Malignant renal tumors 2. Nephroblastoma (Wilm s tumor)
Transcript of Malignant renal tumors 2. Nephroblastoma (Wilm s tumor)
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Malignant renal tumors
2. Nephroblastoma (Wilm’s tumor)
• Uncommon renal tumor but one of the commonestchildhood neoplasms (2-5 yrs).
• Sporadic or familial associated with congenitalmalformations due to WT-1 or WT-2 genesmutaions on chr.11:
WAGR syndrome.
Denys-Drash syndrome (DDS).
Beckwith-Wiedemann syndrome.
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Morphology
• Gross:
▫ Large, well-circumscribed soft,tan-gray, homogenous tumor.
• Microscopic*:
▫ Classic TRIPHASICcomponents:
Blastemal: sheets of undismallblue cells
Epithelial**: abortive tubules
or glomeruli.
Stromal.
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Wilm’s tumor
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Wilm’s tumor
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Clinical features
• Palpable abdominal mass, (may cross themidline) + hematuria, abdominal pain orhypertension
• Prognosis:
▫ Generally good (currently 90% long term survival).
• Treatment:
▫ Combination of nephrectomy & chemotherapy
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Malignant renal tumors
3. Urothelial Carcinoma of the Renal Pelvis
• 5-10% of renal neoplasms
• Often small and present early with, due to:
▫ Painless hematuria.
▫ Pain or mass due to hydronephrosis.
• Linked to Lynch syndrome & analgesicnephropathy.
• In 50% of renal pelvic tumors there is apreexisting or concomitant bladder urothelialtumor* (field effect).
• Prognosis: Variable, depend on stage & grade.
Despite removal by nephrectomy 50% 5 yrs
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Urothelial carcinoma of renal pelvis
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Ureters
• Sites of narrowing:
▫ Ureteropelvic junction.
▫ Ureterovesical junction.
▫ Crossing of iliac vessels.
• In female pelvis, they lie close to uterine arteries
and the cervix. Operations of the female genital
tract and diseases of the cervix and uterus may
affect ureters.
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Causes of Ureteral Obstruction
Intrinsic
▫ Calculi
▫ Strictures
▫ Tumors
▫ Blood clots
▫ Neurogenic
Extrinsic
▫ Pregnancy
▫ Retroperitoneal
fibrosis*
▫ Endometriosis
▫ Tumors
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• Epidemiology:
o 7% of cancers & 3% of cancer deaths.
o M>F. 50-80 yrs.
• Types:
Transitional (urothelial) cell carcinoma (>90% ).
Squamous cell carcinoma (5-7%).
Adenocarcinoma (~2%).
Others.
Urinary bladder
5. Carcinoma of urinary bladder
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• May involve any site of the urinary collectingsystem from renal pelvis to urethra Mostcommonly seen in the bladder.
• Many are multifocal at presentation
Urothelial (transitional cell) neoplasms
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Urothelial carcinoma – Etiology
•Mostly are sporadicwith unknowncause.• Possible causes:▫ Cigarette smoking
(most important).▫ Dye and rubber
industry Aromatic amines 2 -
naphthylamine 4-aminobiphenyl Benzidine. Azo dyes.
▫ Cyclophosphamide
▫ Phenacetin containinganalgesics
▫ S. Hematobiuminfestation(SCC+TCC).
▫ Previous radiation.
• Induction time~22yrs.
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Papillary (commoner)
Flat
Non-invasive WHO/ISUP grade:Urothelial papilloma
Papillary urothelialneoplasms of low malignant potential (PUNLMP)
Papillary urothelialcarcinoma, low grade
Papillary urothelialcarcinoma, high grade
Carcinoma in-situBy definition high grade
Invasive Papillary and invasiveMore in high grade
Flat and invasive
Classification of urothelial neoplasms
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Morphologic patterns of bladder tumors
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Exophytic tumor
(Most arise from the lateral or posterior walls)
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Papilloma
-Small papillary fronds lined by normal-appearing urothelium
-Some have endophytic growth (inverted papilloma)
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PUNLMP
Resembles papilloma but larger & is covered by thicker urothelium
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Low-grade papillary urothelial carcinoma
Mild degree of atypia & mitosis with preserved polarity of cells; the cells
are evenly spaced and cohesive
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High-grade papillary urothelial carcinoma
Increased atypica & mitosis with loss of polarity & dyscohesive cells
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Carcinoma in situ (CIS) or flat urothelial carcinoma
-Defined by the presence of any cytologically malignant cells within a
flat urothelium (high grade by definition).
-Accounts for less than 1-3% of urothelial neoplasms seen in
association with invasive carcinoma (45-65% )
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• May be flat or papillary, low or high grade:
▫ < 10% of low-grade cancers are invasive.
▫ 80% of high-grade carcinomas (including CIS) areinvasive.
• May be superficial (to lamina propria) or deeper(to muscularis propria).
▫ The extent of the invasion is the mostimportant prognostic factor
Invasive urothelial cancer
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Non-invasive vs invasive TCC
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Other bladder tumors
• Squamous cell carcinoma (5%), linked to: Schistosomiasis
Chronic bladder irritation.
Tobacco smoking
• Adenocarcinoma (2%), linked to: Urachal remnants.
Bladder exstrophy.
Cystitis glandularis.
• Sarcomas.
• Paraganglioma (micturition syncope).
• Carcinoid.
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• Painless hematuria is the dominantsymptom.
• Lesions that invade the ureteral or urethralorifices cause urinary tract obstruction.
• Prognosis:
▫ Overall 5-year survival is 57%.
▫ Depends on:
The depth of invasion of the lesion (stage).
Histologic grade.
Clinical features & prognosis
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Urothelial (Transitional) Cell Carcinoma
Gross appearance
Invasion
Adjacent dysplasia & CIS
Recurrences
Progression of disease
10 year survival
Papillary
<10%
none
50%
<10%
>90%
Nodular
flat, papillary
80%-90%
Frequent
80%-90%
65%
40%
Low grade High grade