Flashcards in L83 - Urinary tumours Deck (91):
Name 3 benign kidney tumours? PAO
• Papillary adenoma
Name 6 malignant kidney tumours?
R P C C U N
• Renal cell carcinoma, clear cell type
• Papillary renal cell carcinoma
• Chromophobe renal cell carcinoma
• Collecting duct carcinoma
• Urothelial carcinoma
• Nephroblastoma (Wilms tumour) - childhood
Size and manifestation of papillary adenoma?
Small, discrete tumour
<5 mm in diameter
usually asymptomatic, little clinical significance
What cancer is similar to papillary adenoma in porphology but with much worse outcome?
renal cell carcinoma
LM appearance of papillary adenoma?
Cytologically normal (low N:C, normochromatic)
Origin of angiomyolipoma?
Originating from perivascular epithelioid cells
Diagnotic features of angiomyolipoma?
** imaging by CT demonstrating fat
What disease predisposes angiomyolipoma?
Hereditary disease – Tuberous sclerosis
• 25-50% of patients with this disease develop angiomyolipoma
Genetic nature of Tuberous sclerosis?
Autosomal dominant inheritance
Manifestations of tuberous sclerosis in kidney, liver, brain and heart?
• Angiomyolipoma in kidney, liver
• Astrocytic tumor in brain
• Rhabdomyoma in heart
Tumour size and outcome of angiomyolipoma?
Large tumours (more than 4 cm)
>> present with haemorrhage if ruptures
Tumour morphology in Angiomyolipoma?
• No capsule
• Blood vessels ensheathed by smooth muscle cells
• Lobules of adipose cells
LM appearance of angiomylolipoma?
Many clusters of clear, large cells>> adipose tissue
Usual progression of angiomyolipoma in a patient with tuberous sclerosis?
Bilateral and multiple angiomyolipomas
Origin of renal oncocytoma?
arise from intercalated cells of collecting ducts
Histology of renal oncocytoma?
• Large eosinophilic cells with small round nuclei and large nucleoli
Oncocytomas can occur in different places in the body. How many % of renal neoplasms does it account for?
LM gross appearance of oncocytoma?
Sheets of tumour cells
Renal cell carcinoma affects which gender?
top 10 cancers
Most common renal cell carcinome is which type?
clear cell type = most common
• Clear cell (70-80%)
Name some non-genetic predisposing conditions to Renal cell carcinoma?
Smoking, cadmium, asbestos
Name some genetic predisposing conditions for renal cell carcinoma?
• Von Hippel-Lindau syndrome
• Hereditary papillary renal cancer syndrome
• Hereditary leiomyomatosis and renal cell carcinoma syndrome
Classical triad of presenting features of renal cell carcinoma?
Classical triad: hematuria, loin pain, mass
Paraneoplastic syndrome of renal cell carcinoma?
• Fever of unknown origin
What is the likelihood of metastasis of renal cell carcinoma?
metastasis before symptoms appear
25% of new cases > likely
What are 3 ways that renal cell carcinoma can spread?
Locally to adjacent organ
Blood borne via renal vein
Lymphatic to regional lymph nodes
Common sites for renal cell carcinoma metastasis?
Lungs and Bones
Prognosis of renal cell carcinoma?
• Average 5 year survival: Around 70%
• Around 95% if no distant metastasis
5% of cases of Renal cell carcinoma, clear cell type show what change?
Cell differentiate into mesenchymal and no longer show epithelial architecture
What mutation causes Renal cell carcinoma, clear cell type?
• 95% are sporadic
• Associated with 3p DELETION
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)
What are some clinical features of Von Hippel Lindau syndrome?
• Cerebellar hemangioblastoma
• Cysts in kidney, liver, pancreas
• Renal cell carcinoma
Histology of renal cell carcinoma, clear cell type?
Mostly clear cells, some have eosinophilic or granular cytoplasm
Delicate branching capillary network surrounding tumour cell
Incidence rate of papillary renal cell carcinoma?
10-15% of all renal cell carcinoma
Origin of Papillary renal cell carcinoma?
distal convoluted tubule
Most cases of Papillary renal cell carcinoma is caused by?
sporadic, Cytogenetic changes
• Trisomy 7, 17, loss of Y
What is the syndrome that is associated with papillary renal cell carcinoma?
Hereditary papillary renal cancer syndrome
• Autosomal dominant
• Multiple bilateral tumours
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
Origin of Chromophobe Renal Cell Carcinoma?
arising from intercalated cells of collecting ducts
• Same as benign oncocytoma
What genetic changes cause Chromophobe Renal Cell Carcinoma?
Monosomies of different chromosomes
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
Histology of Chromophobe Renal Cell Carcinoma?
• Cells with prominent cell membranes and pale eosinophilic (pinkish)
• Halo around nucleus
Origin of Collecting duct carcinoma?
Arise from collecting duct cells in medulla
Prognosis of collecting duct carcinoma?
Aggressive carcinoma with unfavourable outcome
• Early metastasis
• Around 50% patients die within 2 years
Compare the origin of Collecting duct carcinoma with other malignant kidney cancers?
Most are from renal cortex
Collecting duct carcinoma is from medulla
Origin of Urothelial (Transitional cell) carcinoma of the
urothelium (transitional cell) of renal pelvis
What is the range of Urothelial carcinoma of the renal pelvis?
Range from benign papilloma to invasive urothelial (transitional cell) carcinoma
What are presentations of urothelial carcinoma of renal pelvis?
-Usually small tumour upon presentation
-Block urinary outflow -> flank pain and hydronephrosis
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
What is the • Most common primary renal tumour of CHILDHOOD?
Nephroblastoma (Wilms Tumour)
20% of malignant childhood tumours
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
What are the chances of bilateral Wilms tumour and what are the sequences of progression?
5-10% have bilateral kidney tumours
• Synchronous (simultaneously)
• Metachronous (one after the other)
What mutation causes Wilms tumour?
Deletions or mutations of WT-1 or WT-2 genes
(both on short arm of chromosome 11)
What is the clinical presentation of Wilm tumour?
• Abdominal mass
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
Treatment of Wilms tumour?
Surgical resection +/- adjuvant chemotherapy and radiotherapy
3 components to the histology of WIlms tumour?
Triphasic combination (3 components)
• Undifferentiated round cells (Blastemal)
• Undifferentiated spindle cells (Stromal)
• Columnar epithelial cells (Epithelial)
Most common Tumors of the low urinary tract?
Urinary Bladder tumors >> Urothelial tumours are the most common
Renal pelvis, and ureter are much less common
What are the 3 layers that make up the urinary bladder wall?
Muscularis propria + layers of Detrussor muscle
What cells cover the urothelium normally?
Given the vast majority of urinary bladder tumours are urothelial tumours, what are some malignant tumours?
• Squamous cell carcinoma
Age, gender affected by urinary bladder tumours?
• Middle aged and elderly
• Men to female (3:1)
• Painless gross hematuria
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
What herbal med is a risk factor for urothelial carcinoma?
What are the 2 types of urinary papilloma benign tumours?
• Urothelial papilloma
• Inverted papilloma
What are the 4 types of malignant urinary bladder tumours?
-Invasive papillary carcinoma
-Flat non-invasive carcinoma
-Flat invasive carcinoma
Benign bladder papilloma represent what proportion of bladder tumours?
Histology of papilloma in urinary bladder?
• Finger like papillae with central fibrovascular core
• Lined by normal urothelium (transitional cell) (umbrella cells)
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
Compare papillary urothelial tumours (non-invasive) with benign papilloma?
• Usually larger than papilloma
• Thicker layer of urothelium (abnormal)
What are the differences between high and low grade urothelial carcinoma?
- Nuclear crowding
- Loss of cellular polarity
- Loss of base to surface differentiation
- Nuclear pleomorphism
- High mitotic rate
Route of spread of papillary urothelial carcinoma?
Capable of spreading within the
urinary system by implantation
>> give multiple foci of cancerous cells
Small minority of low grade papillary urothelial tumours are invasive. True or false?
Small minority of High grade papillary urothelial tumours are invasive. True or false?
Around 80% papillary urothelial carcinoma, high grade are invasive
Which malignant urinary papillary carcinoma can slowly progress to become invasive?
High grade, non-invasive can progress to invasive easily
Another name for Flat noninvasive carcinoma?
urothelial carcinoma in situ
Grade of urothelial carcinoma in situ***?
By default high grade
There is no low grade carcinoma in situ
Histology of Flat noninvasive carcinoma?
Cytological malignant cells within flat urothelium
Large, irregular overlapping nuclei
No papillary frond
Chances of metastasis of flat noninvasive carcinoma?
• Commonly multifocal
• 50-75% progress to invasive cancer if untreated
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
Non-invasive low grade papillary urothelial carcinoma surival rate?
10 year survival rate
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)
What determines the staging of invasive urothelial carcinoma?
depth of invasion:
• Lamina propria invasion (T1)
• Muscularis propria (detrusor
muscle) invasion (T2, T3)
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
Treatment of Invasive urothelial carcinoma with muscularis propria (T2) invasion?
Radical cystectomy (Surgical removal of urinary bladder)
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)
Treatment of Non-invasive papillary neoplasm/carcinoma, low grade?
Sensitivity of urine cytology is low or high in cases of low grade urothelial carcinoma?
Diagnostic sensitivity is low
Use of urine cytology?
screening of urinary
follow up of patients after tumor resection
by transurethral resection
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