Kidney Tumors Flashcards

1
Q

Classification of Primary Tumors

A

A. Benign

  1. Papillary Adenoma (<5mm)
  2. Oncocytoma
  3. Angiomyolipoma

B. Malignant

  1. Renal cell carcinoma
  2. Wilms tumor (children)
  3. Urothelial carcinoma (pelvis)

C. Others

  1. Lymphoma
  2. METASTATIC -RARE
    • Blood borne from lungs breast and stomach
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2
Q

Renal Papillary Adenoma

Origin

A
  • Renal tubules; within cortex
  • 7-22% found at autopsy
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3
Q

Renal Papillary Adenoma

Gross appearance

A
  • Small encapsulated tumor
  • Within cortex as pale yellow discrete nodules
  • <2cm→benign
  • >2cm→malignant
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4
Q

Renal Papillary Adenoma

Histopathology

A
  • Branching papillomatous glands
  • Resembles low grade papillary renal carcinoma therefore all papillary tumors are seen as malignant until proven otherwise
  • Share immunohistochemical and cytogenic features with papillary carcinomas →trisomies 7 and 17
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5
Q

Oncocytoma

Type and origin

A

Type: Benign epithelial tumor

Origin: From intercalated cells of the collecting ducts

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6
Q

Oncocytoma

Histo

A
  • Lots of mitochondria
    • abundant and distorted
    • tan color
    • granular eosinophilic cytoplasm
  • Eosinophilic cells
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7
Q

Oncocytoma

Gross

A
  • Well encapsulated
  • STELLATE SCAR
  • May reach 12 cm in size

NOTE: 5% of ressected tumors

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8
Q

Angiomyolipoma

Type

A
  • Benign
    • Angio→blood vessels
    • Myo→smooth muscle
    • Lipoma→adipose tissue
  • 25-50% of pt with tuberous sclerosis (CM3)
  • Radiological diagnosis
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9
Q

Malignant tumors of kidney

Classification

A

A. Cortex

  • Epithelial
    • Renal cell carcinoma
  • Mesenchymal
    • Sarcoma
    • Lymphoma
    • others
  • Blastema
    • Wilms tumor/nephroblastoma (Peds)

B. Renal pelvis

  • Urothelial carcinoma
  • Squamous cell carcinoma
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10
Q

Renal Cell Carcinoma

Hypernephroma, clear cell carcinoma, adenocarcinoma

Epidemiology

A
  • 1-3% of visceral cancers
  • 85% of adult renal cancers
  • 60-70
  • Male to female 3:1
  • Mostly sporadic
  • AD in younger pts
  • Familial variant in 4%
  • Mostly sporadic with 5% inherited
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11
Q

Renal Cell Carcinoma

Origin

A

Tubular epithelium

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12
Q

Renal Cell Carcinoma

Gross morphology

A

Yellow color

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13
Q

Renal Cell Carcinoma

Risk Factors

A
  • 30X increase in pt with aquired cystic disease in CRF
  • Familial syndromes
  • Tobacco
  • Obesity
  • HTN
  • Unopposed estrogen therapy
  • Exposure to asbestos
  • Petroleum
  • Heavy metals
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14
Q

Heridetary RCC

Types

A

A. Familial Clear Cell Carcinoma

  • 50% risk

B. Von Hippel Lindau VHL disease

  • AD cancer syndrome
  • Cerebellar haemangioblastomas
  • Retinal angiomas
  • Phaeochromocytoma
  • Cysts in various organs

C. Hereditary Papillary RCC

  • Multiple bilateral papillary tumors
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15
Q

RCC

Classical Triad

A

<10%

  • Hematuria (occurs in 50% ie most common symptom)
  • Flank pain
  • Mass
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16
Q

RCC

Metastasis

A

Hematogenous

​Local ie abdominal

17
Q

RCC

Paraneoplastic Syndromes

A
  • PTH, PG→Hypercalcemia
  • Renin→HTN
  • GC→CUSHING
  • Gonadotropins→Feminisation or Masculinisation
  • EPO→Polycythemia

NOTE: 5 year survival→40%

18
Q

RCC

Histological classification

A
  1. Clear cell type
    • 77%
    • Clear cytoplasm with lipid and glycogen
  2. Papillary RCC
    • 15%
    • Papillary structures
  3. Chromophobe cell type
    • 5%
    • Solid growth
    • Perinuclear halo
    • Granular cytoplasm
19
Q

Clear Cell Carcinoma

Epidemiology

A
  • Most common type (70-80% or renal cell cancers)
  • Mostly sporadic→few familial
  • Loss of chromosomal segment 3p14 to 3p26
  • Associated with Hippel-Lindau VHL disease
20
Q

Papillary Carcinoma

characteristics

A
  • 10-15% of RCC
  • Multifocal, bilateral
  • Sporadic →​ trisomies of ch7,16,17
  • Familial→Trisomy of ch7
21
Q

Papillary Renal Cell Carcinoma

Types

A

Morphological overlap but distinct molecular pathway and clinical picture

  1. Type 1
    • Papillae lined by single layer of cells
    • Scanty basophilic cytoplams
    • Low nuclear grade
    • Trisomies 7,17
    • MET mutation
    • better prognosis
  2. Type 2
    • ​​Lined by pseudostratified layers
    • Abundant eosinophilic cytoplasm
    • Higher nuclear grade
    • Multiple genetic changes
    • WORSE prognosis
22
Q

Chromophobe RCC

characteristics

A
  • 5%
  • Arise form Cortical collecting duct or intercalated cells
  • Loss of entire chromosomes: 1,2,6,10,13,17,21
  • HYPOPLOIDY
  • Good prognosis
23
Q

Renal Cell Carcinoma

Staging

A

Stage 1: <7cm; 5 yr survival more than 95%

Stage 2: > 7cm; 88%?

Stage 3: Gerota’s fasia or 1 regional lymphnode involved; 5 yr survival 59%

Stage 4: Beyond gerota’s fascia or > 1 lymph node involved; 5 yr survival 20%

24
Q

Urothelial Carcinoma of Renal Pelvis

Etiology

A

5-10% of primary renal cancers

  • Analgesic abuse
  • dye
  • rubber
25
Q

Urothelial Carcinoma

Clinical presentation

A
  • Hematuria within a short time
  • Small when discovered→not plapable
  • May block urine flow
  • May affect pelvis, urter and bladder
  • Poor prognosis
26
Q

Urothelial Carcinoma of renal pelvis

Grossly

A

May infiltrate wall of pelvis

Papillary urothellial carcinoma enter to urterer

27
Q

Wilms Tumor/Nephroblastoma

Epidemiology

A
  • Childhood malignant neoplasm
  • Commonest solid abdominal tumor in children 2 to 5 yrs
  • Prevalence 1 in 10 thouand
  • Sporadic and unilateral in 90%
  • Familial and bilateral in 20%
28
Q

Wilms Tumor

Triphasic Pattern

A

Embryonic microscopic nephrogenic elements that are a mixture of:

  • Blastema
  • Epithelial structures- immature glomeruli or abortive tubules
  • Stroma

Nephrogenic rests

29
Q

Wilms Tumor

Clinical Presentation

A
  • Palpable abdominal mass
  • Fever
  • Abdominal pain
  • Hematuria
30
Q

Wilms tumor

Morphology

A
  • Large
  • Solitary
  • Lobulated
  • Well circumscribed
  • 10% bilateral or multicentric
31
Q

Syndromes associated with increased risk and often bilateral Wilms tumor

A
  1. WAGR SYNDROME
    • Wilms (30% develop it) Wilms Tumor-1 associated gene on ch11p13
    • Aniridia
    • Genital anomalies
    • Retardation (mental)
32
Q

Syndromes associated with increased risk and often bilateral Wilms tumor

A
  1. Denys Drash Syndrome
  • Gonadal Dysgenesis (male pseudohemaphrodite)
  • Nehropathy (RF)
  • WT-1 on Ch 11p13
33
Q

Syndromes associated with increased risk and often bilateral Wilms tumor

A
  1. Beckwith Weidemann Syndrome
  • Enlarged body organs
  • Hemihypertrophy
  • Renal Medullary Cysts
  • Adrenal cytomegaly

WT-2 gene on Ch 11p15.5

34
Q

Wilms Tumor

Course

A
  • Initially spreads locally thru renal capsule
  • Propensity to extend into renal vein and IVC
  • Metastasizes prefretially to regional LN and Lung
  • Unusual to involve bone marrow
35
Q

Metastatic tumors to the kidney

A
  • Uncommon
  • From
    • Lung
    • Breats
    • Stomach
36
Q

Diagnostic tests

A
  • CBC
  • Renal FT
  • Tumor markers
    • EPO
    • PTH
  • Urine
    • Rouine microscopic
    • Cytologic exam
  • Radio
    • US
    • CT
    • others
  • Uretroscopy, cytoscopy
  • Biopsy
    • FNA
  • Core needle biopsy
37
Q

Staging, screening tests

A

STAGING

  • LFT
  • CXR
  • BONE SCAN
  • imaging

Screening

  • US every 6-8 months in children at high risk