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]]<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 | *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> | ||
*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> | ||
==Microscopic== | ==Microscopic== | ||
Features as per Guo ''et al.''<ref name=pmid25093518/> describes three different morphologies: | Features as per Guo ''et al.''<ref name=pmid25093518/> describes three different morphologies: | ||
#[[Renal angiomyoadenomatous tumour]]-like. | *#[[Renal angiomyoadenomatous tumour]]-like. | ||
#[[Chromophobe renal cell carcinoma]]-like. | *#[[Chromophobe renal cell carcinoma]]-like. | ||
#Macrocystic architecture composed of cells with a granular eosinophilic cytoplasm. | *#Macrocystic architecture composed of cells with a granular eosinophilic cytoplasm. | ||
Notes: | Notes: | ||
Revision as of 15:35, 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]
- 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:
- 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.
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.
- ↑ 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.