Congenital Abnormalities

Question 1

What is agenesis

Answer 1

Absence of one or both kidneys

Question 2

What is hypoplasia

Answer 2

Small kidneys but normal development

Question 3

What happens in horseshoe kidney

Answer 3

fusion at either pole - usually lower

Question 4

What are the consequences of cystic disease

Answer 4

Normally no functional disturbance

Question 5

What happens in infantile polycystic kidney disease

Answer 5

Terminal renal failure and less severe cases can survive for some months

Question 6

How is polycystic disease in passed on

Answer 6

Autosomal recessive inheritence (ARPKD)

Question 7

Describe the appearance of infantile polycystic kidney disease

Answer 7

Elongated cysts
Dilatation of medullary collecting ducts
Can survive for a few months but most patients die before this
Smooth

Question 8

What conditions is infantile polycystic disease associated with

Answer 8

Congenital Hepatic Fibrosis

Question 9

What is the least rare form of congenital cystic disease

Answer 9

Adult polycystic disease

Question 10

How is adult polycystic kidney disease inherited

Answer 10

Autosomal dominant inheritance

Question 11

What 2 chromosomes are involved in adult polycystic kidney disease

Answer 11

16 (90%) ADPKD1

and 4 APKD2

Question 12

When do patients with adult polycystic disease present

Answer 12

usually middle adult life

Question 13

What do patients with adult polycystic disease present with

Answer 13

abdominal mass
haematuria
hypertension
CRF

Question 14

Where else in the body are cysts seen in adult polycystic disease

Answer 14

Liver
pancreas
lung

Question 15

What happens to the size of the kidneys in adult polycystic disease

Answer 15

massive bilateral renal enlargement >1kg

Question 16

What is adult polycystic disease associated with

Answer 16

Berry Aneurysms in circle of Willis > subarachnoid haemorrhage

Question 17

Cystic lesions on the liver always alter function. True or false

Answer 17

False - usually don’t affect the function

Question 18

What is the commonest benign renal tumour

Answer 18

Fibroma

Question 19

Describe the appearance of a fibroma

Answer 19

Medullary origin
white nodules
few mm in size

Question 20

Describe the appearance of a benign adenoma

Answer 20

Yellowish nodules

Question 21

Describe the appearance of a benign Angiomyolipoma

Answer 21

Mixture of fat, smooth muscle and blood vessels

can be multople and bilateral

Question 22

What is a benign angiomyolopoma associated with

Answer 22

Tuberous sclerosis

Question 23

What does JGCT stand for

Answer 23

Juxtaglomelular apparatus tumour

Question 24

What do JGCT produce

Answer 24

renin > secondary hypertension

Question 25

What is the likelihood that an angiomyolipoma will become malignant

Answer 25

none - it has no malignant potential

Question 26

What is the commonest intra-abdominal tumour in children

Answer 26

Nephroblastoma (Wilm's Tumour)

Question 27

What does a nephroblastoma arise from

Answer 27

residual primitive renal tissue

Question 28

Where are urothelial carcinomas found specifically

Answer 28

renal pelvis and calyces

Question 29

Where do renal cell carcinomas arise form

Answer 29

renal tubular epithelium

Question 30

What are 3 types of renal cell carcinomas

Answer 30

Clear cell Ca hypernephroma Grawitz tumour

Question 31

What is the commonest primary renal tumour in adults

Answer 31

Renal cell carcinoma

Question 32

Who is most likely to develop a renal cell carcinoma

Answer 32

55-60 years old | M:F 2:1

Question 33

How do renal cell carcinomas present

Answer 33

Abdominal mass haematuria localised flank pain general features of malignant disease

Question 34

What can polycythaemia secrete

Answer 34

Erythropoeietic stimulating substance

Question 35

Describe the appearance of a renal cell carcinoma

Answer 35

Solid cystic necrotic haemorrhagic areas

Question 36

What complication is common in renal cell carcinoma

Answer 36

renal vein extension- some may have stenosis in the vena cava and up to the right atrium

Question 37

How do renal cell carcinomas metastasise

Answer 37

Blood-borne metastatic spread to lung, bone | Lymphatic spread later

Question 38

What is the grading system used for renal cell carcinomas

Answer 38

Fuhrman

Question 39

Where do transition cell carcinomas arise

Answer 39

transitional epithelium - from pelvicalyceal system to urethra

Question 40

What are some risk factors for developing a transitional cell carcinoma

Answer 40

Aniline dyes rubber industry hydrocarbons smoking

Question 41

What is the commonest presenting symptom of a transitional cell carcinoma

Answer 41

Haematuria

Question 42

Where do the majority of transitional cell carcinomas arise

Answer 42

in the region of trigone - ureteric obstruction

Question 43

What is meant by pTa, pT1

Answer 43

stromal invasion of a transitional cell carcinoma

Question 44

What is meant by a pT2

Answer 44

Muscle invasion of a transitional cell carcinoma

Question 45

where do transitional cell carcinomas spread to

Answer 45

local lymph nodes (obturator nodes in the pelvis), lung, liver

Question 46

How likely is it that a transitional cell carcinoma would recur

Answer 46

Very - tumours often progress to a higher grade / stage as well

Question 47

What is the commonest malignant bladder tumour in children

Answer 47

Embryonal Rhabdomyosarcoma

Question 48

Where are squamous carcinomas found in the kidneys

Answer 48

Calculi (squamous metaplasia)

Question 49

Where are adenocarcinomas found in the kidneys

Answer 49

extroversion (glandular metaplasia)

Question 50

What do squamous carcinomas often produce

Answer 50

keratin