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55 Cards in this Set
- Front
- Back
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• Almost exclusively seen in patients with sickle cell trait or sickle
cell disease (MCQ) |
renal med carcinoma Almost exclusively seen in
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renal med carcinoma Almost exclusively seen in
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• Almost exclusively seen in patients with sickle cell trait or sickle
cell disease (MCQ) |
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PLEOMORPHIC CELLS
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Medullary Renal Carcinoma
Key Points to Remember • Unique association with sickle cell trait or sickle cell disease • Patients are young • Black or Mediterranean race because of the association with sickle cell disease • PLEOMORPHIC CELLS • Patients present with advanced disease • Die within 1 year |
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aka nephroblastoma
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wilms tuma
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• Also known as “Nephroblastoma
• Pediatric tumor |
wilms tumor is called this and found in this age
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wilms tumor arises from
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Arises from NEPHROGENIC RESTS in kidney
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Arises from NEPHROGENIC RESTS in kidney
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wilms tumor arises from
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• NEPHROGENIC REST is:
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Persistent embryonal renal tissue causing wilms tumor
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– Deletion of chromosome 11p13
– WT1 GENE |
familial wilms
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familial wilms
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– Deletion of chromosome 11p13
– WT1 GENE - multiple bilateral tumors |
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– Multiple tumors
– Bilateral tumors – Deletion of chromosome 11p13 – WT1 GENE |
• Familial Wilms
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1. WAGR syndrome
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Wilms’, Aniridia, GU
malformation, Retardation seen in familial wilms |
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Denys‐Drash syndrome
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Denys‐Drash syndrome
• WT, glomerulopathy, gonadal dysgenesis and renal failure seen in familial wilms |
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Beckwith‐Weideman
syndrome |
seen in familial wilms
• WT, hemihypertrophy, large organs and tongue |
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WT, hemihypertrophy, large organs
and tongue |
Beckwith‐Weideman
syndrome |
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WT, glomerulopathy, gonadal
dysgenesis and renal failure |
Denys‐Drash syndrome
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Wilms’, Aniridia, GU
malformation, Retardation |
WAGR syndrome
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Sporadic Wilms is like what
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Patients are less likely to have
multiple or bilateral tumors – Some sporadic cases have WT1 gene mutations and deletions |
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Primitive small round blue cells, tubules and spindle cells
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histo of wilms
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histo of wilms
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primitive round blue cells, tubules and spindle cells
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• Most are resectable
• Prognosis depends on stage • Advanced stage: – Worst prognosis |
wilms
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• MALIGNANT PEDIATRIC TUMOR
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wilms
most resectable |
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Wilms’ Tumor
Key Points to Remember |
• A.K.A Nephroblastoma
• MALIGNANT PEDIATRIC TUMOR • Arises from embryonal nephrogenic rests • Primitive cells seen – Spindled, blastema (blue cells), tubules/ • Sporadic and familial – Remember the chromosomal/gene abnormalities • WT1 gene on chromosome 11 – Associated syndromes • Prognosis related to stage and histology – Better differentiated → better prognosis and vice versa |
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Birt‐Hogg‐Dube syndrome
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Oncocytoma
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Characteristic “central scar”
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Oncocytoma
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“Mahogany brown” color on
gross examination • Composed of large eosinophilic cells |
oncocytoma
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• Benign renal neoplasm
• 5% of all renal masses • Derived from intercalated cells in collecting ducts |
oncocytoma
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what type of cells are oncocytomas composed of
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large eosinophillic ones
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decribe the oncocytomas gross feature
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central scar and mahogony color
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• Sheets of brightly
eosinophilic cells • Bland in appearance |
oncocytoma
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causes the cells to appear bright pink in oncocytoma
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Cytoplasm filled with mitochondria causes
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Oncocytoma
• Benign • Good prognosis • Resection is curative – Unless patient has multiple tumors some of which may be microscopic • More often seen in |
birt hogg dube
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Oncocytoma
Key Points to Remember |
• Most common benign renal tumor
• Derived from intercalated cells in collecting ducts • Sporadic and familial – Specific associated syndrome (Birt • Characteristic “central scar” and “mahogany brown” color • Composed of large eosinophilic • Characteristic EM findings – Abundant mitochondria Birt‐Hogg‐Dube syndrome) cells |
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from the renal pelvis
to the urethra |
transitional cell epithelium
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– Papillary fronds
– Lined by 7 layers of cells – Fibrovascular core |
benign papilloma of the urothelium
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decribe a benign papilloma of the urothelium
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– Papillary fronds
– Lined by 7 layers of cells – Fibrovascular core |
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Urothelial Carcinoma
• Risk factors: |
• Smoking 3‐7 fold ↑ risk
– Longer duration of smoking (60 yrs or >) – Number of cigarettes/day • Occupation – Exposure to aromatic amines in dye industry » Benzidine, 1‐ or 2‐naphthylamine » Makers of leather products including furniture , leather jackets – Cancer develops 15‐40 years after exposure • Drugs – Long term use of analgesics – Chemotherapy » Cyclophosphamide use • Chronic UTI or stones • Arsenic • Radiation – Entire urothelium is exposed |
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• Account for up to 60% of
superficial papillary tumors • May or may not be invasive |
Low Grade UC
• Chromosome 9 monosomy, deletions (9p and 9q) – Tumor suppressor gene p16INK4a |
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Low Grade UC
• Chromosome 9 monosomy, deletions (9p and 9q) – Tumor suppressor gene ????? |
p16INK4a
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Low grad UC genetics profile?
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• Chromosome 9 monosomy,
deletions (9p and 9q) – Tumor suppressor gene p16INK4a • Account for up to 60% of superficial papillary tumors • May or may not be invasive |
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High Grade UC genetic profile
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• Chromosome 17p, 11p, 13q
and 14q deletions • Chr 17p deletions • Involve p53 • Chr 13q deletions • Involve the Retinoblastoma gene • Often invasive carcinomas |
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7-11
13-14 what does this mean to you? |
High Grade UC
• Chromosome 17p, 11p, 13q and 14q deletions • Chr 17p deletions • Involve p53 • Chr 13q deletions • Involve the Retinoblastoma gene • Often invasive carcinomas |
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• Papillary architecture
• Greater than 7 layers thick • Well differentiated tumors |
non invasive PUC
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whats noninvasic low grade PUC like
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Low grade tumors (PUC)
• Papillary architecture • Greater than 7 layers thick • Well differentiated tumors Noninvasive Papillary Urothelial Carcinoma (PUC) • Mild nuclear atypia • Few mitoses • Unlikely to invade lamina propria • But 70% of these tumors recur |
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Unlikely to invade lamina
propria |
noninvasic low grade PUC like
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• Papillary architecture
• Greater than 7 layers thick • Poorly differentiated tumors |
high grade noninvasive PUC
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• More likely to invade the
lamina propria |
high grade noninvasive PUC
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low grade noninvasive PUC almost looks just like:
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normal urothelium
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Tumor cells invade the underlying muscle
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invading UC
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• Non‐invasive urothelial neoplasms can be successfully
treated via |
• Wide excision
• Ablation • BCG therapy • Radiation |
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Prognosis of Urothelial Carcinoma
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Prognosis of Urothelial Carcinoma
• 70% of non‐invasive tumors recur • Non‐invasive urothelial neoplasms can be successfully treated • Wide excision • Ablation • BCG therapy • Radiation • 30% of non‐invasive tumors progress to invasive UC • Invasive UC is usually treated with radical • Invasive tumors have a poor prognosis • Prognosis is related to the depth of invasion into the bladder wall and the presence or absence of metastatic disease |
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• Invasive UC is usually treated with
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radical cystectomy
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• Squamous cell carcinoBmlaa odf bdlaedrd er
• Rare, 5% of bladder tumors • People at risk include those with:????????? |
– Chronic irritation of bladder mucosa
– Chronic indwelling catheters • Paraplegics • Neurogenic bladder (diabetes) • Bladder stones – Schistosomiasis • West Africa, Egypt • Invasive, aggressive • Poor prognosis |
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– Schistosomiasis
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Non‐Urothelial Carcinoma of the
• Squamous cell carcinoBmlaa odf bdlaedrd er |
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Key Points to Remember
Ureteral and Bladder Tumors |
• Bladder and ureteral neoplasms are derived from urothelial
cells (a.k.a transitional cells) • Benign • Malignant • Urothelial carcinoma • Specific risk factors Common molecular cytogenetic abnormalities • • Can be flat or papillary • Can be low or high grade • Low grade recurs often • Can be invasive or non‐invasive • Poor prognosis if invasive • Other rare histologic subtypes include: • Squamous carcinoma • Risk factors • Adenocarcinoma |
