View source for Tuberous sclerosis-associated renal cell carcinoma

'''Tuberous sclerosis-associated renal cell carcinoma''', abbreviated '''TSARCC''', is a [[kidney tumour]] that is associated with the syndrome [[tuberous sclerosis]].<ref name=pmid25093518>{{Cite journal  | last1 = Guo | first1 = J. | last2 = Tretiakova | first2 = MS. | last3 = Troxell | first3 = ML. | last4 = Osunkoya | first4 = AO. | last5 = Fadare | first5 = O. | last6 = Sangoi | first6 = AR. | last7 = Shen | first7 = SS. | last8 = Lopez-Beltran | first8 = A. | last9 = Mehra | first9 = R. | title = Tuberous Sclerosis-associated Renal Cell Carcinoma: A Clinicopathologic Study of 57 Separate Carcinomas in 18 Patients. | journal = Am J Surg Pathol | volume = 38 | issue = 11 | pages = 1457-67 | month = Nov | year = 2014 | doi = 10.1097/PAS.0000000000000248 | PMID = 25093518 }}</ref>

==General==
*Not in the [[Vancouver classification|WHO classification of renal neoplasia]].
*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>

==Microscopic==
Features:
*Guo ''et al.''<ref name=pmid25093518/> describes three different morphologies:
*#[[Renal angiomyoadenomatous tumour]]-like.
*#[[Chromophobe renal cell carcinoma]]-like.
*#Macrocystic architecture composed of cells with a granular eosinophilic cytoplasm.
*Yang ''et al.''<ref name=pmid24832166/> describes three patterns:
*#TSC-associated papillary RCC.
*#[[Renal cell carcinoma unclassified]].
*#Hybrid oncocytic/chromophobe tumour (HOCT).

DDx:
*[[Renal angiomyoadenomatous tumour]].
*[[Chromophobe renal cell carcinoma]].
*[[Renal oncocytoma]].
*[[Hereditary leiomyomatosis renal cell carcinoma syndrome-associated renal cell carcinoma]].
*[[Renal cell carcinoma unclassified]].

==See also==
*[[Renal tumours]].
*[[Tuberous sclerosis]].

==References==
{{Reflist|2}}

[[Category:Kidney tumours]]