GENITOURINARY PATH Flashcards
What are the Medullary cystic disease complex subtypes
- Medullary sponge kidney
- Nephronophthisis spectrum - Juvenile familial subtype (most common)
- Adult onset medullary cystic disease
Medullary spong kidney disease definition, clinical presentation, renal function, end result ?
- Multiple cystic dilatation of the collecting ducts in the medulla of unknown cause
- Occurs in adults as an incidental finding
- Normal renal function
- Most cases develop medullary nephrocalcinosis
Nephronophthisis Spectrum
- Definition
- Most common type
- Associations?
Definition: Cysts at the medulla and corticomedullary junction
Subtype:
- Juvenile familial subtype (most common type) - AR
Associations
- retinal malformation
- Joubert syndrome
- Meckel Gruber syndrome
Adult onset medullary cystic disease
- Inheritance
- AD
- Distinct from the nephronophthisis spectrum as it has separate genetics BUT similar morphology and progression to end stage renal disease in adult life.
Multicystic dysplastic kidney
- Inheritance
- Develops when ?
- Clinical presentation ?
Inheritance
- Sporadic
Develops
- in-utero
Clinical
- common cause of renal agenesis, following complete involution during childhood
- The affected kidney becomes nonfunctional. If bilateral renal failure results.
Multicystic dysplastic kidney associations ?
vesicoureteric reflux: most common and seen in up to 20%
pelviureteric junction obstruction
Acquired renal cystic disease
- epidemiology
- association
Epi
- Dialysis pt
Association
- RCC (12-18x increase)
- 7% dialysis will develop RCC over 10 years
Renal TB
- Definition
- Epidemiology
- Clinical
- RF
Def
- TB may involve the genitourinary tract with OR without lung involvement
- 2nd most common site after lungs
Epi
- Common globally, uncommon in australia
Clinical
- Asymptomatic
- May have cystitis or pyelonephritis
- May have frequency, nocturia, hematuria and pyruia
RF
- Low SES
- Smoking
- Low BMI
Renal TB
- Aetiology
- Pathophysio
Aetiology
- Usually Hematogeneously from pulmonary TB (but doesnt have to be)
Patho
- Colonizes the medulla*
- Infection contained by formation of granulomas with caseous necrosis
- In immune competent pts these remain stable or heal*
- however reactivation may occur if become immunocompromised (decades of latency)
- Caseous necrosis => Papillary Necrosis => SLough into calyces infecting and obstructing collecting system
- Ulcero-Cavernous lesions: when papillary lesions erode into the pelvicalyceal system => antegrade infection of lower tract
Morphology of Renal TB stages
Acute
- multiple cavities with yellow friable necrotic material involving the medulla
- Papillary necrosis
Late stage
- Cement of putty kidney with small contracted kidney
End stage
- Autonephrectomy with small calcified kidney
- Ureteral stricture common
- Bladder granuloma common - small bladder that can’t contract
Microscopic appearance of Renal TB
Chronic tubulointerstitial nephritis with caseating granulomas
Renal TB association
Amyloidosis
TCC
- definition
- epi
Definition
- Malignant tumour arising from the epithelium of the renal tract (urothelium/transitional epithelium).
In descending frequency occurs most commonly in bladder, pelvis, ureter and urethra.
Epidemiology
- Age range is 50-80.
- More common in males by 3:1
Classification (of epithelial bladder tumours - same as lung):
TCC Transitional cell (urothelial) carcinoma
- papillary
- carcinoma in situ / flat non-invasive
SCC
- Schistosomiasis
- due to chronic bladder irritation or infection from calculi
- often found later so higher mortality
Adenocarcinoma
- Rare, similar to GIT adenocarcinomas
- arise from urachal remnant
- Mortality same as SCC
SCC
- indistinguishable from SCC of the lung histologically - rare
Others
- Leiomyoma, embryonal sarcoma, lymphoma
TCC
- Clinical presentation ?
- RF ?
Clinical presentation
- Painless hematuria
- Hydronephrosis with flank pain if ureter obstructed
- Urinary retention if bladder outlet obsturcted
RF
- Smoking (highest risk)
- Aniline dyes, rubber and textiles
- Cancer treatment: Cyclophosphamide
- Radiation
- Chronic infection: Schistosomiasis
What are the 2 precursor lesions of TCC ?
-
Non-invasive papillary lesions
- Common with a range of atypia -
Carcinoma in situ (flat non-invasive)
- Cytologically malignant cells present within a flat epithelium – the cells tend to be dyscohesive so shed easily in the urine
Morphology of TCC
- usually T2 at diagnosis
- Papillary, nodular, or flat infiltrating
- Papillary low grade, can still invade but rarely fatal
- CIS is commonly multifocal*
Treatment for T1 and T2 cancer of TCC
T1: into lamina propria
- local endoscopic resection and intravesicle BCG
T2: into musuclaris propria (detrusor)
- Cystectomy and radiotherapy required
Complications and prognosis of TCC
- Recurrences common and often high grade
- 98% 10 year survival in low grade even with recurrence
- Late stage survival is poor
Schistosomiasis pathophysio
- Parasitic disease caused by immunologic reaction to schistosoma eggs trapped in tissue
- Infected with the larval penetrating the skin, swimming in infected water
- Chronic inflammatory response -> probably cycles of cell damage and repair
- Initial bladder wall thickening. Chronic phase -> contracted calcified thick walled bladder
Types of renal stones
Calcium
- Supersaturation of stone constituents exceed solubility of urine
- Hypercalciuria (most common), dehydration, hypercalcemia, sarcoidosis, hyperparathyroidism
Struvite (mag, ammonia, phosphate)
- Proteus or staph infection
- alkaline urine
Urate
- Hyperurecemia patients
- Gout, hematological malignancy, acidic urine
- Radiolucent
- Acidic urine
Cystine
- Genetic defects in reasorption of amino acids including cystine
Adrenal adenoma Microscopy
- Misoses absent or rare
- Aldosterone Adrenal adenoma: Spironolactone bodies
- <5cm
- Solitary, Encapsulated Golden yellow (from lipids*)
Adrenal carcinoma vs adenoma
Adrenal carcinoma
- More functional: usually virilising when functional (c.f. adenoma non-functional, asymptomatic 95%)
- Bi-modal and more common in kids than Adenoma
- Often large: 20cm (c.f. Adenoma <5cm)
- Smaller lesions may look like adenoma**
Adrenal carcinoma RF
- if seen in child must exclude Li-Fraumeni (TP53)
- also associated with Beckwith-Wiedemann
Prostate cancer generally what type ?
Acinar subtype*
Loss of the basal cell layer**
Prostate cancer clinical presentation ?
- Asymptomatic, usually detected by PSA / rectal exam
Prostate cancer
- Aetiology
- Androgen has a role (target of hormonal therapy)
- Family history
- High fat diet
- BRCA2 mutation (20x)
What causes BPH ?
- Dihydrotesterone (DHT), an androgen derived from testosterone via 5-alpha-reductase is the major hormonal stimulus for hyperplasia
- Located in stromal cells* -> spreads to epithelial cells later
Tx
- alpha-blockers
- 5-alpha-reductase inhibitors
Wilms tumor clinical presentation
- PAINLESS upper quadrant abdominal mass
- Haematuria is seen in ~20% of cases
- hypertension
- von Willebrand disease is seen in
HPV related SCC prognosis vs non-HPV related SCC
- HPV related SCC has better prognosis
How many % of nephroblastomatosis will turn into Wilms ?
30-40%
Crossed fused renal ectopia which side more common ? Complications ?
Location
- Left-to-right (most common)
Complications
- Nephrolithiasis, infection, and hydronephrosis approaches ~50%
Associations
- ureteropelvic junction obstruction, vesicoureteral reflux, and renal multicystic dysplasia.
- Müllerian agenesis
HIV nephropathy imaging ?
- Rapidly enlarging bilateral echogenic kidneys
- No hydronephrosis
- Rapid renal failure
- Striated kidneys on nephrogram
Ureteritis cystica aetiology ?
Results from chronic urinary tract irritation due to stones and/or infection.
Organisms frequently involved include:
- Escherichia coli (most common)
Seen in diabetics with recurrent urinary tract infections.
causes of Papillary necrosis ?
NSAIDS
Sickle cell disease
Acetominophen
Infection (TB)
Diabetes*
Imaging of Papillary necrosis
- Sloughed papillae appear as filling defects in the collecting system and ureters and may obstruct them and cause renal colic
- “club-shaped” calices
Causes of Medullary nephrocalcinosis
- hyperparathyroidism
- medullary sponge kidney
- renal tubular acidosis (type 1)
- hypervitaminosis D
- milk-alkali syndrome
- sarcoidosis
Causes of Cortical nephrocalcinosis
- Renal cortical necrosis (sepsis, ischemia)
- Chronic glomerulonephritis
- Chronic pyelonephritis
- Oxalosis
- Vesicoureteral reflux (VUR)
Causes of renal artery stenosis
- FMD: mid to distal segment, string of pearls
- Atherosclerosis: ostium
- Takayasu arteritis: young woman with aortic involvement, ostium, long segment
- NF-1: ostial and long segment
What is Waterhouse-Friderichsen syndrome
Atraumatic adrenal haemorrhage in consequence of septicaemia
Causative agents of Waterhouse-Friderichsen syndrome
Neisseria meningitidis (meningococcus): classically-described cause
Haemophilus influenzae
Pseudomonas aeruginosa
Escherichia coli
Streptococcus pneumoniae
Explain embryology of Horsehore kidney
Ascent into the abdomen is restricted by the inferior mesenteric artery (IMA) which hooks over the isthmus. Hence horseshoe kidneys are low lying.
Connected by an isthmus of either functioning renal parenchyma or fibrous tissue. In the vast majority of cases, the fusion is between the lower poles (90%)
Explain embryology of crossed fused renal rectopia
Consequence of abnormal renal ascent in embryogenesis with the fusion of the kidneys within the pelvis.
The normal ascent of the kidneys is required for the formation of the extraperitoneal perirenal fascial planes and therefore ectopia (or renal agenesis) results in failure of development of fascial layers in the flanks on the side not occupied by renal tissue.
The lack of restraining fascia leads to possible malposition of bowel into the extraperitoneal fat of the empty renal fossa and relaxation of mesenteric supports for bowel loops in this region.
Spermatic cord mass differentials
Spermatic cord lipoma / liposarcoma (most common mass)
Rhabdomyosarcoma (most common; ~40% of paratesticular tumours)
Others:
leiomyoma/leiomyosarcoma, schwannoma, malignant fibrous histiocytoma, and fibrous pseudotumour.
Epididymal mass differentials
Adenomatoid tumour (most common epididymal mass)
Epididymal leiomyoma
Epidermoid inclusion cyst
Epididymal papillary cystadenoma (associated with von Hippel Lindau disease)
Clincal presentation of FMD
Hypertension, or less commonly renal impairment, due to renal artery stenosis
CNS symptoms (e.g. headache, neck pain, pulsatile tinnitus, Horner syndrome) from transient ischemic attack, stroke, or dissection due to carotid and vertebral artery involvement
Angina, myocardial infarction or sudden cardiac death due to coronary artery involvement
Mesenteric ischemia (mesenteric infarction is rare due to the formation of collateral supply)
Most common layer affected by FMD ?
Fibromuscular dysplasia is classified into five categories according to the vessel wall layer affected:
Media (95% affected)
Scleroderma renal findings ?
- Spotted nephrogram
Addisons disease cause ?
Adrenal insufficiency caused by:
- TB
- Idiopathic
- Waterhouse Fredericson
Lab findings of Addisions disease
chemistry: hyponatraemia, hyperkalaemia, azotemia, hypercalcaemia, hypoglycaemia
What test to do for Addisions ?
Adrenocorticotrophic hormone (ACTH) stimulation test:
1. primary adrenal insufficiency
- cortisol levels fail to rise after synthetic ACTH stimulation due to the destruction of the adrenal cortex
- secondary adrenal insufficiency
- cortisol levels rise after synthetic ACTH stimulation
- this indicates decreased ACTH secretion due to pituitary dysfunction
Sclerosing encapsulating peritonitis causes?
- Dialysis
- TB
Sclerosing mesenteritis (mesenteric panniculitis) Associations ?
Malignancy
- Lymphoma (up to 10% risk of developing lymphoma)
Recent abdominal surgery
Systemic inflammatory conditions
- Retroperitoneal fibrosis
- Sclerosing cholangitis
- Riedel thyroiditis
3 stages of Sclerosing mesenteritis (mesenteric panniculitis)
- mesenteric lipodystrophy: degeneration of mesenteric fat
- mesenteric panniculitis: inflammatory reaction
- retractile mesenteritis / sclerosing mesenteritis: fibrosis, which may be associated with distortion or lymphatic obstruction
Retroperitoneal fibrosis differentials
Retroperitoneal Lymphoma
Retroperitoneal Erdheim-Chester disease
- Hairy kidney
- inferior vena cava and pelvic ureters are typically spared (c.f Retroperitoneal fibrosis often involves)
- Coated aorta (c.f. Retroperitoneal fibrosis usually ascends from aortic bifurcation)
Extramedullary haematopoiesis (rare)
Causes of anterior vs posterior urethral injury
posterior urethral injury is caused by a crushing force to the pelvis due to the urethra fixed attachment to pelvic bones 8. Such injury is associated with pelvic fractures (~10%) and bladder injury
anterior urethral injury is usually caused by a straddle injury and is an isolated injury
Complication post urethral injury
Urethral stricture
Why is it important to differentiate anterior to posterior urethral injury
Both types of urethral injuries require careful diagnosis and management, with the anterior injury often being more straightforward, while posterior injuries usually demand more complex delayed repairs due to their association with pelvic trauma.
Initial Management:
- Urinary catheter, suprapubic catheter
Definitive Management
- Urethroplasty, end-to-end anastomosis
Varicocele primary cause ? which side more common
Primary varicocele
- result from incompetent or congenitally absent valves in the testicular vein
- Left varicocele more common due to longer course (more back pressure)
Secondary causes of Varicocele
- nutcracker syndrome
- renal mass
- renal vein thrombosis
Varicocele ultrasound findings
dilatation of pampiniform plexus veins >2-3 mm in diameter
characteristically have a serpiginous appearance
there can be flow reversal with the Valsalva maneuver
Doppler ultrasound can be used to grade the degree of reflux
Neurogenic bladder causes ?
- Post stroke
- Parkinsons
- Multiple sclerosis
Why is it important to differentiate Emphysematous pyelitis to emphysematous pyelonephritis ?
Emphysematous pyelitis generally carries a good prognosis and usually responds to intravenous antibiotics followed by oral antibiotics
