Urinary Tract Tumours Flashcards
Describe acquired (dialysis associated) renal cystic disease?
- Seen in 40-50% of dialysis patients; thought to be related to uremia, not dialysis per se
- Bilateral + affects all parts of kidney
- Increased (7x) risk of renal cell carcinoma
Describe the gross morphology of acquired (dialysis associated) renal cystic disease?
- moderately enlarged kidneys with cysts up to 2 cm containing clear fluid
- papillary hyperplasia common
What is the micro morphology of Acquired (dialysis associated) renal cystic disease?
may have atypical epithelial proliferations
Name inherited cystic non-neoplastic lesions?
- Adult polycystic kidney disease (DPKD)
- ## Childhood polycystic kidney disease (RPKD)
What is adult polycystic kidney disease?
- Autosomal dominant, high penetrance
- 1/1000 live births
- causes 10% of chronic renal failure requiring transplantation or dialysis
- Multiple expanding cysts of both kidneys destroys renal parenchyma slowly
What is DPKD associated with?
- hepatic cysts, berry aneurysms, mitral valve prolapse, cysts in pancreas, lung, spleen, pineal gland, seminal vesicles; aortic aneurysms, hepatic fibrosis, intestinal diverticula
- loss of ability to concentrate urine, hypertension by age 20, infection, hematuria
- Cysts compress and obstruct only part of normal nephron, so normal renal function maintained until age 30-49
What is the cause of DPKD cysts?
unknown
Poor prognostic factors of DPKD?
- sickle cell trait (not disease)
- MEN
- hypertension
Molecular morphology of DPKD?
- PKD1 gene on Chrom. 16p13.3 (altered in 85% of cases) produces polycystin 1; function unknown
- PKD2 gene on 4q22 (altered in 10% of cases) produces polycystin 2
- PKD3 gene: minority of cases, gene unmapped
What is the gross morphology of DPKD?
- BIG kidneys (up to 4kg)
- bilateral, bosselated outer surface composed of mass of cysts up to 4 cm
Micro morphology of DPKD?
- functional nephrons between cysts
- occasionally papillary projections and polyps protrude into lumen
- tubular atrophy and pyelonephritis
What is Childhood polycystic kidney disease (RPKD)?
- Autosomal recessive
- rare (1/6000 births)
- bilaterally enlarged kidneys at birth
- Mutation on chromosome 6
- children often die in childhood
- Liver always affected : congenital hepatic fibrosis
- no cysts in other organs
Gross morphology of RPKD?
- markedly enlarged kidneys with smooth surface
- small cysts in cortex and medulla
- dilated channels are perpendicular to cortical surface
- cysts present in medulla (collecting duct)
Micro morphology of RPKD?
- dilatation of all collecting tubules
- cysts lined by cuboidal cells (from collecting tubules)
What is Wilms tumour??
Aka Nephroblastoma
- paediatric tumour
- Most common kidney tumor of childhood
- Usually ages 2-5, occasionally in teenagers or adults
What is the presentation of Wilms tumour?
- Large abdominal mass felt by mother holding the child
- Bilateral tumor is associated with urogenital congenital abnormalities
Gross morphology of Wilms tumour?
- large, solitary, well-circumscribed mass soft, homogenous, tan-gray
- hemorrhage, necrosis, cysts are common,
Describe the spread of Wilms tumour?
- into perirenal soft tissue, may invade renal vein
- metastasizes to lymph nodes, lung, liver, peritoneum, rarely bone
Micro morphology of Wilms?
- triphasic
- with undifferentiated blastema (small blue cells with scanty cytoplasm)
- stroma (fibroblast-like)
- epithelium (abortive tubules, glomeruli), - may have smooth muscle, cartilage, adipose tissue, squamous/mucinous epithelium, bone, neural tissue
- Prominent skeletal muscle component is associated with bilateral tumors in 50% of cases
Differential diagnosis of Wilms?
- small blue cell tumors-neuroblastoma
- multicystic nephroma
- renal cell carcinoma
Describe what constitutes a poor prognosis?
- Stage and grade of the tumor
- anaplasia (large, hyperchromatic, pleomorphic nuclei 3 x normal and abnormal mitotic figures)
- tumors with rhabdomyomatous features
- age >2 years
- Overall 90% long term survival
What is renal cell carcinoma?
Usually > 50 years old, M/F = 2:1
Risk factors for renal cell carcinoma?
tuberous sclerosis, von Hippel-Lindau disease, renal transplantation, dialysis
Clinical presentation of renal cell carcinoma?
“Classic” clinical features of costoverterbral pain, palpable mass and hematuria - present in only 10%
“Great mimic” due to associated paraneoplastic syndromes (hypercalcemia, hypertension, gynecomastia, Cushing’s syndrome
Presents 25% of time as metastases (lung, bones, lymph nodes, adrenals, liver, brain)
Variants of renal cell carcinoma?
Clear cell type Papillary type Medullary carcinoma Chromophobe type Sarcomatoid carcinoma Oncocytoma
Prognostic factors for RCC?
stage and nuclear grade most important
Treatment for RCC?
- radical nephrectomy
- partial nephrectomy for small peripheral tumors
- cure possible even with extension into renal vein, inferior vena cava and right atrium
Note: chemotherapy ineffective
5 year survival - 45%
Gross morphology of RCC?
well circumscribed, centered on cortex, often extends into renal vein or vena cava
Differential diagnosis of RCC?
clear cell hepatocellular carcinoma
Describe Urothelial carcinoma of renal pelvis?
Present with hematuria due to fragmentation within renal pelvis
5 year survival 10% due to invasion of wall of pelvis and calyces.
May be associated with phenactin nephropathy, Thorotrast radiologic dye or cyclophosphamide
May have increased incidence in horseshoe kidneys
50% have preexisting or coexisting bladder urothelial tumors
Treatment for urothelial carcinomas of the renal pelvis?
nephroureterectomy including bladder cuff since multicentric
Gross morphology of UCs of the renal pelvis?
soft, gray-red masses with smooth glistening surfaces
Micro morphology of UCs of the renal pelvis?
resemble other urothelial carcinomas
carcinoma in situ may be present in non-adjacent areas
What are urothelial carcinomas of the bladder?
90% of bladder tumors
Arise anywhere in bladder often multifocal
M > F, cigarettes, urban, age 50+
Causes of urothelial carcinoma of the bladder?
cigarette smoking (50-80% of cancers); arylamines (2-naphthylamine) Schistosoma haematobium (ova are deposited in bladder wall chronic inflammatory response, squamous metaplasia, dysplasia; 70% are squamous cell carcinomas) phenacetin use (usually long term use in younger women, tumors involve upper collecting system)
Describe the clinical course of UCs of the bladder?
initial symptoms are painless hematuria, infection, obstruction if near ureteral orifices
10 year survival only 40% for high grade tumors
Metastases to lymph nodes, lung, liver, bone, CNS
Describe the prognostic factors of UCs of the bladder?
Tumor stage-most important, Nodal involvement
Grade (high grade vs. low grade), Age
Site (ureter, lateral wall - tend to be low grade; dome - high grade; bladder neck poor prognosis), and Vascular invasion
What is squamous cell carcinoma of the bladder?
5% of tumors in US, more common in Egypt/Sudan; mixed urothelial and squamous carcinomas are more common
Arises in background of chronic cystitis with squamous metaplasia, also bladder exstrophy, neurogenic bladder, chronic infection, stones, chronic indwelling catheters, prolonged cyclophosphamide treatment, Schistosoma haematobium.
Also associated with smoking
Gross morphology of SCC of the bladder?
large, ulcerated, necrotic
Micro morphology of SCC of the bladder?
usually poorly differentiated with muscularis propria invasion; rarely is papillary
