - 10 year follow up