Renal and Urinary Tract Flashcards
A patient has a history of adenocarcinoma of the bladder. Which of the following is most correct?
- This is the most common form of bladder tumor
- While rare in adults it is the most common pelvic paediatric tumor
- Approximately 20 – 40% are associated with a urachal remnant.
- They suggest with a urachal remnant or chronic irritation (e.g. bladder calculus or chronic infection including schistosomiasis)
- It is most commonly due to “drop” metastasis from the upper urinary tract
- *Approximately 20 – 40% are associated with a urachal remnant.
You notice a thick walled urinary bladder which may reflect cystitis. Which of the following is LEAST accepted to be a cause of cystitis?
- Leishmaniasis
- Radiation therapy for cervical carcinoma
- Methotrexate chemotherapy
- Schistosoma haematobium
- Candida
- *Leishmaniasis
Which of the following statements concerning TCC of the bladder is LEAST correct?
- Morphologically TCC may appear flat
- The majority of tumors are low grade, with less than 10% of these showing deep invasion
- Due to the internal sphincter/ capsule, prostatic invasion is rare (<1% of invasive malignancies)
- About 40% of deeply invasive tumors metastasise to pelvic nodes
- Haematogenous spread is principally to lung and bone marrow
- *Due to the internal sphincter/ capsule, prostatic invasion is rare (<1% of invasive malignancies)
A patient on regular TCC follow up has a bladder biopsy which the pathologist says look like a “small cell carcinoma of the lung”. Which of the following is most correct?
- The setting and description are consistent with a small cell carcinoma of the bladder.
- The description strongly suggests metastatic disease
- The pattern is recognised post diathermy of the bladder
- The pattern suggests bladder pheochromocytoma
- The appearances can be seen with de-differentiated TCC.
- *The setting and description are consistent with a small cell carcinoma of the bladder.
What does analgesic nephropathy cause? (August 2014)
a. Renal papillary necrosis
Analgesic nephropathy is usually caused by excessive intake of a mixture containing phenacetin
o Interstitial nephritis begins two weeks post ingestion (Immunizing haptens)
o Drugs bind to the tubular cellular or matrix components and incite an IgE antibody and T-cell mediated response
- Tubular necrosis, inflammation and oedema
Clinical:
- Renal papillary necrosis secondary to ischaemia: compression of blood vessels by markedly oedematous tissues (Haematuria and/or ureteric obstruction)
- Fever, eosinophilia, rash, sterile pyuria, azotaemia, acute renal failure
ANSWER: Analgesic nephropathy causes renal papillary necrosis
Which causes papillary necrosis? (March 2017)
a. NSAIDs
b. Steroids
c. Warfarin
d. Streptokinase
Causes of renal papillary necrosis (POST CARDS): o Pyelonephritis o Obstruction o Sickle cell disease o Tuberculosis o Cirrhosis o Analgesic abuse (NSAIDs) o Renal vein thrombosis o Diabetes mellitus o Systemic vasculitis
ANSWER: NSAIDs are a recognized cause of papillary necrosis
Which condition does sarcoid not cause? (March 2016)
a. Chronic glomerulonephritis
b. Choroid retinitis
c. Splenomegaly
d. Non-caseating granulomas in the lungs
Sarcoid renal disease includes: o Abnormal calcium metabolism o Nephrolithiasis o Nephrocalcinosis o Acute interstitial nephritis (with or without granuloma formation)
ANSWER: Sarcoidosis does not cause chronic glomerulonephritis, it causes acute interstitial nephritis. Also does not cause choroid retinitis - causes anterior uveitis.
Which is false of polycystic kidney disease? (March 2015)
a. Fibrosis of the liver
b. Liver cysts
c. Caroli disease
d. Alagille syndrome
Autosomal dominant PCKD associated findings:
o Cerebral berry aneurysms
o Intracranial dolichoectasia: elongated and ectatic arteries
o Hypertension (80%)
o Colonic diverticulosis
o Bicuspid aortic valve
o Mitral valve prolapse
o Aortic dissection
o Multiple biliary hamartomas (von Meyerberg complexes)
o Cysts in other organs:
- Liver (75%), Ovaries, Spleen, Seminal vesicles (60%), Prostate, Pancreas (pancreatic cysts are more common in VHL)
Alagille syndrome:
o Most common cause of hereditary cholestasis
o Renal assoc most commonly is renal dysplasia (with or w/o cysts). Can be associated with cystic kidney disease, small echogenic kidneys and nephrocalcinosis
Caroli disease: o Associations: - Congenital hepatic fibrosis - Medullary sponge kidney - Autosomal dominant PCKD - Autosomal recessive PCKD
ANSWER: Alagille Sydrome
Which is true? (March 2015)
a. Proteus is associated with the formation of struvite stones
b. Multiple myeloma is associated with recurrent pyelonephritis
Renal stones: o Calcium oxalate and phosphate (70%) - Hypercalciuria – idiopathic (50%) - Hypercalcaemia w hypercalciuria (10%) - Formed by nucleation of calcium oxalate crystals by uric acid in the collecting tubules
o Struvite (5-10%)
- Formed after infection w urea splitting bacteria e.g. proteus, some staphylococcus
- Convert urea to ammonia which causes precipitation of magnesium ammonium phosphate salts
- Can form very large (staghorn) calculi
o Uric acid (5-10%)
- Patients with hyperuricaemia - Gout or high cell turnover (leukaemia)
- Half do not have an underlying hyperuricaemia
o Cysteine (1-2%)
- Genetic defects in the renal absorption of amino acids
- Stones form at low urinary pH
o Others/unknown (5%)
Multiple myeloma can be complicated by infections
o Pneumonia & pyelonephritis most common, especially in the months after commencing chemotherapy
o Pneumonia: pneumococcus, staph aureus and klebsiella
o Pyelonephritis: E. coli and other gram negatives
ANSWER: Both are true
Which is the most likely composition of renal stones in a patient with leukaemia? (March 2017)
a. Uric acid
ANSWER: Uric acid stones are most likely in a patient with leukaemia due to high cell turnover (increased purine metabolism)
Which is the most common presentation of angiomyolipoma? (March 2016)
a. Haematuria
b. Retroperitoneal haemorrhage
c. Obstruction
Presentation of angiomyolipomas: o Incidental findings when imaging for other reasons (most common) o Retroperitoneal haematoma (most common symptomatic) o Other: - Palpable mass - Haematuria - UTI - HTN - Renal failure
Epidemiology:
o 80% sporadic - Adults, more common in females (4:1)
o 20% associated w phakomatoses
- Tuberous sclerosis accounts for the majority
- Rarely seen w VHL & NF1
ANSWER: Most common presentation of AML is an incidental finding. The second most common presentation is w retroperitoneal haemorrhage, particularly in large lesions
Regarding the affects of RCC (March 2015)
a. Hypertension
b. Feminisation
c. Limbic encephalitis
Epidemiology:
- Most common primary malignant renal tumour (80-90%)
- 50-70 years, more common in men (2:1)
Clinical presentation: - Macroscopic haematuria (60%) - Flank pain (40%) - Palpable flank mass (30-40%) - Triad of these findings in 10-15% • More commonly RCC is an incidental finding
Paraneoplastic syndromes (25%):
- Hypercalcaemia (20%) - HTN (20%) - Polycythaemia - Stauffer syndrome – hepatic dysfunction not related to metastases - Feminisation - Limbic encephalitis
ANSWER: All three options may be part of a paraneoplastic syndrome secondary to RCC
Which is associated with the highest risk for RCC? (March 2016)
a. Nephronopthisis
b. Medullary cystic disease
c. Acquired renal cystic disease
d. ARPKD
e. ADPKD
Risk factors for RCC: o Smoking o Dialysis related cystic disease o Obesity o Treatment w cyclophosphamide (chemotherapy)
ANSWER: Acquired renal cystic disease
Which is not associated? (April 2013)
a. Pyelocalyceal obstruction and xanthogranulomatous pyelonephritis
b. Horseshoe kidney and renal calculi
c. ARPKD with congenital hepatic fibrosis
d. Renal cell carcinoma and analgesic abuse
e. Schistosomiasis and bladder wall calcification
ANSWER: RCC is not associated w analgesic abuse
Regarding Wilms tumour, which is true? (September 2013)
a. Usually diagnosed before age 2
b. WAGR – the ‘a’ represents adrenal tumours
c. Carries a poor prognosis
d. If bilateral, is associated w nephrogenic rests -> both unilateral and bilateral are associated with rests, 40% in unilateral and 100% in bilateral
e. Is associated with a syndrome of deletion of the 11p chromosome
Wilms tumour
o Epidemiology:
- Most common paediatric renal mass (85%) of cases
- 6% of childhood cancers
- Early childhood (age range 1-11), peak age 3-4 years
• 80% before age five
• Occur earlier (2-24months) if associated with a syndrome
- No gender predilection, presentation may be slightly later in females
o Usually unilateral - 5% bilateral
- Bilateral lesions are assoc w nephrogenic rests (nephroblastomatosis) in 99% - Assoc of nephroblastomatosis: Beckwith-Wiedemann syndrome, hemihypertrophy, WAGR - Types of rests: perilobar (90%) & intralobar (10%) - Intralobar associated with Wilms tumour
o Clinical:
- Haematuria (20%) - HTN due to increased renin production in 25% - Acquired von Willebrand disease in 8%
o Associations (although >95% are sporadic):
- Overgrowth syndromes (WT2 gene, Chr 15p15):
• Beckwith-Wiedemann
• Perlman syndrome
• Simpson-Golabi-Behmel syndrome
• Sotos syndrome
- Non-overgrowth syndromes (WT1 gene, Ch 11p13):
• WAGR syndrome (Wilms, Aniridia, Genitourinary abnormalities, Retardation)
• Denys-Drash syndrome
- Isolated abnormalities:
• Cryptorchidism
• Hemihypertrophy
• Hypospadias
• Sporadic aniridia
• Renal fusion
o Pathology:
- Arise from mesodermal precursor cells of the renal parenchyma (metanephros) - Well circumscribed macrolobulated cancers - Haemorrhage & central necrosis common
o Imaging:
- Large lesions - Usually solid but can be predominantly cystic
o Metastases:
- Lung – 85% - Liver & local LNs - May form tumour thrombus (renal vein, IVC, right atrium)
o Prognosis:
- Treatment: combination of chemoTx & nephrectomy
- Treatment is curative in 90%
- Local recurrence can occur in the tumour bed or within the liver or lungs
Hemihypertrophy:
- Asymmetry in size bw right & left side of the body, more than can be attributed by normal variation
- Cells are hyperplastic rather than hypertrophied
Aetiology: Sporadic or as part of a syndrome: o Beckwith-Wiedemann syndrome o Proteus Syndrome o Klippel-Trenaunay syndrome o NF1 o Hemihyperplasia-multiple lipomatosis o McCune-Albright syndrome o Langer Giedeon - 5% increased risk of malignancy, particularly Wilms tumour
ANSWER: Wilms tumour is associated w a deletion of 11p chromosome (WT1 gene – 11p13 deletion)
