Pathology Flashcards
Explain each of the following terms:
Agenesis
Hypoplasia
Horseshoe Kidney
Agenesis - absence of one or both kidneys
Hypoplasia - small kidneys, normal development
Horseshoe kidney - fusion at either (usually inferior) pole.
What is the appearance of a child who has bilateral renal agenesis and why?
Looks squashed - most of the amniotic fluid is formed from foetal urine.
Agenesis of one kidney can live a normal life - true/false
True - kidneys have a massive reserve so one kidney is sufficient.
In hypoplasia there may be normal or diminished function - true/false
True
What clinical consequence(s) may occur from a horseshoe kidney?
Very few
May cause obstruction of urinary tract if very large bridge - usually an incidental finding.
What is the most common cystic kidney disease?
Simple cyst
What cell type is a simple kidney cyst lined by?
Squamous epithelium
Simple cysts rarely compromise renal function - true/false
True
Simple cysts are usually incidental/symptomatic findings and can be a consequence of ______
Incidental finding
Consequence of long term haemodialysis.
There are two types of polycystic disease - what are they?
Infantile type PKD & Adult type PKD.
Infantile type PKD is fairly common - true/false
Fairly rare
In the perinatal group, polycystic kidney disease is terminal - true/false. if true, why? if false, why not?
True
Neonates are not suitable for transplanting and not suitable for long term haemodialysis.
Infantile PKD follows which inheritance pattern?
Autosomal recessive
What is seen in infantile type PKD?
Uniform, bilateral renal enlargement
Elongated cysts
Dilation of the medullary collecting ducts
What other disease is infantile type PKD associated with?
Congenital hepatic fibrosis.
Adult PKD is the more common that infantile type PKD - true/false
False - this is the least common form of congenital cystic disease
what chromosomes/genes are affected in adult type PKD?
Chromosome 16 - ADPKD1 (90% of cases)
Chromosome 4 - ADPKD2 (10%)
Why is genetic counselling important?
it presents later so it is important if they wish to have a family.
How does adult PKD usually present?
mid-life
Abdominal mass, haematuria, hypertension and CRF
What percentage of patients on haemodialysis and on the transplant registries have adult PKD?
8%
On examination what will you find in adult PKD?
Grossly enlarged kidney
Multiple cysts, vary in size
distortion of renal shape.
What weight is a normal kidney? How does this compare to PKD?
100g normal
Can be up to and above 1 kg in PKD.
Cysts may spread to _____ & ______ in PKD
Liver and lungs
In PKD, liver and lung cysts have no functional effect; true/false
True
What percentage of patients with PKD will have lung and liver cysts?
33% (1/3)
Subarachnoid haemorrhage is associated with PKD - explain.
there is an increased incidence of berry aneurysm in the circle of willis in PKD patients. this predisposes to a subarachnoid haemorrhage.
Give the 4 types of benign tumour in the kidneys.
Fibroma
Adenoma
Angiomyolipoma
IGCT
Describe a renal fibroma
Common, medullary in origin, white nodules
Describe a renal adenoma
Yellowish nodules, less than 2cm and cortical tumour
Describe a renal angiomyolipoma
Mixture of fat, muscle and blood vessels. Can be multiple, bilateral and associated with tuberous sclerosis.
What percentage of tuberous sclerosis patients will develop renal angiomyolipoma?
70%
Describe a renal IGCT
Benign tumour of the juxtaglomerular apparatus and so secretes renin. A cause of secondary hypertension.
What is a nephroblastoma?
Malignant intra-abdominal mass, occurs between 1 and 10 years and is most common tumour in children.
Nephroblastoma occurs from which tissue(s)?
Residual primitive renal tissue
What is the most common tumour of the kidney?
Renal cell carcinoma
Renal cell carcinoma arises from the renal tubular epithelium - true/false
true
Renal cell carcinoma accounts for __% of all adult visceral tumours and is peak age between ____ & _____. it is ___x more/less common in females than males
3% of all adult visceral tumours
55-60years old
2x more common in females
How will renal cell carcinoma usually present?
Palpable abdominal mass
Haematuria
Flank pain
General symptoms of malignancy
list and explain some of the paraneoplastic manifestations of renal cell carcinoma?
Polycythaemia - increased erythropoietin secretion
Hypercalcaemia - secretes PTH to causes bone resorption.
Renal cell carcinoma on examination will be ___
Well defined Large centred on cortex yellow solid/necrotic/cystic/haemorrhagic areas.
renal vein extension is uncommon in a renal cell carcinoma - true/false
false - it is common and may even extend into the IVC and right atrium - associated with poor prognosis.
majority of spread of a renal cell carcinoma is via _____
Haematogenous spread first lymphatic spread later.
Where are the common metastatic sites for renal cell carcinomas?
Lungs
Bones
Transitional cell carcinoma can occur anywhere from _____ to _____
Pelvicocalyceal system to the urethra
Transitional cell carcinoma accounts for ___% of bladder tumours
90%
Transitional cell carcinoma is most common is which age group?
over 50s
What other hazards are there for transitional cell carcinoma except for age?
aniline dyes Rubber industry work Benzidine Cyclophosphamide analgesics schistosomiasis smoking
What is the commonest symptom of transitional cell carcinoma?
haematuria
If you find blood in urine, you should ____
Escalate it asap
85% of transitional cell carcinoma occur in the trigonal area - true/false
false - 75%
Trigonal transitional cell carcinoma can lead to ____
ureteric obstruction (uni/bilateral) and/or urethral obstruction
Carcinoma in situ can occur in the ____ transitional epithelium
flat transitional epithelium
Carcinoma in situ will spread to which local lymph nodes? Which organs may it spread to?
Obturator
Liver and lungs
Recurrence of transitional cell carcinoma is vanishingly rare - true/false
False - it is very common
Tumours of the penis are common - true/false
False
Tumours of the penis are normally what histological type?
Squamous cell carcinoma arising from the epithelium of the skin.
Like most squamous cell carcinoma, tumours of the penis often have an in-situ component; where are these normal located?
Skin
Glans penis
Give the two clinical appearances of a penile tumour
Bowen’s disease - dried, crusty appearance of the skin. Can get it anywhere that there is skin.
Erythroplasia of Queyrat - pre malignancy where the glans develops a raised, red velvety area
What is seen in either Bowen’s disease or erythroplasia of Queyrat on histological examination?
Full thickness dysplasia of the epidermis in carcinoma in situ.
What percentage of carcinoma in situ will under go malignant change?
5%
Squamous cell carcinoma of the penis is uncommon in the Uk - true/false
True
The incidence of a squamous cell carcinoma is higher in some parts of the world; where?
Latin America, Africa, far East.
Squamous cell Carcinoma of the penis are almost exclusively contained to men who ____
Have not been circumcised.
Circumcision in adulthood gives the same protection from squamous cell carcinoma of the penis as in childhood - true/false
False - childhood circumcision gives much more protection than adult circumcision.
What is the aetiology of squamous cell carcinoma?
poor hygiene allows carcinogen build up.
HPV