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.[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
- TSC/mTOR pathway mutation.[2]
- A somewhat non-specific finding as several renal tumours (with a different morphology) have TSC/mTOR pathway mutations.
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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
- ↑ 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.
- ↑ 2.0 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.
- ↑ 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.