Renal & Urinary Neoplasia Flashcards
What is an angiomyolipoma associated with?
Tuberous sclerosis
How would you classify an angiomyolipoma?
Hamartoma
Where does a renal cell carcinoma usually arise from?
Kidney tubules
What is the classic triad of someone presenting with renal cell carcinoma?
Hematuria, palpable abdominal mass, flank pain –> rarely see all three together!
Name some other symptoms associated with renal cell carcinoma?
- Systemic - fever, weight loss
- Paraneoplastic syndromes: reactive polycythemia, HTN, Hypercalcemia, Cushing’s
- Left-sided varicocele (rare)
What does renal cell carcinoma kidneys look like
- Gross appearance
- Microscopic appearance
- Yellow mass
2. Clear cytoplasm (most common)
Which gene is affected in renal cell carcinoma? What chromosome is it on?
Tumor suppressor gene VHL on chromosome 3p
What are the consequences of the loss of the gene involved in renal cell carcinoma?
- Increased IGF-1 –> promotes growth
2. Increased HIF –> promotes VEGF & PDGF
Sporadic renal cell carcinoma tumors are commonly seen in which population group? Where is this tumor classically located?
Adult males (peak in 60s); single tumor in upper pole of kidney
What is the greatest risk factor for developing a sporadic renal cell carcinoma?
Cigarette smoke!!!
Hereditary renal cell carcinoma tumors are commonly seen in which population group? Where is this tumor classically located?
= Von Hippel Lindau disease; in younger adults; often bilateral tumors
Besides renal cell carcinoma, what else is Von Hippel Lindau disease associated with?
Most commonly hemangioblastomas in cerebellum; but tumors can also form in KAPICKE: kidney 1, adrenal gland, pancreas, inner ear, CNS, kidney 2, eyes
What is the inheritance pattern for von Hippel lindau disease?
Autosomal dominant
What vein is commonly involved in renal cell carcinoma? What risk does this impose?
Renal vein –> can allow hematogenous spread to lungs and bones
What lymph nodes does renal cell carcinoma typically spread to?
Retroperitoneal lymph nodes
A Wilms tumor is composed of which types of cells?
Blastema = immature kidney mesenchyme
Primitive glomeruli and tubules
Stromal cells
What is the most common malignant renal tumor in children?
Wilms tumor
What is the typical presentation of someone with a Wilms tumor?
Three year old with a large, unilateral flank mass, hematuria, HTN
What mutation is a Wilms tumor associated with?
WWTI mutation
What are the two types of syndromes a Wilms tumor can result in?
- WAGR Syndrome: Wilms tumor, Aniridia, Genital abnormalities, mental/motor Retardation
- Beckwith-Wiedermann Syndrome: Wilms tumor, neonatal hypoglycemia, muscular hemihypertrophy, organomegaly
What are the three types of lower urinary tract carcinomas?
- Transitional cell –> urothelial carcinoma (entire LUT, usually bladder)
- Squamous cell carcinoma (usually bladder)
- Glandular cells –> adenocarcinoma (usually bladder)
What is the most common type of lower urinary tract carcinoma?
Urothelilal/ transitional cell carcinoma
What region do lower urinary tract cancers usually involve?
Bladder
What is the major risk factor for urothelial carcinoma? Can you name some others?
Cigarette smoke! Others include napthylamine, azo dyes, long-term cyclophosphamide or phenacetin use
Describe the classic clinical presentation of someone with urothelial carcinoma.
Older adult presenting with PAINLESS hematuria
What are the two pathways through which urothelial carcinomas can develop?
- Flat
2. Papillary
Describe features of urothelial carcinoma developing by the flat pathway.
- Starts as high-grade flat tumor then invades
2. Associated with early p53 mutations
Describe features of urothelial carcinoma developing by the papillary pathway.
- Starts as low-grade papillary then progresses to high-grade papillary and then invades
- Not associated with early p53 mutations
Describe the ‘field defect’ associated with urothelial carcinomas.
Entire urothelial surface hit with carcinogens –> entire field mutated –> see multifocal tumors that recur
What usually precedes a squamous cell carcinoma?
Squamous metaplasia –> normal bladder surface is usually transitional epithelium:
Squamous metaplasia –> dysplasia –> carcinoma
Name three risk factors for developing squamous cell carcinoma.
- Chronic cystitis (older woman)
- Schistosoma hematobium infection (Egyptian male)
- Long-standing nephrolithiasis (chronic irritation leads to development of metaplasia)
What are three conditions from which an adenocarcinoma of the bladder arises?
- Urachal remnant –> urachus lined by grandular cells & passes through dome of bladder
- Cystitis glandularis: chronic bladder inflammation –> columnar metaplasia
- Exstrophy –> congenital failure to form caudal portion of anterior abdominal & bladder walls –> bladder surface exposed to outside world
