Clear cell renal cell carcinoma

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Author: Mikael Häggström [note 1]
Clear cell renal cell carcinoma (CCRCC):

Gross processing

As per: Kidney with tumor

Microscopic examination

Clear cell renal cell carcinoma.

CCRCC typically shows cells in nests or solid pattern, and a delicate branching fibrovascular network.[1]

Differential diagnosis: Further information: Kidney with tumor
If unsure, useful immunohistochemistry markers that are positive in CCRCC are vimentin, EMA, keratin, CD10, Pax2, RCC, CAIX (circumferential membranous).[1]

Grading

CCRCCs are graded by the ISUP/WHO as follows:[2][3]

  • Grade 4: Extreme pleomorphism or rhabdoid and/or sarcomatoid morphology

Staging

By AJCC 8th edition:[4][5] edit

Stage TNM Description
Tx, N0, M0 Tumor cannot be assessed
T0, N0, M0 No evidence of primary tumor
I T1, N0, M0 Tumor ≤7 cm; limited to kidney
T1a, N0, M0 Tumor ≤4 cm; limited to kidney
T1b, N0, M0 Tumor 4-≤7 cm; limited to kidney
II T2, N0, M0 Tumor >7 cm; limited to kidney
T2a, N0, M0 Tumor 7-≤10 cm; limited to kidney
T2b, N0, M0 Tumor >10 cm; limited to kidney
III T3, N0, M0 Tumor extends to major veins or perinephric tissue but not into ipsilateral adrenal gland nor beyond Gerota's fascia
T3a, N0, M0 Tumor grossly extends into renal vein or its segmental branches, or tumor invades the pelvicalyceal system, or tumor invades perirenal and/or renal sinus fat but not beyond Gerota's fascia
T3b, N0, M0 Tumor grossly extends into vena cava below the diaphragm
T3c, N0, M0 Tumor grossly extends into vena cava above the diaphragm or invades the wall of the vena cava
T1-T3, N1, M0 The main tumor can be any size and may be outside the kidney, but it has not spread beyond Gerota’s fascia. The cancer has spread to regional lymph nodes (N1) but has not spread to distant lymph nodes or other organs (M0).
IV T4, any N, M0 Tumor invades beyond Gerota's fascia
Any T, any N, M1 Tumor has spread to distant lymph nodes and/or other organs.

Microscopy report

There should preferably be a free text report and a synoptic report, the free text report including:

  • Diagnosis of clear cell renal cell carcinoma
  • Greatest dimension (from grossing)
  • ISUP/WHO grade
  • Radicality

Example:

Kidney, left, partial nephrectomy:
  • Clear cell renal cell carcinoma, 3.2 cm in greatest dimension, ISUP/WHO grade 3.
  • Surgical margins are negative for carcinoma.

Synoptic report

The synoptic report should include:
Specimen:

  • Procedure: Total or partial nephrectomy
  • Laterality: Left or right

Tumor

  • Histologic type: Such as papillary renal cell carcinoma, type 1
  • Histologic grade
  • Tumor size: From gross description
  • Tumor focality: Unifocal or multifocal
  • Tumor extension: Such as tumor limited to kidney
  • Sarcomatoid features: Identified or not identified
  • Rhabdoid features: Identified or not identified
  • Tumor necrosis: Identified or not identified

Margins

  • Margin involvement: Involved by invasive carcinoma (and in which direction), or negative for malignancy
  • Margins type(s): Generally renal parenchymal margin if partial nephrectomy

Staging

  • Regional lymph nodes: Involved by invasive carcinoma (and how many), or "no lymph nodes submitted or found"
  • Pathologic stage classification (by pTNM, AJCC 8th Edition):
  • Primary tumor (pT)
  • Regional lymph nodes (pN): Such as pNX if no lymph nodes submitted or found

Any pathologic findings in non-neoplastic kidney

Notes

  1. For a full list of contributors, see article history. Creators of images are attributed at the image description pages, seen by clicking on the images. See Patholines:Authorship for details.

Main page

References

  1. 1.0 1.1 Sirohi, Deepika; Smith, Steven C.; Agarwal, Neeraj; Maughan, Benjamin L. (2018). "Unclassified renal cell carcinoma: diagnostic difficulties and treatment modalities ". Research and Reports in Urology Volume 10: 205–217. doi:10.2147/RRU.S154932. ISSN 2253-2447. 
  2. YiFen Zhang. What is the ISUP/WHO grading system for renal cell carcinoma (RCC)?. Medscape. Updated: Jul 02, 2019
  3. Moch, H. (2016). "WHO-ISUP-Graduierungssystem für Nierenkarzinome ". Der Pathologe 37 (4): 355–360. doi:10.1007/s00292-016-0171-y. ISSN 0172-8113. 
  4. . Kidney Cancer Stages. cancer.org. Last Revised: February 1, 2020
  5. Swami, Umang; Nussenzveig, Roberto H.; Haaland, Benjamin; Agarwal, Neeraj (2019). "Revisiting AJCC TNM staging for renal cell carcinoma: quest for improvement ". Annals of Translational Medicine 7 (S1): S18–S18. doi:10.21037/atm.2019.01.50. ISSN 23055839. 

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