Tuberous sclerosis-associated renal cell carcinoma
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Micrograph showing renal cell carcinoma in the context of tuberous sclerosis complex. H&E stain. (WC/Nephron)
Tuberous sclerosis-associated renal cell carcinoma, abbreviated TSARCC, is a kidney tumour that is associated with the syndrome tuberous sclerosis.[1]
General
- Not in the WHO classification of renal neoplasia or the Vancouver modification of the WHO classification.
- Afflicts individuals at a younger age than sporadic renal cell carcinoma.[2]
- 2-4% of individual with tuberous sclerosis develop renal cell carcinoma.[3]
Microscopic
Features as per Guo et al.[1] describes three different morphologies:
- Renal angiomyoadenomatous tumour-like.
- Chromophobe renal cell carcinoma-like.
- Macrocystic architecture composed of cells with a granular eosinophilic cytoplasm.
Notes:
- The macrocystic architecture composed of cells with granular eosinophilic cytoplasm pattern in individuals without documented tuberous sclerosis is an evolving entity known as eosinophilic, solid and cystic renal cell carcinoma.[4]
- There is also a paper by Yang et al.; it describes three different patterns:[3]
- TSC-associated papillary RCC.
- Renal cell carcinoma, unclassified.
- Hybrid oncocytic/chromophobe tumour (HOCT).
DDx:
- Renal angiomyoadenomatous tumour.
- Chromophobe renal cell carcinoma.
- Renal oncocytoma.
- Papillary renal cell carcinoma.
- Hereditary leiomyomatosis renal cell carcinoma syndrome-associated renal cell carcinoma.
- Unclassified renal cell carcinoma.
Images
RCC-TSC - very low mag. (WC)
RCC-TSC - low mag. (WC)
RCC-TSC - intermed. mag. (WC)
RCC-TSC - high mag. (WC)
RCC-TSC - very high mag. (WC)
RCC-TSC - high mag. (WC)
RCC-TSC - very low mag. (WC)
RCC-TSC - low mag. (WC)
RCC-TSC - intermed. mag. (WC)
RCC-TSC - intermed. mag. (WC)
RCC-TSC - high mag. (WC)
RCC-TSC - very high mag. (WC)
See also
- Renal tumours.
- Tuberous sclerosis.
- ELOC-mutated renal cell carcinoma.
- Renal neoplasms with TSC/mTOR pathway mutations.
References
- ↑ 1.0 1.1 Guo, J.; Tretiakova, MS.; Troxell, ML.; Osunkoya, AO.; Fadare, O.; Sangoi, AR.; Shen, SS.; Lopez-Beltran, A. et al. (Nov 2014). "Tuberous Sclerosis-associated Renal Cell Carcinoma: A Clinicopathologic Study of 57 Separate Carcinomas in 18 Patients.". Am J Surg Pathol 38 (11): 1457-67. doi:10.1097/PAS.0000000000000248. PMID 25093518.
- ↑ Bjornsson, J.; Short, MP.; Kwiatkowski, DJ.; Henske, EP. (Oct 1996). "Tuberous sclerosis-associated renal cell carcinoma. Clinical, pathological, and genetic features.". Am J Pathol 149 (4): 1201-8. PMID 8863669.
- ↑ 3.0 3.1 Yang, P.; Cornejo, KM.; Sadow, PM.; Cheng, L.; Wang, M.; Xiao, Y.; Jiang, Z.; Oliva, E. et al. (Jul 2014). "Renal cell carcinoma in tuberous sclerosis complex.". Am J Surg Pathol 38 (7): 895-909. doi:10.1097/PAS.0000000000000237. PMID 24832166.
- ↑ Trpkov, K.; Hes, O.; Bonert, M.; Lopez, JI.; Bonsib, SM.; Nesi, G.; Comperat, E.; Sibony, M. et al. (Sep 2015). "Eosinophilic, Solid, and Cystic Renal Cell Carcinoma: Clinicopathologic Study of 16 Unique, Sporadic Neoplasms Occurring in Women.". Am J Surg Pathol. doi:10.1097/PAS.0000000000000508. PMID 26414221.