Chapter 21: Lower Urinary Tract - Ureters, Bladder, Urethra Flashcards
Most common cause of hydronephrosis in children/infants?
Ureteropelvic junction (UPJ) obstruction
If Ureteropelvic junction (UPJ) Obstruction occurs in children which sex is preferentially affected?
Bilateral or unilateral?
In adults?
- Children —> males; sometimes bilateral
- Adults —> woman; most often unilateral
About 1/3 of children w/ recurrent UTI’s are found to have what?
Vesicoureteral reflux; most commonly dx in infancy/childhood
Although rare, what is the most common primary malignant tumor of the Ureters?
Most often occurs when and causes what?
- Urothelial carcinomas
- 6th-7th decade of life causing obstruction
What is the most common malignant mesenchymal tumor of the bladder seen in infants/children?
Often seen manifesting with what type of growth pattern?
- Embryonal rhabdomyosarcoma
- Manifest as a polypoid grapelike mass = Sarcoma botryoides
What is the most common malignant mesenchymal tumor seen in the bladder of adults?
Leiomyosarcoma
What are the 2 distinct precursor lesions of invasive urothelial carcinoma of the bladder and which is most common?
Type of grade for each?
- Papillary urothelial carcinoma (low- or high-grade) = most common
- Flat urothelial carcinoma in situ (uniformly high grade)
Non-invasive high-grade urothelial carcinoma is associated with what genetic mutations allowing for progression to invasion?
Loss of TP53 or RB —> frequently progesses to muscle invasive dz
Non-invasive low-grade papillary urothelial carcinoma is associated with what genetic mutations?
Gain of function FGFR3 and HRAS mutations
What are 6 risk factors associated with urothelial carcinoma of the bladder?
- Cigarette smoking –> 3-7x ↑ risk
- Industrial exposure to aryl amines
- Schistosoma hematobium (SCC-70%)
- Long-term use of analgesics
- Long-term exposure to cyclophosphamide
- Radiation
Loss of genetic material on which chromosome are particularly common in urothelial carcinoma?
- Chromosome 9
- Monosomy or deletions of 9p and 9q
Which genetic mutation associated with urothelial carcinoma is a poor prognostic indicator?
p53
What are 2 treatment options utilized in the majority of patients with non-invasive urothelial carcinoma of the bladder?
- Transurethral resection (TUR) and surveillance
- Intravesical therapy - chemotherapy or instillation of an attenuated strain of Mycobacterium bovis called bacillus Calmette-Guerin (BCG)
What is the dominant and sometimes only clinical finding associated with urothelial carcinoma of the bladder?
Painless Hematuria
What is the critical prognostic factor for bladder cancer?
- Extent of spread (STAGE) = critical prognostic factor
- Muscle invasion i.e., depth of muscle invasion is major prognostic issue to be established
Major decrease in survival rates associated with bladder cancer is due to?
Invasion of the muscularis propria (detrusor m.)
Unilateral ureteral obstruction typically results from _______ causes, whereas bilateral obstruction arises from ________ causes
Unilateral ureteral obstruction typically results from proximal causes, whereas bilateral obstruction arises from distal causes
Sclerosing Retroperitoneal Fibrosis most often occurs in which age group and sex?
Characterized by and leads to what?
- M > F; occurs in middle to late age
- Fibrotic proliferative inflammatory process of retroperitoneal structures causing hydronephrosis –> urethral narrowing/obstruction
A subset of Sclerosing Retroperitoneal Fibrosis is associated with what immunological related disease?
IgG4-related disease
What are the 4 major extrinisic causes of Ureteral Obstruction?
- Pregnancy –> relaxation of smooth m. or pressure on ureters
- Periureteral inflammation –> salpingitis, diverticulitis, peritonitis
- Endometriosis
- Tumors
Most cases of Sclerosing Retroperitoneal Fibrosis arise how and are known as?
- No obvious cause = considered primary or idiopathic
- Ormond disease
What are some of the common secondary causes of retroperitoneal fibrosis, which may lead to ureteral obstruction?
- Drug exposures (ergot derivatives, β-blockers)
- Adjacent inflammatory conditions –> vasculitis, diverticulits, Chron’s
- Malignant disease –> lymphomas, urinary tract carcinomas
Initial treatment and long term treatment of Sclerosing Retroperitoneal Fibrosis?
- Corticosteroids
- Eventually require ureteral stents or surgical extrication of ureters from surrounding fibrous tissue (ureterolysis)
Acquired diverticulae of the bladder are most often seen in association with?
Clinical significance?
- Most often w/ prostatic enlargement (hyperplasia or neoplasia)
- Produce obstruction to urine outflow and marked bladder wall thickening
Bladder diverticulae are clinically significant because they may predispose to what?
- Sites of urinary stasis–>infectionand formingbladder calculi
- Predispose to vesicoureteral reflux if impinge on ureter
- Rarely, carcinoma may arise; tend to be in more advanced stage
The exposed bladder mucosa in exstrophy of the bladder may undergo what transformation?
Is subject to what?
- Colonic glandular metaplasia
- Subject to infections which may spread to upper levels of urinary tract
Patients with exstrophy of the bladder have an increased risk of what malignancy?
Adenocarcinoma arising in bladder remnant
Abdominal pain and fever in a 4 yo w/ a CT showing a heterogenous mass in the midline anterior to the bladder is consistent with what?
Infected urachal remnant/cyst
Which malignancy may potentially arise from a Urachal Cyst?
Bladder adenocarcinoma
Which gram negative, obligate intracellular parasite may cause cystitis?
Chlamydia
Which 2 organisms are a cause of cystitis in immunocompromised pts or those receiving long-term Abx?
- Candidia albicans = more common
- Cryptococcal agents
Which virus may causes cystitis and hemorrhagic cystitis?
Adenovirus
Classic triad of cystitis sx’s?
1) Frequency –> every 15-20 mins in acute cases
2) Lower abdominal pain - suprapubic region
3) Dysuria - pain or burning on urination
What are 2 patterns of chronic cystitis that are not always related to infection?
- Follicular cystitis
- Eosinophilic cystitis
Patients receiving which drugs may develop hemorrhagic cystitis and are also at an increased risk for carcinoma of the bladder?
Cytotoxic antitumor drugs, such as cyclophosphamide
How does chronic cystitis differ from acute in the nature of the inflammatory infiltrate?
Chronic associated with mononuclear inflammatory infiltrates
In acute cystitis how does the mucosa appear and what type of inflammatory infiltrate is present?
- Hyperemia of the mucosa
- Neutrophilic infiltrate, sometimes associated w/ exudate
Chronic, persistent, painful form of cystitis characterized by intermittent, often severe suprapubic pain, urinary frequency, urgency, hematuria and dysuria without evidence of bacterial infection is known as?
Interstitial Cystitis (Chronic Pelvic Pain Syndrome)
What will cystoscopy show in someome with Interstital Cystitis?
Fissures/punctate hemorrhages in bladder mucosa (glomerulations)
Chronic mucosal ulcers (Hunner ulcers) associated with Interstitial Cystitis is characterized by increased numbers of which immune cells?
Mucosal mast cells
What is Malakoplakia and what is it caused by?
Related to what infection?
- Chronic inflammatory reaction in the bladder
- Acquireddefectsinphagocyte functionand related to chronic infection, mainly byE. coliand occasionallyProteus
Malakoplakia occurs with increased frequency in which patients?
Immunosuppressed transplant recipients
Major histomorphological characteristics of the mucosal plaques associated w/ Malakoplakia?
Soft, yellow, slightly raised and filled w/ large foamy macrophages w/ occasional multinucleate giant cells + lymphocytes
What are the characteristic morphological findings of the macrophages associated w/ Malakoplakia?
- Macrophages w/ adundant granular cytoplasm
- Laminated mineralized concretions due to deposition of calcium in enlarged lysosomes, known as Michaelis-Gutmann bodies, present in macrophages
Polypoid Cystitis is most commonly due to what?
What is the characteristic morphology of the urothelium?
- Indwelling catheters (most common), but any injurious agent may cause
- Urothelium thrown into broad bulbous polypoid projections as result of marked submucosal edema
Polypoid Cystitis may be confused with what malignancy both clinically and histologically?
Papillary urothelial carcinoma
Cystitis glandularis and cystitis cystica are common lesions of the bladder characterized by what?
Nests of urothelium (Brunn nests) grow downward into lamina propria
Epithelial cells undergoing metaplasia in the center of Brunn nests taking on a cuboidal or columnar appearance is known as?
Cystitis glandularis
Which lesion is a result of implantation of shed renal tubular epithelial cells at sites of injured urothelium?
Nephrogenic adenoma
Implantation of shed renal tubular cells at sites of injured urothelium (nephrogenic adenoma), the overlying urothelium may be focally replaced by what?
Assuming what type of growth pattern?
- Replaced by cuboidal epithelium
- Assume a papillary growth pattern
In two-pathway model, low-grade superficial papillary tumors are characterized by what genetic mutations first and then second?
1) FGFR3 and RAS mutations + chromosome 9 deletions
2) May then lose TP53 or RB —> invasion