Renal pathology 1 Flashcards
List the common congenital abnormalities of the renal abnormalities?
Agenesis
Hypoplasia
Horseshoe kidney
Duplex (ureter, pelvis)
What is kidney agenesis?
Absent kidney (or both kidneys)
What is kidney hypoplasia?
Normal kidney develops but at a smaller size
What is horseshoe kidney?
Kidneys fuse at upper or lower pole (more commonly lower pole)
What is the prognosis of kidney agenesis of both kidneys?
Death
Kidney agenesis can result in a characteristic facial appearance. Why?
diminished urine production –>
diminished amniotic fluid –>
squished nose, recessed chin & low ears
What pathology can horseshoe kidney cause?
Obstruction (rarely)
How common are simple cysts?
Common
How do simple cysts affect the kidney?
They don’t usually have a functional effect
What size are simple cysts?
Can be large or small
When might simple cysts be secondary?
Long term kidney dialysis
Are simple cysts uni or bilobular?
Unilobular (but can have multiple cysts)
What are simple cysts filled with?
Fluid (NO blood)
How common is infantile type polycystic kidney disease?
Rare (there are varying subtypes but all are rare)
How does infantile type polycystic kidney disease cause?
Terminal renal failure (may die immediately or survive for several months)
Is infantile type polycystic kidney disease autosomal dominant or recessive?
Recessive (autosomal recessive polycystic kidney disease)
How are the kidneys affected in infantile polycystic kidney disease?
Bilateral enlargement. Maintenance of reniform shape. Dilation of medullary collecting ducts
What is infantile polycystic kidney disease always associated with?
Congenital hepatic fibrosis
How common is adult polycystic kidney disease?
Rare (BUT least rare of all the congenital cystic diseases)
Is adult polycystic kidney disease autosomal dominant or recessive?
Dominant
Where are the gene mutations of ADPCKD type 1 and type 2 respectively? Which is the most common?
Type 1 - chromosome 16 (most common)
Type 2 - chromosome 4
How does adult polycystic kidney disease present?
Middle aged
Abdominal mass
Haematuria
Chronic renal failure –> +/- hypertension
How are the kidneys affected in adult polycystic kidney disease?
Huge bilateral enlargement. Cysts of varying size cause distortion of the reniform shape.
Where are the cysts in ADPCKD found?
Nephron (any part)
What is ADPCKD associated with?
Berry aneurysms at the circle of willis
What two types of haemorrhage can be caused by ADPCKD?
Subarachnoid haemorrhage (circle of willis) Intracerebral haemorrhage (hypertension)
What other sites might cysts caused by ADPCKD arise? What problems do they cause?
Liver
Pancreas
Lung
No functional problems
List the benign renal tumours
Adenoma
Fibroma
Angiomyolipoma
Juxtaglomerular cell apparatus tumour (JGCT)
Describe the features of a renal adenoma
Describe the features of a renal fibroma
Common
Found in the medulla
Describe the features of a renal angiomyolipoma
Consists of vascular, muscle and fat cells
May be multiple/bilateral
What is renal angiomyolipoma associated with?
Tuberous sclerosis
What does JGCT cause?
Hypertension through increased renin secretion
List the malignant renal tumours
Nephroblastoma/Wilm’s tumour
Urothelial/transitional epithelial tumours
Renal cell carcinoma
Describe the features of nephroblastoma/wilm’s tumour
Arises from residual primitive renal tissue
What age group is affected by Wilm’s tumour? How common is it?
Children (1-10 y/o)
Commonest intra-abdominal tumour (in children)
How might nephroblastoma present?
Abdominal mass
Describe the features of renal cell carcinoma
Arises from renal tubular epithelium
What other names can renal cell carcinoma go by?
Clear cell carcinoma
Hypernephroma
Grawitz tumour
How common is renal cell carcinoma?
Commonest primary renal tumour in adults (overall rare)
Which age group is most commonly affected by renal cell carcinoma? Which sex?
55-60 y/o
Males
How might renal cell carcinoma present?
Abdominal mass Haematuria Flank pain Systemic symptoms Paraneoplastic syndrome (hypercalcaemia, polycythemia)
What is polycythemia? What is it often a feature off in relation to the renal system?
Increased red cells
Cancer (paraneoplastic)
Where does renal cell cancer commonly spread to?
Renal veins & IVC
How does renal cancer spread?
Haematogenously
Lymphatic spread late in disease
Which type of renal cell carcinoma is the most common? What is unique about these types of cells?
Clear cell
Increased lipids and glycogen
Which grading system is used in renal cell carcinoma?
Fuhrman
Where can transitional cell carcinomas be found?
Anywhere along the length of the urinary system from the renal pelvis to the urethra
Where do most transitional cell carcinomas arise?
Bladder
In which age group are transitional cell carcinomas found?
> 50 y/o
What are the risk factors for transitional cell carcinomas?
Dyes Rubber Analgesics (particularly renal pelvis) Schistosomiasis Smoking Cyclophosphamide
What is schistosomiasis?
Chronic parasitic kidney infection
How does transitional cell carcinoma commonly present?
Haematuria
Where in the bladder do most transitional cell carcinomas commonly arise? What is this a risk factor for?
Trigone Ureteric obstruction (+/- hydroureter & hydronephrosis)
Describe the morphology of transitional cell carcinomas
Solid vs papillary
Describe the grading system of transitional cell carcinomas
pTa - transitional cell cacner
pT1 - stromal invasion
pT2 - detrusor invasion
What is a risk factor for the areas surrounding transitional cell carcinomas?
Carcinoma in situ
How does transitional cell carcinoma spread?
Lymphatic (obturator, lung, liver)
How common is transitional cell carcinoma recurrence?
Common - tends to be higher grade each time it reoccurs
What is renal adenocarcinoma associated with?
Extrovesion (lower abdo wall absent and bladder open) Urachral remains (dome) Cystitis cystica (long standing)
What is squamous cell carcinoma associated with?
Schistomiasis
Calculi (–> metaplasia)
What is the commonest malignant bladder tumour in children?
Embryonal rhabdomyosarcoma
