L83 - Urinary tumours Flashcards

(91 cards)

1
Q

Name 3 benign kidney tumours? PAO

A
  • Papillary adenoma
  • Angiomyolipoma
  • Oncocytoma
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2
Q

Name 6 malignant kidney tumours?

R P C C U N

A
  • Renal cell carcinoma, clear cell type
  • Papillary renal cell carcinoma
  • Chromophobe renal cell carcinoma
  • Collecting duct carcinoma
  • Urothelial carcinoma
  • Nephroblastoma (Wilms tumour) - childhood
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3
Q

Size and manifestation of papillary adenoma?

A

Benign
Small, discrete tumour
<5 mm in diameter

usually asymptomatic, little clinical significance

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4
Q

What cancer is similar to papillary adenoma in porphology but with much worse outcome?

A

renal cell carcinoma

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5
Q

LM appearance of papillary adenoma?

A

Fibrovascular core

Cytologically normal (low N:C, normochromatic)

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6
Q

Origin of angiomyolipoma?

A

Originating from perivascular epithelioid cells

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7
Q

Diagnotic features of angiomyolipoma?

A

** imaging by CT demonstrating fat
attenuation

Renal mass

Haematuria

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8
Q

What disease predisposes angiomyolipoma?

A

Hereditary disease – Tuberous sclerosis

• 25-50% of patients with this disease develop angiomyolipoma

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9
Q

Genetic nature of Tuberous sclerosis?

A

Autosomal dominant inheritance

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10
Q

Manifestations of tuberous sclerosis in kidney, liver, brain and heart?

A
  • Angiomyolipoma in kidney, liver
  • Astrocytic tumor in brain
  • Rhabdomyoma in heart
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11
Q

Tumour size and outcome of angiomyolipoma?

A

Large tumours (more than 4 cm)

> > present with haemorrhage if ruptures

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12
Q

Tumour morphology in Angiomyolipoma?

A
  • No capsule
  • Blood vessels ensheathed by smooth muscle cells
  • Lobules of adipose cells
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13
Q

LM appearance of angiomylolipoma?

A

Many clusters of clear, large cells» adipose tissue

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14
Q

Usual progression of angiomyolipoma in a patient with tuberous sclerosis?

A

Bilateral and multiple angiomyolipomas

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15
Q

Origin of renal oncocytoma?

A

arise from intercalated cells of collecting ducts

usually solitary

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16
Q

Histology of renal oncocytoma?

A

• Large eosinophilic cells with small round nuclei and large nucleoli

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17
Q

Oncocytomas can occur in different places in the body. How many % of renal neoplasms does it account for?

A

5-15%

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18
Q

LM gross appearance of oncocytoma?

A

Sheets of tumour cells

Abundant cytoplasm

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19
Q

Renal cell carcinoma affects which gender?

A

top 10 cancers

amongst male

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20
Q

Most common renal cell carcinome is which type?

A

clear cell type = most common

• Clear cell (70-80%)

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21
Q

Name some non-genetic predisposing conditions to Renal cell carcinoma?

A

Smoking, cadmium, asbestos

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22
Q

Name some genetic predisposing conditions for renal cell carcinoma?

A
  • Von Hippel-Lindau syndrome
  • Hereditary papillary renal cancer syndrome
  • Hereditary leiomyomatosis and renal cell carcinoma syndrome
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23
Q

Classical triad of presenting features of renal cell carcinoma?

A

Classical triad: hematuria, loin pain, mass

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24
Q

Paraneoplastic syndrome of renal cell carcinoma?

A
  • Fever of unknown origin

* Polycythemia

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25
What is the likelihood of metastasis of renal cell carcinoma?
metastasis before symptoms appear 25% of new cases > likely
26
What are 3 ways that renal cell carcinoma can spread?
Locally to adjacent organ Blood borne via renal vein Lymphatic to regional lymph nodes
27
Common sites for renal cell carcinoma metastasis?
Lungs and Bones
28
Prognosis of renal cell carcinoma?
* Average 5 year survival: Around 70% | * Around 95% if no distant metastasis
29
5% of cases of Renal cell carcinoma, clear cell type show what change?
sarcomatoid change Cell differentiate into mesenchymal and no longer show epithelial architecture
30
What mutation causes Renal cell carcinoma, clear cell type?
* 95% are sporadic | * Associated with 3p DELETION
31
Apart from 95% cases where VHL gene mutation causes renal cell carcinoma (clear-cell), what causes the remaining 5% cases?
Von Hippel Lindau syndrome Autosomal dominant > Defective VHL on 3p (Not DELETION as seen in sporadic)
32
What are some clinical features of Von Hippel Lindau syndrome?
* Cerebellar hemangioblastoma * Cysts in kidney, liver, pancreas * Renal cell carcinoma
33
Histology of renal cell carcinoma, clear cell type?
Mostly clear cells, some have eosinophilic or granular cytoplasm Delicate branching capillary network surrounding tumour cell
34
Incidence rate of papillary renal cell carcinoma?
10-15% of all renal cell carcinoma
35
Origin of Papillary renal cell carcinoma?
distal convoluted tubule
36
Most cases of Papillary renal cell carcinoma is caused by?
sporadic, Cytogenetic changes • Trisomy 7, 17, loss of Y
37
What is the syndrome that is associated with papillary renal cell carcinoma?
Hereditary papillary renal cancer syndrome * Autosomal dominant * Multiple bilateral tumours
38
What is the histology of papillary renal cell carcinoma?
* Cuboidal or low columnar cells arranged in papillary formation * Foam cells (macrophages) common in papillary cores * Highly vascular stroma
39
Origin of Chromophobe Renal Cell Carcinoma?
arising from intercalated cells of collecting ducts | • Same as benign oncocytoma
40
What genetic changes cause Chromophobe Renal Cell Carcinoma?
Monosomies of different chromosomes
41
Compare prognosis of chromophobe renal cell carcinoma with other maligant cancers?
5% of all renal cell carcinoma Better prognosis than renal cell carcinoma, clear cell type and papillary renal cell carcinoma
42
Histology of Chromophobe Renal Cell Carcinoma?
• Cells with prominent cell membranes and pale eosinophilic (pinkish) cytoplasm • Halo around nucleus
43
Origin of Collecting duct carcinoma?
Arise from collecting duct cells in medulla
44
Prognosis of collecting duct carcinoma?
Aggressive carcinoma with unfavourable outcome * Early metastasis * Around 50% patients die within 2 years
45
Compare the origin of Collecting duct carcinoma with other malignant kidney cancers?
Most are from renal cortex Collecting duct carcinoma is from medulla
46
Origin of Urothelial (Transitional cell) carcinoma of the | renal pelvis?
urothelium (transitional cell) of renal pelvis
47
What is the range of Urothelial carcinoma of the renal pelvis?
Range from benign papilloma to invasive urothelial (transitional cell) carcinoma
48
What are presentations of urothelial carcinoma of renal pelvis?
- Usually small tumour upon presentation - Haematuria - Block urinary outflow -> flank pain and hydronephrosis
49
What is the progression of urothelial carcinoma of renal pelvis?
multiple involving renal pelvis, ureters and urinary bladder (All lined by urothelium) Infiltration of wall of pelvis and calyces is common
50
What is the • Most common primary renal tumour of CHILDHOOD?
Nephroblastoma (Wilms Tumour) 20% of malignant childhood tumours
51
What is the incidence of Wilm tumour according to age?
95% of tumour before age of 10 Highest incidence between 2 and 5 years old
52
What are the chances of bilateral Wilms tumour and what are the sequences of progression?
5-10% have bilateral kidney tumours Progression: • Synchronous (simultaneously) • Metachronous (one after the other)
53
What mutation causes Wilms tumour?
Deletions or mutations of WT-1 or WT-2 genes | both on short arm of chromosome 11
54
What is the clinical presentation of Wilm tumour?
* Abdominal mass * Haematuria * Pain
55
What is the mode of spread of Wilms tumour?
* Local to adjacent organs, usually late * Blood borne to lungs and liver * Lymphatic to regional lymph nodes
56
Treatment of Wilms tumour?
Surgical resection +/- adjuvant chemotherapy and radiotherapy
57
3 components to the histology of WIlms tumour?
Triphasic combination (3 components) • Undifferentiated round cells (Blastemal) • Undifferentiated spindle cells (Stromal) • Columnar epithelial cells (Epithelial)
58
Most common Tumors of the low urinary tract? Less common?
Urinary Bladder tumors >> Urothelial tumours are the most common Renal pelvis, and ureter are much less common
59
What are the 3 layers that make up the urinary bladder wall?
Urothelium Lamina propria Muscularis propria + layers of Detrussor muscle
60
What cells cover the urothelium normally?
Umbrella cells
61
Given the vast majority of urinary bladder tumours are urothelial tumours, what are some malignant tumours?
* Squamous cell carcinoma | * Adenocarcinoma
62
Age, gender affected by urinary bladder tumours? | Presentation?
* Middle aged and elderly * Men to female (3:1) • Painless gross hematuria
63
What are some risk factors to urothelial carcinoma?
Occupational history: e.g. dyes and rubber Smoking, renal stones, food and some herbal meds, Analgesic nephropathy
64
What herbal med is a risk factor for urothelial carcinoma?
Aristolochic acid
65
What are the 2 types of urinary papilloma benign tumours?
Benign • Urothelial papilloma • Inverted papilloma
66
What are the 4 types of malignant urinary bladder tumours?
- Pailloma-papillary carcinoma - Invasive papillary carcinoma - Flat non-invasive carcinoma - Flat invasive carcinoma
67
Benign bladder papilloma represent what proportion of bladder tumours?
1%
68
Histology of papilloma in urinary bladder?
Benign: • Finger like papillae with central fibrovascular core • Lined by normal urothelium (transitional cell) (umbrella cells)
69
What are the 3 types of Papillary urothelial tumours (Non-invasive)?
1. Papillary urothelial neoplasm of low malignant potential 2. Papillary urothelial carcinoma, low grade 3. Papillary urothelial carcinoma, high grade
70
Compare papillary urothelial tumours (non-invasive) with benign papilloma?
* Usually larger than papilloma | * Thicker layer of urothelium (abnormal)
71
What are the differences between high and low grade urothelial carcinoma?
High grade: - Hyper-cellularity - Nuclear crowding - Loss of cellular polarity - Loss of base to surface differentiation - Nuclear pleomorphism - High mitotic rate
72
Route of spread of papillary urothelial carcinoma?
Capable of spreading within the urinary system by implantation >> give multiple foci of cancerous cells
73
Small minority of low grade papillary urothelial tumours are invasive. True or false?
True
74
Small minority of High grade papillary urothelial tumours are invasive. True or false?
False Around 80% papillary urothelial carcinoma, high grade are invasive
75
Which malignant urinary papillary carcinoma can slowly progress to become invasive?
High grade, non-invasive can progress to invasive easily
76
Another name for Flat noninvasive carcinoma?
urothelial carcinoma in situ
77
Grade of urothelial carcinoma in situ***?
By default high grade There is no low grade carcinoma in situ
78
Histology of Flat noninvasive carcinoma?
Cytological malignant cells within flat urothelium Large, irregular overlapping nuclei No papillary frond
79
Chances of metastasis of flat noninvasive carcinoma?
* Commonly multifocal | * 50-75% progress to invasive cancer if untreated
80
Difference in metastasis and prognosis between non-invasive and invasive malignant bladder tumours?
* Non-invasive carcinoma do not metastasize by blood or by lymphatics and has a good prognosis * Invasive carcinoma DO metastasize by blood and lymphatics to other organs and has a poor prognosis
81
Non-invasive low grade papillary urothelial carcinoma surival rate?
95% | 10 year survival rate
82
Invasive urothelial carcinoma invades into what structure? Treatment and prognosis?
Invading into muscularis propria of bladder wall (through urothelium) 70% survival rate even after radical cystectomy (surgical removal of urinary bladder)
83
What determines the staging of invasive urothelial carcinoma?
depth of invasion: • Lamina propria invasion (T1) • Muscularis propria (detrusor muscle) invasion (T2, T3)
84
What is the tumour grading of invasive urothelial carcinoma?
Low or High (unlike urothelial carcinoma in situ which is by default high grade) Both papillary and flat type urothelial carcinoma can be invasive
85
Treatment of Invasive urothelial carcinoma with muscularis propria (T2) invasion?
Radical cystectomy (Surgical removal of urinary bladder)
86
Treatment of Non-invasive carcinoma, high grade or invasive urothelial carcinoma with lamina propria invasion (T1) ?
* Transurethral resection | * Intravesicle instillation of BCG (shown to decrease recurrence)
87
Treatment of Non-invasive papillary neoplasm/carcinoma, low grade?
Transurethral resection
88
Sensitivity of urine cytology is low or high in cases of low grade urothelial carcinoma?
Diagnostic sensitivity is low
89
Use of urine cytology?
screening of urinary tumor follow up of patients after tumor resection by transurethral resection
90
What does the Bladder cancer kit do?
detect aneuploidy for chromosomes 3, | 7, 17, and loss of the 9p21 locus via fluorescence in situ hybridization
91
When is Bladder cancer kit used?
persons with hematuria suspected of | having bladder cancer