Urinary Tract Flashcards
what is the most typical presenting symptom of bladder cancer
Painless hematuria, maybe dysuria
How common is bladder cancer
6th most common cancer in the US
What are some treatments for bladder cancer
Surgery, single-dose intravesical or systemic chemotherapy, radiation
What cancer syndromes can be associated with bladder cancer?
Lynch syndrome, Cowden syndrome, RB1
How common are ureter and renal pelvis cancers?
Very rare, only account for 4% of urothelial malignancies
How common is kidney cancer?
8th most common cancer in the US
What are the symptoms of kidney cancer?
Hematuria, low back pain, fatigue, weight loss
What are some treatments for kidney cancer?
Partial or total nephrectomy, chemotherapy, radiation (if mets)
What are risk factors for bladder cancer
Smoking, male, chronic bladder infections/irritations, workplace exposures, not drinking enough water, chemotherapy/radiation
What are risk factors for kidney cancer
Smoking, obesity, hypertension, family history, male, advanced kidney disease, workplace exposures
What are some red flags for an inherited kidney cancer?
First or Second degree relative with kidney cancer, onset under 46 y/o, multiple or bilateral renal masses, personal/family history consistent with syndrome, pathology consistent with syndrome
What pathologies of kidney cancers are associated with what genes?
Clear cell- VHL, BAP1
Papillary Type 1- MET
Chromophobe, hybrid, oncocytoma- FLCN
Papillary Type 2- FH
Oncocytic- SDHB, SDHC, SDHD
Angiomyolipoma- TSC1, TSC2
What is the inheritance pattern and de novo rate for VHL?
AD inheritance
20% de novo rate
highly penetrant
What are the common types of abdominal VHL lesions?
Renal: multiple or bilateral renal cysts, renal cell carcinoma occurs in 7-% by age 60
Pheo/PGL: usually benign, can be functional leading to hypertension
Pancreatic: benign cysts that can be numerous, but rarely symptomatic. Neuroendocrine tumors occur in about 15017% of individuals, nonfunctioning, but can be symptomatic due to the obstruction.
What are the properties of hemangioblastomas in VHL?
Blood vessel-rich tumors in brain, spinal cord, or retina that generally are slow growing
Symptoms occur due to pressure on adjacent nerves and tissues (include headache, visual disturbances, pain, vomiting, or ataxia)
Not treated until symptoms arise, treated with surgery or radiation
What are the main lesions seen in VHL?
Hemangioblastomas, abdominal (renal, pheo/PGL, pancreatic), endolymphatic sac tumors, epididymal/broad ligament cystadenomas
What are the guidelines for VHL surveillance?
MRI of brain and total spine every 2 years starting at age 16, Abdominal ultrasounds annually starting at age 8, MRI scan of the abdomen every 2 years starting at age 16, ophthalmology evaluation annually starting at age 1, plasma or 24-hour urine tests annually starting at age 5, audiology assessment every 203 yers starting at age 5 and increasing frequency if hearing loss, tinnitus or vertigo present)
What are the features of Wilms Tumor?
Most frequent kidney tumor in infants/children (avg age of onset, 3-4 years)
~10% children have association congenital malformation syndrome
May have associated hemihyperplasia and urinary tract anomalies
Describe WAGR
Wilms tumor (45-50% risk)
Aniridia (no iris)
Genitourinary anomaly (cryptorchidism, nonfunctional ovaries, bicordinate uterus)
Mental Retardation (gross)
del 11p13 (WT1 and PAX6 genes included)
0.4% prevalence in children with Wilms tumor
What syndromes can be associated with an increased risk of Wilms tumor?
Frasier syndrome
Denys-Drash syndrome
WAGR
Beckwith-Wiedemann syndrome
Perlman syndrome
Simpson-Golabi-Behmei syndrome
CLOVES syndrome
Sotos syndrome
9q22.3 microdeletion
Bloom syndrome
Li-Fraumeni syndrome
Alagille syndrome
Bohring-Opitz syndrome
What adult-onset syndromes can be associated with renal cell cancer?
Birt-Hogg-Dube
Hereditary papillary RCC
BAP1-associated tumor predisposition syndrome
MITF-associated cancer syndrome
Hereditary leiomyomatosis and RCC
Cowden
Tuberous Sclerosis
How common is prostate cancer?
3rd most common cancer in the US
1 in 9 men are diagnosed in their lifetime
