Difference between revisions of "Tuberous sclerosis-associated renal cell carcinoma"

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[[Image:Renal cell carcinoma in TSC - alt -- high mag.jpg|thumb|right|[[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]].<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>
'''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>


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Notes:
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]]''.<ref name=pmid26414221>{{Cite journal  | last1 = Trpkov | first1 = K. | last2 = Hes | first2 = O. | last3 = Bonert | first3 = M. | last4 = Lopez | first4 = JI. | last5 = Bonsib | first5 = SM. | last6 = Nesi | first6 = G. | last7 = Comperat | first7 = E. | last8 = Sibony | first8 = M. | last9 = Berney | first9 = DM. | title = Eosinophilic, Solid, and Cystic Renal Cell Carcinoma: Clinicopathologic Study of 16 Unique, Sporadic Neoplasms Occurring in Women. | journal = Am J Surg Pathol | volume =  | issue =  | pages =  | month = Sep | year = 2015 | doi = 10.1097/PAS.0000000000000508 | PMID = 26414221 }}</ref>
*There is also a paper by Yang ''et al.''; it describes three different patterns:<ref name=pmid24832166/>
*There is also a paper by Yang ''et al.''; it describes three different patterns:<ref name=pmid24832166/>
*#TSC-associated [[papillary RCC]].
*#TSC-associated [[papillary RCC]].
*#[[Renal cell carcinoma, unclassified]].
*#[[Renal cell carcinoma, unclassified]].
*#Hybrid oncocytic/chromophobe tumour (HOCT).
*#[[Renal hybrid oncocytic/chromophobe tumour|Hybrid oncocytic/chromophobe tumour]] (HOCT).


DDx:
DDx:
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*[[Unclassified renal cell carcinoma]].
*[[Unclassified renal cell carcinoma]].


===Images===
<gallery>
Image: Renal cell carcinoma in TSC -- very low mag.jpg | RCC-TSC - very low mag. (WC)
Image: Renal cell carcinoma in TSC -- low mag.jpg | RCC-TSC - low mag. (WC)
Image: Renal cell carcinoma in TSC -- intermed mag.jpg | RCC-TSC - intermed. mag. (WC)
Image: Renal cell carcinoma in TSC -- high mag.jpg | RCC-TSC - high mag. (WC)
Image: Renal cell carcinoma in TSC -- very high mag.jpg | RCC-TSC - very high mag. (WC)
Image: Renal cell carcinoma in TSC - alt -- high mag.jpg | RCC-TSC - high mag. (WC)
Image: Renal cell carcinoma in TSC - b -- very low mag.jpg | RCC-TSC - very low mag. (WC)
Image: Renal cell carcinoma in TSC - b2 -- low mag.jpg | RCC-TSC - low mag. (WC)
Image: Renal cell carcinoma in TSC - b1 -- intermed mag.jpg | RCC-TSC - intermed. mag. (WC)
Image: Renal cell carcinoma in TSC - b2 -- intermed mag.jpg | RCC-TSC - intermed. mag. (WC)
Image: Renal cell carcinoma in TSC - b -- high mag.jpg | RCC-TSC - high mag. (WC)
Image: Renal cell carcinoma in TSC - b -- very high mag.jpg | RCC-TSC - very high mag. (WC)
</gallery>
==See also==
==See also==
*[[Renal tumours]].
*[[Renal tumours]].
*[[Tuberous sclerosis]].
*[[Tuberous sclerosis]].
*[[TCEB1-mutated renal cell carcinoma]].
*[[ELOC-mutated renal cell carcinoma]].
*[[Renal neoplasms with TSC/mTOR pathway mutations]].


==References==
==References==

Latest revision as of 04:17, 20 March 2024

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

Microscopic

Features as per Guo et al.[1] describes three different morphologies:

  1. Renal angiomyoadenomatous tumour-like.
  2. Chromophobe renal cell carcinoma-like.
  3. Macrocystic architecture composed of cells with a granular eosinophilic cytoplasm.

Notes:

DDx:

Images

See also

References

  1. 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.
  2. 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. 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.
  4. 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.