pathology station-Clear Cell Renal Cell Carcinoma

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Describtion

Kidney tumor
Adult renal cell carcinoma
Clear cell renal cell carcinoma
Definition / general

  • Morphologically heterogeneous group of renal carcinomas with clear or eosinophilic cytoplasm, characteristic vasculature and common molecular signature of VHL inactivation
Essential features
  • Architecturally and cytologically diverse: solid, alveolar, acinar, cystic growth patterns of cells with clear or eosinophilic cytoplasm
  • Characteristic network of small, thin walled, "chicken wire" vasculature
  • Inactivation of VHL and upregulation of hypoxia inducible factor
 
Clinical features
  • 60% - 80% are found incidentally on imaging
  • Metastases often hematogenous via renal veins: lung, liver, bone, soft tissue, adrenal
  • Risk factors: adult polycystic disease, smoking, obesity in women, hypertension, von Hippel-Lindau (VHL) disease, tuberous sclerosis (but not via VHL mutations, 
  • Both papillary and chromophobe have a better prognosis than clear cell RCC

von Hippel-Lindau disease:
  • Autosomal dominant syndrome with hemangioblastomas of retina and cerebellum, cysts of pancreas, liver and kidney, clear cell tumors of other sites, papillary cystadenoma of epididymis, pheochromocytoma
  • Associated with bilateral or multiple renal cell carcinomas in 50%
Gross:
  • Golden yellow tumor (due to lipid content) with hemorrhages and necrosis (image A).
  • +/- cystic changes; more solid fleshy (non golden-yellow) area may represent sarcomatoid change (imageB)
  • clear_cell-figureA_Big.jpg
clear_cell-figureB_Big.jpg

  • Microscopic (histologic) description
  •  
  • Compact, alveolar, tubulocystic or rarely papillary architecture of cells with clear cytoplasm (from lipid / glycogen), distinct but delicate cell boundaries; cell size is 2x normal epithelial tubule cell
  • Characteristic network of small, thin walled, "chicken wire" vasculature
  • May have angioleiomyomatous features smooth muscle stroma
  • myospherulosis overlapping morphologic features of papillary carcinoma
  • Some tumors may have pseudopapillary architecture due to high grade changes and loss of cell cohesion
  •  
  • Grade 1: round or uniform nuclei; nucleoli not discernible or absent
  • Grade 2: slightly irregular nuclear contours; nucleoli visible at 400x
  • Grade 3: moderately to markedly irregular nuclear contours; nucleoli visible at 100
  • Grade 4: multilobular nuclei or bizarre nuclei and large and prominent nucleoli
  • fuhrman_grade-figureA_Big-(1).jpg
  •  
fuhrman_grade-figureB_Big.jpg
fuhrman_grade-figureC_Big.jpg

fuhrman_grade-figureD_Big.jpg

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