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13. Urogenital Pathology > 8. Renal neoplasms > Flashcards

8. Renal neoplasms Flashcards

1
Q

Types of renal neoplasm

A
  1. Angiomyolipoma
  2. Oncocytoma
  3. Renal Cell Carcinoma
  4. Nephroblastoma (Wilms Tumour)
  5. Urothelial (Transitional Cell) Carcinoma
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2
Q

Definition of angiomyolipoma

A

Most common benign mesenchymal tumour of the kidney

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3
Q

Epidemiology & associations with angiomyolipoma

A
  1. Belongs to the family of PEComas (tumours containing perivascular epithelioid cells) which includes:
    - Lymphangioleiomyomatosis
    - Clear cell sugar tumour of the lung, pancreas, uterus
    - Cardiac rhabdomyomas
  2. Occurs at all ages, but typically resected at age 40 or older
  3. Most commonly found in kidneys, but can also occur in:
    - Liver
    - Retroperitoneum
    - Lungs
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4
Q

Pathogenesis of angiomyolipoma

A
  1. Sporadic
  2. Familial: associated with tuberous sclerosis
    - Loss of function mutations of TSC1 or TSC2 tumour suppressor genes
    - Characterized by cerebral cortical lesions (producing epilepsy & mental retardation), skin abnormalities & unusual benign tumours (e.g. cardiac tumours)
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5
Q

Morphology of angiomyolipoma

A
  1. Grossly:
    - Unencapsulated
    - Variegated cut surface with yellow fatty areas
  2. Histologically:
    - Mixture of myoid spindle cells, epithelioid cells, adipocytes & blood vessels (often thick walled)
    - Myoid cells show immunostaining for HMB-45
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6
Q

Clinical features of angiomyolipoma

A
  1. Can be accurately diagnosed on CT scan due to its large fat content (appears radiolucent)
  2. May rupture & bleed
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7
Q

Definition of oncocytoma

A

Benign tumour arising from intercalated cells of the collecting tubule

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8
Q

Morphology of oncocytoma

A
  1. Grossly:
    - Tan, mahogany brown mass
    - Well-encapsulated, can reach up to 12cm
  2. Histologically:
    - Tumour cells large, round, eosinophilic
    - Benign looking nuclei with large nucleoli
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9
Q

Definition of renal cell carcinoma

A

Malignant tumour arising from the renal tubular epithelium

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10
Q

Epidemiology of renal cell carcinoma

A
  1. Accounts for 85% of malignant renal tumours in adults
  2. Mostly occurs in the 6th & 7th decades of life
  3. Male : female = 2 : 1
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11
Q

Types of renal cell carcinoma

A
  1. Clear cell carcinoma (70-80%)
  2. Papillary carcinoma (10-15%)
  3. Chromophobe carcinoma (5%)
  4. Collecting duct carcinoma (<1%)
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12
Q

Clear cell carcinoma (70-80%)

A

Arises from the proximal tubules

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13
Q

Pathogenesis of clear cell carcinoma

A
  1. Sporadic
  2. Familial: von Hippel Lindau syndrome
    - loss of sequences on short arm of chromosome 3 → leading to mutations of the VHL gene (a tumour suppressor gene that encodes part of a ubiquitin ligase complex vital for the degradation of hypoxia-inducible factor 1)
    - associated with pheochromocytoma, hemangioblastomas of cerebellum & retina & cysts involving the liver, kidneys & pancreas
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14
Q

Morphology of renal cell carcinoma (clear cell type)

A
  1. [Grossly]
    - Solitary, unilateral mass
    - Circumscribed appearance
    - Yellowish cut surface with foci of necrosis & hemorrhage
    - Invasion of renal vein (can grow as a continuous tumour cord into right heart)
    - Tendency to metastasize widely even before any local symptoms
  2. [Histologically]
    - Polygonal cells with clear cytoplasm arranged in tubular or trabecular architecture
    - Delicate branching vasculature
    - Invasion of renal vein or its branches may be seen
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15
Q

Papillary carcinoma

A

Arises from the distal tubules

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16
Q

Pathogenesis of papillary carcinoma

A

MET oncogene

17
Q

Morphology of papillary carcinoma

A
  1. Can be multifocal & bilateral
  2. Cystic & hemorrhagic
  3. Cuboidal/columnar cells lining papillae
  4. Foam cells, psammoma bodies in papillary core
18
Q

Chromophobe carcinoma

A

Arises from the intercalated cells of collecting tubule

19
Q

Prognosis of chromophobe carcinoma

A

Excellent

20
Q

Morphology of chromophobe carcinoma

A
  1. Pale eosinophilic cells with perinuclear halo
  2. Arranged in solid sheets
  3. Largest cells are concentrated around vessels
21
Q

Collecting duct carcinoma

A

Arises from the collecting ducts

22
Q

Morphology of collecting duct carcinoma

A

Irregular channels lined by highly atypical epithelium with a hobnail pattern

23
Q

Clinical features of renal cell carcinoma

A
  1. Painless hematuria
  2. Mass in flank
  3. Fever (due to necrosis)
  4. Costovertebral pain
  5. Paraneoplastic syndromes (polycythaemia, hypertension, hypercalcemia, Cushing syndrome, amyloidosis etc)
24
Q

Definition of nephroblastoma (Wilm’s tumour)

A

Paediatric renal tumour

25
Q

Epidemiology & associations with nephroblastoma (Wilm’s tumour)

A
  1. Usually diagnosed between ages 2 & 5
  2. Associated with congenital malformation syndromes:
    - WAGR syndrome
    - Denys-Drash syndrome
    - Beckwith-Wiedemann syndrome
26
Q

Morphology of nephroblastoma (Wilm’s tumour)

A
  1. Grossly:
    - Well circumscribed grayish soft mass
    - Foci of hemorrhage, cysts & necrosis
    - Begins in renal cortex & eventually replaces kidney
  2. Histologically:
    - Sheets of small blue cells (blastemal component)
    - Abortive tubular & glomeruloid structures (epithelial
    component)
    - Spindle shaped cells (stromal component)
    - Heterologous elements such as striated/smooth muscle & cartilage may also be found
27
Q

Clinical features of nephroblastoma (wilm’s tumour)

A
  1. Signs & symptoms:
    - Large abdominal mass
    i. Usually unilateral, bilateral in 10-15% of cases
    ii. May cause intestinal obstruction
    - Hematuria
    - Fever
  2. Metastases:
    - Hematogenous & lymphatic spread to lungs, liver, brain & lymph nodes
  3. Treatment:
    - Combination nephrectomy & chemotherapy
    - Generally good prognosis even for tumours which have spread beyond the kidney
28
Q

Definition of Urothelial (Transitional Cell) Carcinoma

A

Neoplasm arising from the urothelium (transitional cell epithelium) lining the renal calyces, pelvis, ureter & bladder

29
Q

Types of urothelial carcinoma

A
  1. Papillary urothelial carcinoma (non-invasive)

2 Invasive urothelial carcinoma

30
Q

Morphology of urothelial carcinoma

A
  1. Identical to urothelial carcinoma of the bladder
  2. Exophytic mass growing from the surface of the areas lined by urothelium (renal calyces & pelvis)
  3. Usually associated with urothelial carcinoma of the ureter & bladder
31
Q

Clinical features of urothelial carcinoma

A

Usually discovered early

  1. Fragmentation gives rise to noticeable hematuria
  2. Blockage of urinary outflow gives rise to hydronephrosis & flank pain

13. Urogenital Pathology (12 decks)

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