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REGUB > 19. Renal And Bladder Tumours > Flashcards

19. Renal And Bladder Tumours Flashcards

1
Q

List the classification of renal tumours.

Benign?
(3)

Malignant?
(4)

Other?
(2)

A

BENIGN
1. Adenoma

  1. Angioma
  2. Angiomyolipoma

MALIGNANT

  1. Wilms Tumor (nephroblastoma in children)
  2. Grawitz tumor (adenocarcinoma, hypernephroma)
  3. Transitional Cell Carcinoma: renal pelvis and collecting system
  4. Squamous carcinoma of the renal pelvis

OTHERS

  1. Oncotyoma
  2. Angiomyolipoma: a/w tuberous sclerosis, contain fat and can bleed
    - It’s a Hartmann’s, with main complication being hemorrhage
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2
Q

What is the epidemiology of renal tumours?

What is the aetiology and risk factors?
(5)

What is the pathology?
(4)

A

EPIDEMIOLOGY
- Incidence has increased x2-4% over last 3 decades
—> Increased incidental detection
—> Unidentified environmental factors
- Steady rise in mortality over last 3 decades
- Slow stage migration over last 3 decades
—> Localised disease (53%)
—> Regionally advanced (20%)
—> Metastatic disease (22%)

AETIOLOGY AND RISK FACTORS
1. Unknown
2. Tobacco (increases relative risk x1.4-2.5)
3. 10-20% higher incidence in African Americans
4. Acquired renal cystic disease: 1/3 of patients develop ARCD after 3 years of dialysis
—> Risk of developing RCC is 3-6 times greater in ARCD
5. Nutrition: Asian migrants to western countries are at increased risk

PATHOLOGY

  1. Adenocarcinoma
  2. Originates in proximal tubules
  3. Tumour cells are large with clear cytoplasm (accumulation of glycogen and lipid) hence name clear cell carcinoma
  4. Multifocal in 7-20%
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3
Q

What is sporadic vs hereditary forms?
(2)

What is Von Hippel-Lindau?

A
  • 96%: sporadic
  • 4%: familial syndromes (all AD)
    o Von Hippel-Lindau (VHL): 3p25
    o Hereditary Paillary RCC: 7q34
    o Birt-Hogg-Dubé: 17p11.2
    o Hereditary Leiomyoma RCC: 1q42

VON HIPPEL-LINDAU

  • AD syndrome, present in 3rd, 4th or 5th decade
  • 50% will develop RCC, often bilateral and multifocal
  • Phaeochromocytomas, renal and pancreatic cysts, cerebellar haemangioblastomas, retinal angiomas, epididymal cystadenomas
  • Loss of both copies of VHL (tumour suppressor)-effects on gene transcription (HIF-1)-upregulation of VEGF
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4
Q

How do renal tumours spread metastatically?

4

A
  1. Invasion of Surrounding Structures
    a. Direct extension to the adrenal gland (7.5% in tumours >5cm)
    b. Through renal capsule
  2. Vascular invasion is associated with thrombosis
    a. Renal Vein (5% at presentation)
    b. IVC (Sub/Supra-Diaphragmatic)
    c. Right atrium
  3. Distance metastases: lymphatics = hilar and para-aortic nodes
  4. Haematogenous: pulmonary (75%) - cannonball metastases
    a. Bone 20%, liver 18%, adrenal
    b. Cerebral / CNS 8%
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5
Q

How do you stage renal tumours?

What is the histological classification?
(5)

What is the prognosis of 5y survival?
(3)

A 
TNM
-	Most important prognostic factor 
1. T1: confined to kidney ≤7cm
o	T1a: ≤4cm
o	T1b: 4cm and ≤7cm
  1. T2: confined to kidney >7cm
  2. T3: locally advanced within Gerota’s fascia
    o T3a: adrenal and/or perinephric fat
    o T3b: renal vein or IVC below diaphragm
    o T3c: IVC above diaphragm
  3. T4: invades beyond Gerota’s fascia
  • N0: no regional lymph node involvement
  • N1: mets in single node
  • N2: mets in more than one node
  • M0: no distant mets
  • M1: distant mets

HISTOLOGICAL CLASSIFICATION

  1. Conventional (clear cell): 70-80%
  2. Papillary: 10-15%, 40% multifocal, bilateral
  3. Chromophobe: 5%
  4. Collecting Duct (Bellini): rare<1%, young pts, poor prognosis

PROGNOSIS (5y Surgical

  1. Confined to kidney: 79%
  2. Nodes or IVC involved: 40%
  3. Distant Mets: 8%
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6
Q

What are the clinical features of renal tumours?

What is paraneoplastic syndrome?

What investigations do you perform?
(4)

A

CP
- Incidental Finding on Imaging: >50%
- Symptomatic
- Classic Triad: pain, mass, haematuria  <10%
—> Haematuria: 50%
—> Mass: 30%
—> Metastatic Disease: 25%
- CP: bone pain, haemoptysis, oedema, weight loss, fatigue, night sweats
- Non-reducing, acute varicocoele, lower limb oedema
- Paraneoplastic syndrome: 10-40%

PARANEOPLASTIC SYNDROME
1. Polycythaemia: excess erythropoietin
2. Iron deficiency anaemia
3. Hypertension: excess renin production, renal artery compression
4. Hypercalcaemia: due to production of parathyroid-like hormone (10-20%)
5. Pyrexia of unknown origin
6. Elevated ESR
7. Cushing’s (ectopic secretion of ACTH)
Stauffer’s syndrome (Hepatic dysfunction, fever, anorexia)

INVESTIGATIONS

  1. Labs: FBC, ESR, U&E, LFT’S (calcium, erythropoietin, renin)
  2. Initial Imaging: US, CT (triphasic, enhancement>15-20 Hounsfield units)
  3. Specialist Imaging for Local Disease: CT, MRI (venous disease, pre and post gadolinium), ANGIO
  4. Imaging for Metastatic Disease: CXR, Bone Scan, CT Brain
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7
Q

What is the management of renal tumours?

6

A
  1. Radical Nephrectomy: open or laparoscopic or robot-assisted laparoscopic
  2. Nephron-sparing surgery
  3. Vascular Extension
    a. Renal Vein Thrombus
    b. IVC Thrombus below the Diaphragm
    c. IVC Thrombus above the Diaphragm (including atrium)
    - Radiotherapy is given post operatively in cases of spread into the perinephric fat, para-aortic nodes or the IVC
  4. Thermal ablation techniques (<3cm): renal cryosurgery, radiofrequency ablation
  5. Active surveillance
    a. Natural history of small renal lesions better defined
    b. Useful for elderly patients with comorbidities
    c. 30% of small masses show no growth over 3 years, risk of mets low (1%)
  6. Advanced Disease: cytoreductive nephrectomy
    a. Isolated pulmonary or cerebral metastases are occasionally excised
    b. RCC responds poorly to chemotherapy (2-6% response to multimodal therapies)
    c. Radiotherapy —> bony metastases
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8
Q

What immunotherapy is used for metastatic disease?

3

A
  1. Interferon a: response rates: 12% // 1.8% complete response rate, median overall survival 5-15 months
  2. Interleukin 2: response rates: 15%, complete responses in 6-8%, median overall survival 12-18 months
  3. Combination therapy
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9
Q

What is the incidence of bladder cancer?

What are the risk factors?
(5)

What are high risk occupations?
(12)

A

INCIDENCE

  • 2nd most common urological malignancy
  • 3% of all cancer deaths
  • UK incidence 13,000/year, 4973 deaths
  • Average age 8th decade and only 1% are less than 40 years
  • 2.5:1 (M:F) ratio

RISK FACTORS

  1. Industrial carcinogens: aromatic amines, aniline dyes
  2. Pharmaceutical compounds: Saccharin, Phenacetin, Insecticides)
  3. Cigarettes: 2-5 fold increase (30-50% caused by smoking)
  4. Schistosomiasis: squamous cell carcinoma
  5. Cyclophosphamide

HIGH RISK OCCUPATIONS

  1. Textile workers
  2. Dye workers
  3. Tyre rubber workers
  4. Petrol workers
  5. Leather workers
  6. Shoe manufacturers
  7. Painters
  8. Hairdressers
  9. Lorry drivers
  10. Chemical workers
  11. Rodent exterminators
  12. Sewage workers
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10
Q

What is the pathology of bladder cancer?

4

A
  • Transitional Cell Carcinoma: >90%
    o Carcinoma in Situ: 10%
    o Non-muscle invasive (superficial): 70%
    o Muscle invasive: 20%
  • Squamous Cell Carcinoma: 1-7%
  • Adenocarcinoma: 2%
  • Sarcomas: 1%
  • 70% of TCC is papillary, usually G1 or G2
    o Papillary TCC is usually superficial  confined to the mucosa (Ta) or the submucosa (lamina propria (T1))
    o T1G3 can be more aggressive, 40% subsequently upstaged to muscle invasive
  • 10% have mixed papillary and solid morphology, 10% are solid
  • Usually these are G3, and half are muscle invasive at presentation
  • 10% is CIS  40-83% of CIS will progress to muscle-invasive TCC if untreated
    o CIS is therefore most aggressive form of superficial TCC
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11
Q

What are the clinical features of bladder cancer?
(5)

Where does it spread metastatically?
(3)

What investigations do you perform?
(5)

A

CP

    1. Gross Total Painless Haematuria: 85%
      1. Microscopic Haematuria —> 20%
      2. Irritative Voiding Symptoms —> 25% (think CIS)
      3. Clot Retention —> 17%
      4. Metastatic Disease
  • Physical Exam generally normal (unless there is a mass palpable on rectal examination or fixation of pelvic structures)

METASTATIC SPREAD

  1. Invasion of Surrounding Structures: peri-vesical fat and contiguous organs and pelvic side wall
  2. Lymphatic: pelvic Nodes, para-aortic
  3. Vascular: lungs, bones, liver, brain
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12
Q

What are the prognostic features of bladder cancer?

What is the prognosis based on 5y survival?
(3)

How do you stage it?
(3)

What is the management?
(3)

A

PROGNOSTIC FEATURES

  • Multiple initial tumours twice as likely to recur
  • Tumours greater than 3cm —> more than 60% recurrence
  • Solid broad-based tumours likely to recur
  • High Grade Disease (grade III)
  • Submucosal Invasion
  • Vascular Invasion (70% metastasis rate)
  • C.I.S: 82% recur, 73% progress to invasion

5Y SURVIVAL

  1. Confined: 50%
  2. Nodes or local spread: 15%
  3. Distant Mets: 0%

STAGING

  1. Superficial non-muscle invasive (70%)
    a. Excellent prognosis with prompt treatment
    b. 10-15% of high grade tumours progress to invasion
  2. Muscle invasive (20%)
    a. Subject to local invasion and metastasis
    b. 50% have occult metastasis
  3. Flat non-invasive carcinoma in situ (10%)

MANAGEMENT

  1. Primary Lesion:
    a. TURBT: removes lesion, provides histology
    b. Surveillance: for superficial, low grade
  2. Invasive or High Grade Disease:
    a. Radical Cystoprostatectomy and urinary diversion
    - Ileal conduit
    - Ileal neobladder
    b. Neoadjuvant Chemotherapy
    c. Radiation therapy
  3. Metastatic Disease:
    a. Radiation therapy
    b. Palliative Care
    c. Radiotherapy
    - For primary tumour
    i- For bony metastases
    d. Chemotherapy
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13
Q

What is carcinoma in situ of bladder? —> “frank malignancy rather than precancerous condition”

What is the clinical presentation?
(3)

What are the investigations used?
(4)

What is the natural history?
(2)

How is it management?
(2)

A

CP

  1. Asymptomatic (25%)
  2. Have irritative voiding symptoms (75%)
  3. Microscopic haematuria (75%)

INVESTIGATIONS

  1. Cytology: 90-100% positive
  2. Cytoscopy: no consistent or specific features, bladder appears normal, sometimes patchy, velvety lesion
  3. Biospy
  4. CT Scan: to see beyond mucosa

NATURAL HX

  1. Diffuse CIS: majority of patients, and most will become muscle invasive
  2. Localized CIS: only minority will become muscle invasive

MANAGEMENT

  1. Intra-vesical Chemotherapy with BCG
    - Minor reactions common
    - Fever occurs in 3% requiring anti-TB drugs
    - Granulomatous prostatitis occurs in 0.9%
    - BCG SEPSIS: intravascular absorption of a lethal dose, 20% mortality, treat with steroids & antiTB medications
  2. Radical Cysto-prostatectomy
    - For failure of intravesical chemotherapy
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14
Q

What are other bladder tumours? Describe.

2

A
  1. Squamous cell carcinoma
    - 5-7% of bladder tumours
    - Develop without prior history
    - Rapidly growing aggressive tumours
    - Present as solitary, invasive and high stage
    - Predisposing Factors: bladder diverticulae, chronic indwelling catheters, Schistosomiasis (55-70%), bladder stones, chronic UTI
  2. Adenomacarcinoma
    - 2% of all bladder cancers
    - 6th -7th decade, 2X more frequent in males
    - Typically solitary - trigonal or urachal
    - Commonest tumour in Exstrophy
    - Signet cell type infiltrative diffusely
    - Make sure its not colorectal primary
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