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Clear-cell renal-cell carcinoma

From Wikipedia, the free encyclopedia

Clear-cell renal-cell carcinoma (CCRCC) is a type of renal-cell carcinoma.

Genetics

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Cytogenetics

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Molecular genetics

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Several frequently mutated genes were discovered in CCRCC: VHL, KDM6A/UTX, SETD2, KDM5C/JARID1C and MLL2. PBRM1 is also commonly mutated in CCRCC.[citation needed]

Histogenesis

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CCRCC is derived from the proximal convoluted tubule.[citation needed]

Microscopy

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Generally, the cells have a clear cytoplasm, are surrounded by a distinct cell membrane and contain round and uniform nuclei.[citation needed]

Microscopically, CCRCCs are graded by the ISUP/WHO as follows:[1][2]

  • Grade 1: Inconspicuous and basophilic nucleoli at magnification of 400 times
  • Grade 2: Clearly visible and eosinophilic nucleoli at magnification of 400 times
  • Grade 3: Clearly visible nucleoli at magnification of 100 times
  • Grade 4: Extreme pleomorphism or rhabdoid and/or sarcomatoid morphology

Epidemiology

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  • CCRCC most commonly affects male patients in their sixties and seventies.
  • Majority of cases arise sporadically.
  • Only 2–4% of the cases presenting as part of an inherited cancer syndrome, such as von Hippel–Lindau disease.

Images

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References

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  1. ^ YiFen Zhang. "What is the ISUP/WHO grading system for renal cell carcinoma (RCC)?". Medscape. Updated: Jul 02, 2019
  2. ^ Moch, H. (2016). "WHO-ISUP-Graduierungssystem für Nierenkarzinome". Der Pathologe. 37 (4): 355–360. doi:10.1007/s00292-016-0171-y. ISSN 0172-8113.