Difference between revisions of "Tuberous sclerosis-associated renal cell carcinoma"
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==General== | ==General== | ||
*Not in the [[WHO classification of renal neoplasia]] or the [[Vancouver classification|Vancouver modification of the WHO classification]]. | *Not in the [[WHO classification of renal neoplasia]] or the [[Vancouver classification|Vancouver modification of the WHO classification]]. | ||
*Afflicts individuals at a younger age than sporadic [[renal cell carcinoma]] | *Afflicts individuals at a younger age than sporadic [[renal cell carcinoma]]<ref name=pmid8863669>{{Cite journal | last1 = Bjornsson | first1 = J. | last2 = Short | first2 = MP. | last3 = Kwiatkowski | first3 = DJ. | last4 = Henske | first4 = EP. | title = Tuberous sclerosis-associated renal cell carcinoma. Clinical, pathological, and genetic features. | journal = Am J Pathol | volume = 149 | issue = 4 | pages = 1201-8 | month = Oct | year = 1996 | doi = | PMID = 8863669 }}</ref> - average age of 36 years.<ref name=pmid9827727>{{cite journal |author=Al-Saleem T, Wessner LL, Scheithauer BW, ''et al.'' |title=Malignant tumors of the kidney, brain, and soft tissues in children and young adults with the tuberous sclerosis complex |journal=Cancer |volume=83 |issue=10 |pages=2208–16 |year=1998 |month=November |pmid=9827727 |doi= |url=}}</ref> | ||
*2-4% of individual with tuberous sclerosis develop renal cell carcinoma.<ref name=pmid24832166>{{Cite journal | last1 = Yang | first1 = P. | last2 = Cornejo | first2 = KM. | last3 = Sadow | first3 = PM. | last4 = Cheng | first4 = L. | last5 = Wang | first5 = M. | last6 = Xiao | first6 = Y. | last7 = Jiang | first7 = Z. | last8 = Oliva | first8 = E. | last9 = Jozwiak | first9 = S. | title = Renal cell carcinoma in tuberous sclerosis complex. | journal = Am J Surg Pathol | volume = 38 | issue = 7 | pages = 895-909 | month = Jul | year = 2014 | doi = 10.1097/PAS.0000000000000237 | PMID = 24832166 }}</ref> | *2-4% of individual with tuberous sclerosis develop renal cell carcinoma.<ref name=pmid24832166>{{Cite journal | last1 = Yang | first1 = P. | last2 = Cornejo | first2 = KM. | last3 = Sadow | first3 = PM. | last4 = Cheng | first4 = L. | last5 = Wang | first5 = M. | last6 = Xiao | first6 = Y. | last7 = Jiang | first7 = Z. | last8 = Oliva | first8 = E. | last9 = Jozwiak | first9 = S. | title = Renal cell carcinoma in tuberous sclerosis complex. | journal = Am J Surg Pathol | volume = 38 | issue = 7 | pages = 895-909 | month = Jul | year = 2014 | doi = 10.1097/PAS.0000000000000237 | PMID = 24832166 }}</ref> | ||
Revision as of 15:32, 10 December 2014
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] - average age of 36 years.[3]
- 2-4% of individual with tuberous sclerosis develop renal cell carcinoma.[4]
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:
- There is also a paper by Yang et al.; it describes three different patterns:[4]
- 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.
See also
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.
- ↑ Al-Saleem T, Wessner LL, Scheithauer BW, et al. (November 1998). "Malignant tumors of the kidney, brain, and soft tissues in children and young adults with the tuberous sclerosis complex". Cancer 83 (10): 2208–16. PMID 9827727.
- ↑ 4.0 4.1 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.