bladder and kidneys - tumours

Question 1

who gets clear cell renal cell carcinoma

Answer 1

• more common in men
• ages 50-70
  risk factors
• smoking
• hypertension
• obesity
• occupational exposure to toxins (cadmium, asbestos, petroleum by-products)
• genetic factors

Question 2

RCC occurs in (body part)

Answer 2

arise from the epithelial cells of the proximal convoluted tubule in the renal cortex
usually solid but can be cystic

Question 3

RCC histology

Answer 3

tumour composed of clear cells

cytoplasm is filled with carbohydrates and lili, so look yellow macroscopically

Question 4

von hippel-lindau disease

Answer 4

• rare genetic disorder
• develop visceral cysts and tumours
• angiomas, haemangioblastomas, phaeochromocytomas, renal cell carcinoma, pancreatic cysts
• caused by mutation in VHL tumour suppressor gene on chromosome

Question 5

sporadic clear cell RCC

Answer 5

loss of the short arm of chromosome 3, either by deletion or unbalanced translocation
loss of one VHL gene
the other VHL gene undergoes mutation or hypermethylation

Question 6

once VHL genes are lost

Answer 6

VHL is aa tumour supressor gene
when inactivated, IGF-1 is increased (growth factor)
dysregulated cell growth
IGF-1 unregulated hypoxia inducible factors, which unregulated vascular endothelial growth factor and receptors
angiogenesis occurs
tumour formation

Question 7

clinical presentation of RCC

Answer 7

silent cancer
patients often asymptomatic and don’t present until there is advanced disease
modern imagine means that many are now picked up incidentaly on scans done for other indications

Question 8

classic symptoms of RCC

Answer 8

haematuria
abdominal mass
flank pain
if tumour is large/already metastasised

also - weight less, fever etc.

Question 9

haematuria in RCC

Answer 9

when renal mass invades into the collecting system and renal pelvis
can cause clots that get stuck in the ureter - colicky pain
advanced sign

Question 10

abdominal mass/pain in RCC

Answer 10

can be palpated in skinny adult
feels firm, non-tender, moves with respiration
pain is in the costovertabral angle

Question 11

scrotal varices in RCC

Answer 11

when the renal cell occurs on the left side, it can spread into the IVC and obstruct the left gonadal vein

Question 12

paraneoplastic syndromes

Answer 12

hypercalcaemia - from overproduction of parathyroid hormone-related protein, can also be from metastasis to the bone
erythrocytosis - from overproduction od arythropoeitin
hypertension - overproduction of renin
Cushing syndrome - over production of ACTH
other cytokines - fever, weight loss

Question 13

RCC in veins

Answer 13

• as the carcinoma grows, first it gains access to the veins in the renal sinus
• then the renal vein, then the IVC, then metastasises to the lung, bond brain

Question 14

when RCC is suspected

Answer 14

abdominal CT
chest CT
tissue biopsy
bone scan if patient has bone pain

Question 15

treatment for RCC

Answer 15

• mainstay of treatment is surgery as RCC is relatively resistant to traditional chemotherapy and radiotherapy
  immunomodulatory therapies
  targeted therapies - block VEGF pathways

Question 16

urothelial carcinoma

Answer 16

previously known as transitional cell carcinoma
anywhere in the urinary tracy, most common in the bladder
clinically divided into superficial (non-muscle invasive), muscle-invasive and metastatic urothelial carcinoma

Question 17

who gets bladder carcinoma

Answer 17

older people 
men > women 
risk factors 
- smoking 
- occupational carcinogen exposure, painters, rubber industry, textile industry, metal workers

Question 18

urothelium

Answer 18

3-7 cell layers thick

umbrella cells

Question 19

2 pathways to getting bladder cancer

Answer 19

flat pathway - flat surface and invade quickly into the deeper layers of the bladder
papillary pathway - grows into the lumen of the bladder

Question 20

papillary pathway

Answer 20

p53 independant

• low grade papillary tumours
• less aggressive
• FGFR3 and RAS mutations
• chromosome 9 deletions
• may the lose TP53 functions and/or RB function, then invade

Question 21

flat pathway

Answer 21

p53 dependant
more aggressive
tend to be flat tumours
invade early

Question 22

theories for urothelial carcinoma

Answer 22

• field effect - cancers are as a result of carcinogens
• implantation theory - tumours implant at different parts of the urinary tact

to explain why this tumour in multifocal and recurrent

Question 23

symptoms of bladder cancer

Answer 23

• haematuria, painless
• frequency
• anaemia
• pain - when very late stage
  no physical signs

Question 24

patient work up when bladder tumour is suspected

Answer 24

• urine cytology
• cystoscopy
• biopsy
• resection
• imagine of the upper urinary tract (as urothelial cacrinoma can be multifocal)
• if metastasis is suspected, chest CT, bone scan

Question 25

staging of bladder cancer

Answer 25

t1 - into lamina propriety
t2 - into muscularis propria
t3 - perivesical fat
t4 - adjacent organs

Question 26

treatment of bladder cancer

Answer 26

In non-muscle invasive urothelial carcinoma 
- resection 
- +/- local chemotherapy 
in muscle-invasive urothelial carcinoma 
- cystectomy/cystoprostatectomy 
- radiation 
- chemotherapy

Question 27

squamous cell carcinoma

Answer 27

uncommon in western countries

in areas with schistosomiasis, very common form of bladder cancer

Question 28

summary of bladder tumours

Answer 28

• usually urothelial - rarely squamous cell carcinoma in western places
• more common In males
• smoking is a risk
• painless haematuria
• flat carcinoma in situ and papillary urothelial carcinoma are precursor lesions
  TP53 and RB are central to the development of muscle invasive urothelial carcinoma
  schistosomiasis infection is a rask factor for developing SCC