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Renal Medullary Carcinoma

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  • Highly aggressive renal neoplasm that has association with sickle cell trait or rarely hemoglobin SC disease (unknown mechanism).
  • Some considered this be a variant of collecting duct carcinoma.
  • Usually encountered in young patients, male > female (2:1), mostly African-American and with sickle cell hemoglobinopathies.
  • Gross: centered in medullary region of the kidney with white or gray cut surfaces and central necrosis is common (image A).

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  • Histology:
    • Most common pattern is that of a reticular or cribriform pattern (image B) and (image C).
    • High-grade cell cytology.
    • Presence of desmoplastic stroma.
    • May see sickling of RBC cells (image D).
  • Immunohistochemistry: loss of nuclear expression of INI1.
  • Very aggressive, mean survival less than 6 months.
  • DDX: collecting duct carcinoma: No hemoglobinopathes; INI1 +.