RENAL - CANCERS

Question 1

How common is kidney cancer?

Answer 1

8th commonest cancer in men
9th in women

Question 2

Where do most renal cancers occur?

Answer 2

85% in renal parenchyma (renal cell carcinoma)

Question 3

What proportion of renal cancers occur outside the renal parenchyma? What are these?

Answer 3

6% occur in renal pelvis
6% occur in ureter
Both transitional cell carcinomas

Question 4

What are the risk factors for kidney cancer?

Answer 4

Increasing age
Men:female
Obesity
Smoking
Adult poly cystic kidney disease 3-4x increase
Renal dialysis - increased risk x7 after 10 years
Radiotherapy for previous testicular or gynaecological cancer
Hypertension
Poor intake of fruit and veg
FHx - e.g. Von-hippel lindau disease

Question 5

What is Von-Hippel lindau disease?

Answer 5

a hereditary condition associated with tumors arising in multiple organs because of a mutation in the VHL tumour suppressor protein. VHL-related tumors include hemangioblastomas, retinal angiomas, clear cell renal cell carcinoma, pancreatic neuroendocrine tumor. Tumors of the adrenal gland or pheochromocytoma can also develop.

Question 6

What are the 5 histological sub-groups of renal cell carcinoma?

Answer 6

Clear cell RCC - 80%
Papillary RCC 15%
Chromophobic RCC 5%
Collecting duct RCC 1%
Unclassified

Question 7

Outline the staging for renal cell carcinoma?

Answer 7

T1a - <4cm confined to kidney
T1 b - 4-7cm confined to kidney
T2 - >7cm and confined to kidney
T3 a - tumour into renal vein or perinephric fat
T3 b- tumour into IVC below diaphragm
T3 c - tumour into IVC above diaphragm
T4 - tumour through Gerotas fascia or adrenal gland

Question 8

How does kidney cancer present?

Answer 8

Usually asymptomatic

Can present with haematuria, palpable mass, weight loss, night sweats, anaemia, bone pain or fractures

Question 9

What paraneoplastic syndromes are associated with kidney cancer?

Answer 9

Polycythemia due to ectopic EPO secretion
Hypertension due to ectopic renin secretion
Cushing syndrome due to ectopic PTHrP which increases calcium and ACTH

Question 10

Why can renal call carcinomas cause varicoceles?

Answer 10

On the left side because it can impede venous drainage of the left testes (gonadal vein drains into left renal vein but on right it drains into the IVC directly)

Question 11

How do you investigate kidney cancer?

Answer 11

Ultrasound and CT for formal staging

Question 12

How do you treat small tumours in the elderly or those with significant medical co-morbidity?

Answer 12

Observation

Question 13

How is a localised kidney tumour treated?

Answer 13

Partial or total nephrectomy
Radio frequency ablation or cytotherapy

Question 14

How is metastatic kidney cancer treated?

Answer 14

Nephrectomy
Biological therapy with interfero gamma, tyrosine kinase inhibitors, temsirolimus

Question 15

Whats an example of a tyrosine kinase inhibitor used to treat renal cancer?

Answer 15

Sunitinib

Question 16

Where does kidney cancer mostly metastasise?

Answer 16

Lungs and bones

Question 17

Whats the prognosis of kidney cancer?

Answer 17

5 year survival overall is 50%
Average survival with metastatic disease is 18-24 months

Question 18

Outline the pathology of renal cell carcinoma?

Answer 18

Mutations on short arm of chromosome 3 cause epithelial cells in PCT to become cancerous

Question 19

Outline the main differences between sporadic kidney tumours and those caused by VHL disease?

Answer 19

Sporadic tumours mostly affect the upper pole of a singular kidney in old men who smoke
VHL disease typically involves both kidneys and affects younger men/women

Question 20

What is angiomyolipoma?

Answer 20

The most common benign tumour in the kidneys
Can also be found in the liver and, rarely, the reproductive system
It’s a tumour composed of blood vessels, smooth muscle and adipose tissue
A hamartoma

Question 21

What is a hamartoma?

Answer 21

mostly a benign mass of disorganized tissue native to a particular anatomical location

Question 22

Which kidney is more commonly affected in angiomyolipoma?

Answer 22

Right

Question 23

What can cause angiomyolipoma?

Answer 23

Mostly sporadic
Can be associated with tuberous sclerosis (caused by a mutation in a tuberous sclerosis gene TSC1 or TSC 2)

Question 24

What is tuberous sclerosis?

Answer 24

a rare genetic condition that causes mainly benign tumours to develop in different parts of the body.

Question 25

Whats the main complication of angiomyolipoma?

Answer 25

Retroperitonel haemorrhage - blood vessels are irregular, dilate causing aneurysm and can rupture

Question 26

How is angiomyolipoma treated?

Answer 26

Embolization

Question 27

What is Wilm’s tumour?

Answer 27

A kidney tumour composed of metanephric blastemal cells (cells involved in kidney development)
Also called a nephroblastoma
Most common kidney tumour in children and rarely seen in adults

Question 28

What chromosome is usually affected in Wilm’s tumour?

Answer 28

11

Question 29

What is WAGR syndrome?

Answer 29

a disorder that affects many body systems and is named for its main features: Wilms tumor, aniridia, genitourinary anomalies, and intellectual disability (formerly referred to as mental retardation).

Question 30

Why is WAGR known as a contiguous deletion syndrome?

Answer 30

Because there is a deletion or duplication that removes several genes lying in close proximity to one another on the chromosome.
In this case its the deletion of the p arm on chromosome 11

Question 31

What is Beckwith Wiedemann syndrome?

Answer 31

an overgrowth disorder (means that affected infants are larger than usual - macrosomia) characterized by an increased risk of childhood cancer.
Patients with Beckwith-Wiedemann often present with Wilms tumor, macroglossia, organomegaly, and hemihypertrophy.

Question 32

What is wilms’ nephroblastoma?

Answer 32

one of the most common childhood malignancies. It typically presents in children under 5 years of age, with a median age of 3 years old
It’s most commonly found as an abdominal mass, painless haematuria, flank pain