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>


==General==
==General==
*Not in the [[Vancouver classification|WHO classification of renal neoplasia]].
*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 }}</ref>
*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 - one of three morphologies:<ref name=pmid25093518/>
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:
*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/>
*#TSC-associated [[papillary RCC]].
*#[[Renal cell carcinoma, unclassified]].
*#[[Renal hybrid oncocytic/chromophobe tumour|Hybrid oncocytic/chromophobe tumour]] (HOCT).


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

  • 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

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.
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