Difference between revisions of "Renal cell carcinoma with fibromyomatous stroma"

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'''Renal cell carcinoma with fibromyomatous stroma''', abbreviated '''RCC FMS''', is an evolving subtype of [[renal cell carcinoma]].<ref>{{cite journal |vauthors=Trpkov K, Williamson SR, Gill AJ, Adeniran AJ, Agaimy A, Alaghehbandan R, Amin MB, Argani P, Chen YB, Cheng L, Epstein JI, Cheville JC, Comperat E, da Cunha IW, Gordetsky JB, Gupta S, He H, Hirsch MS, Humphrey PA, Kapur P, Kojima F, Lopez JI, Maclean F, Magi-Galluzzi C, McKenney JK, Mehra R, Menon S, Netto GJ, Przybycin CG, Rao P, Rao Q, Reuter VE, Saleeb RM, Shah RB, Smith SC, Tickoo S, Tretiakova MS, True L, Verkarre V, Wobker SE, Zhou M, Hes O |title=Novel, emerging and provisional renal entities: The Genitourinary Pathology Society (GUPS) update on renal neoplasia |journal=Mod Pathol |volume=34 |issue=6 |pages=1167–1184 |date=June 2021 |pmid=33526874 |doi=10.1038/s41379-021-00737-6 |url=}}</ref>
'''Renal cell carcinoma with fibromyomatous stroma''', abbreviated '''RCC FMS''', is an evolving subtype of [[renal cell carcinoma]].<ref>{{cite journal |authors=Trpkov K, Williamson SR, Gill AJ, Adeniran AJ, Agaimy A, Alaghehbandan R, Amin MB, Argani P, Chen YB, Cheng L, Epstein JI, Cheville JC, Comperat E, da Cunha IW, Gordetsky JB, Gupta S, He H, Hirsch MS, Humphrey PA, Kapur P, Kojima F, Lopez JI, Maclean F, Magi-Galluzzi C, McKenney JK, Mehra R, Menon S, Netto GJ, Przybycin CG, Rao P, Rao Q, Reuter VE, Saleeb RM, Shah RB, Smith SC, Tickoo S, Tretiakova MS, True L, Verkarre V, Wobker SE, Zhou M, Hes O |title=Novel, emerging and provisional renal entities: The Genitourinary Pathology Society (GUPS) update on renal neoplasia |journal=Mod Pathol |volume=34 |issue=6 |pages=1167–1184 |date=June 2021 |pmid=33526874 |doi=10.1038/s41379-021-00737-6 |url=}}</ref>


''Renal cell carcinoma with leiomyomatous stroma'' redirects here.


==General==
*Rare tumour: 12 in >500 renal cell carcinomas with clear cells.<ref name=pmid36592877/>
==Microscopic==
Features:<ref name=pmid36592877>{{cite journal |authors=Tjota MY, Sharma A, Wanjari P, Fitzpatrick C, Segal J, Antic T |title=TSC/MTOR mutated renal cell carcinoma with leiomyomatous stroma is a distinct entity: a comprehensive study of 12 cases |journal=Hum Pathol |volume=134 |issue= |pages=124–133 |date=April 2023 |pmid=36592877 |doi=10.1016/j.humpath.2022.12.015 |url=}}</ref>
*Fibromyomatous stroma (smooth muscle stroma) - '''key feature'''.
*Varied architecture: nested, solid, tubular, tubulopapillary.
*WHO/ISUP grade 3 or 4.
*Nuclei ''not'' polarized in the cell.
DDx:
*[[Clear cell renal cell carcinoma]].
*[[ELOC-mutated renal cell carcinoma]].
*[[Clear cell papillary renal cell tumour]].
==IHC==
Features:<ref name=pmid36592877/>
*CD10 +ve.
*CK7 +ve (most).
*CA9 +ve.
===Comparison between some renal tumours with clear cells===
{| class="wikitable sortable"
!Tumour
!CK7
!CD10
!CK34betaE12 (K903)
![[GPNMB]]
|-
|Clear cell renal cell carcinoma
| -ve (may be focal)
| +ve
| -ve
| usu. -ve
|-
|Clear cell papillary renal cell tumour
| +ve (diffuse)
| -ve
| +ve
| -ve<ref name=pmid37661807>{{cite journal |authors=Li H, Argani P, Halper-Stromberg E, Lotan TL, Merino MJ, Reuter VE, Matoso A |title=Positive GPNMB Immunostaining Differentiates Renal Cell Carcinoma With Fibromyomatous Stroma Associated With TSC1/2/MTOR Alterations From Others |journal=Am J Surg Pathol |volume=47 |issue=11 |pages=1267–1273 |date=November 2023 |pmid=37661807 |doi=10.1097/PAS.0000000000002117 |url=}}</ref>
|-
|ELOC-mutated renal cell carcinoma
| +ve
| +ve
| -ve
| -ve
|-
|Renal cell carcinoma with fibromyomatous stroma
| +ve
| +ve
| -ve
| +ve
|-
|}
==Molecular==
*[[Renal neoplasms with TSC/mTOR pathway mutations|TSC/mTOR pathway mutation]].<ref name=pmid36592877/>
**A somewhat non-specific finding as [[Renal neoplasms with TSC/mTOR pathway mutations|several renal tumours (with a different morphology) have TSC/mTOR pathway mutations]].
==Sign out==
===Molecular lacking===
<pre>
Right Kidney, Radical Nephrectomy:
    - RENAL CELL CARCINOMA with clear cells, cysts and leiomyomatous stroma, see comment.
    -- Margin clear.
    -- Please see synoptic report for details.
Comment:
The tumour stains as follows:
POSITIVE: CD10 (moderate), EMA (patchy), CK7 (diffuse), PAX8 (diffuse).
NEGATIVE: CK34betaE12, CK20.
The differential diagnosis includes: (1) clear cell renal cell carcinoma, (2) RCC with a mTOR pathway mutation, and (3) ELOC-mutated RCC.
</pre>


==See also==
==See also==
*[[Renal cell carcinoma]].
*[[Renal cell carcinoma]].
*[[Clear cells in the kidney]].


==References==
==References==

Latest revision as of 20:32, 21 October 2024

Renal cell carcinoma with fibromyomatous stroma, abbreviated RCC FMS, is an evolving subtype of renal cell carcinoma.[1]

Renal cell carcinoma with leiomyomatous stroma redirects here.

General

  • Rare tumour: 12 in >500 renal cell carcinomas with clear cells.[2]

Microscopic

Features:[2]

  • Fibromyomatous stroma (smooth muscle stroma) - key feature.
  • Varied architecture: nested, solid, tubular, tubulopapillary.
  • WHO/ISUP grade 3 or 4.
  • Nuclei not polarized in the cell.

DDx:

IHC

Features:[2]

  • CD10 +ve.
  • CK7 +ve (most).
  • CA9 +ve.

Comparison between some renal tumours with clear cells

Tumour CK7 CD10 CK34betaE12 (K903) GPNMB
Clear cell renal cell carcinoma -ve (may be focal) +ve -ve usu. -ve
Clear cell papillary renal cell tumour +ve (diffuse) -ve +ve -ve[3]
ELOC-mutated renal cell carcinoma +ve +ve -ve -ve
Renal cell carcinoma with fibromyomatous stroma +ve +ve -ve +ve

Molecular

Sign out

Molecular lacking

Right Kidney, Radical Nephrectomy: 
     - RENAL CELL CARCINOMA with clear cells, cysts and leiomyomatous stroma, see comment.
     -- Margin clear.
     -- Please see synoptic report for details.

Comment:
The tumour stains as follows:
POSITIVE: CD10 (moderate), EMA (patchy), CK7 (diffuse), PAX8 (diffuse).
NEGATIVE: CK34betaE12, CK20.

The differential diagnosis includes: (1) clear cell renal cell carcinoma, (2) RCC with a mTOR pathway mutation, and (3) ELOC-mutated RCC.

See also

References

  1. ↑ Trpkov K, Williamson SR, Gill AJ, Adeniran AJ, Agaimy A, Alaghehbandan R, Amin MB, Argani P, Chen YB, Cheng L, Epstein JI, Cheville JC, Comperat E, da Cunha IW, Gordetsky JB, Gupta S, He H, Hirsch MS, Humphrey PA, Kapur P, Kojima F, Lopez JI, Maclean F, Magi-Galluzzi C, McKenney JK, Mehra R, Menon S, Netto GJ, Przybycin CG, Rao P, Rao Q, Reuter VE, Saleeb RM, Shah RB, Smith SC, Tickoo S, Tretiakova MS, True L, Verkarre V, Wobker SE, Zhou M, Hes O (June 2021). "Novel, emerging and provisional renal entities: The Genitourinary Pathology Society (GUPS) update on renal neoplasia". Mod Pathol 34 (6): 1167–1184. doi:10.1038/s41379-021-00737-6. PMID 33526874.
  2. ↑ 2.0 2.1 2.2 2.3 Tjota MY, Sharma A, Wanjari P, Fitzpatrick C, Segal J, Antic T (April 2023). "TSC/MTOR mutated renal cell carcinoma with leiomyomatous stroma is a distinct entity: a comprehensive study of 12 cases". Hum Pathol 134: 124–133. doi:10.1016/j.humpath.2022.12.015. PMID 36592877.
  3. ↑ Li H, Argani P, Halper-Stromberg E, Lotan TL, Merino MJ, Reuter VE, Matoso A (November 2023). "Positive GPNMB Immunostaining Differentiates Renal Cell Carcinoma With Fibromyomatous Stroma Associated With TSC1/2/MTOR Alterations From Others". Am J Surg Pathol 47 (11): 1267–1273. doi:10.1097/PAS.0000000000002117. PMID 37661807.
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