Difference between revisions of "Renal angiomyoadenomatous tumour"

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'''Renal angiomyoadenomatous tumour''', abbreviated '''RAT''', is an uncommon [[kidney tumour]] not recognized by the [[WHO]] classification of renal neoplasia or the Vancouver modification.
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Clear_cell_tubulopapillary_RCC_with_smooth_muscle_stroma_-_a2_--_intermed_mag.jpg
| Width      =
| Caption    = Renal cell carcinoma with clear cells and leiomyomatous bands. [[H&E stain]].
| Synonyms  = renal cell carcinoma with angioleiomyoma-like stroma, renal cell carcinoma associated with prominent angioleiomyoma-like proliferation
| Micro      = leiomyomatous capsule of variable thickness, tubular structures with (1) apical snouts, and (2) clear cells with basal nuclei and mild [[nuclear atypia]]
| Subtypes  =
| LMDDx      = [[clear cell renal cell carcinoma]], [[papillary renal cell carcinoma]], (epithelioid) [[angiomyolipoma]], [[mixed epithelial and stromal tumour of the kidney|mixed epithelial and stromal tumour]], [[tuberous sclerosis-associated renal cell carcinoma]]
| Stains    =
| IHC        = CK7 +ve, CK20 +ve, CAM5.2 +ve
| EM        =
| Molecular  =
| IF        =
| Gross      = tan-brown mass +/-cystic component
| Grossing  = [[partial nephrectomy grossing]], [[total nephrectomy for tumour grossing]]
| Site      = [[kidney]] - see ''[[kidney tumours]]''
| Assdx      = possibly related to [[clear cell papillary renal cell carcinoma]] - the WHO lumps this entity with it
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = extremely rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = good
| Other      =
| ClinDDx    =
| Tx        =
}}
'''Renal angiomyoadenomatous tumour''', abbreviated '''RAT''', is an uncommon [[kidney tumour]] not recognized by the [[WHO]] classification of renal neoplasia.
 
''The [[Vancouver classification|Vancouver modification]] of the WHO classification''<ref name=pmid24025519>{{Cite journal  | last1 = Srigley | first1 = JR. | last2 = Delahunt | first2 = B. | last3 = Eble | first3 = JN. | last4 = Egevad | first4 = L. | last5 = Epstein | first5 = JI. | last6 = Grignon | first6 = D. | last7 = Hes | first7 = O. | last8 = Moch | first8 = H. | last9 = Montironi | first9 = R. | title = The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia. | journal = Am J Surg Pathol | volume = 37 | issue = 10 | pages = 1469-89 | month = Oct | year = 2013 | doi = 10.1097/PAS.0b013e318299f2d1 | PMID = 24025519 }}</ref> lumps it with [[clear cell papillary renal cell carcinoma]] (also known as ''clear cell tubulopapillary renal cell carcinoma'').
 
Synonyms are '''renal cell carcinoma with angioleiomyoma-like stroma''' and '''renal cell carcinoma associated with prominent angioleiomyoma-like proliferation'''.<ref name=pmid25189644>{{Cite journal  | last1 = Williamson | first1 = SR. | last2 = Cheng | first2 = L. | last3 = Eble | first3 = JN. | last4 = True | first4 = LD. | last5 = Gupta | first5 = NS. | last6 = Wang | first6 = M. | last7 = Zhang | first7 = S. | last8 = Grignon | first8 = DJ. | title = Renal cell carcinoma with angioleiomyoma-like stroma: clinicopathological, immunohistochemical, and molecular features supporting classification as a distinct entity. | journal = Mod Pathol | volume = 28 | issue = 2 | pages = 279-94 | month = Feb | year = 2015 | doi = 10.1038/modpathol.2014.105 | PMID = 25189644 }}</ref>
 
It may be one and the same with [[TCEB1-mutated renal cell carcinoma]].


==General==
==General==
*May be on a spectrum with [[clear cell papillary renal cell carcinoma]].<ref name=pmid23434146>{{Cite journal  | last1 = Petersson | first1 = F. | last2 = Grossmann | first2 = P. | last3 = Hora | first3 = M. | last4 = Sperga | first4 = M. | last5 = Montiel | first5 = DP. | last6 = Martinek | first6 = P. | last7 = Gutierrez | first7 = ME. | last8 = Bulimbasic | first8 = S. | last9 = Michal | first9 = M. | title = Renal cell carcinoma with areas mimicking renal angiomyoadenomatous tumor/clear cell papillary renal cell carcinoma. | journal = Hum Pathol | volume = 44 | issue = 7 | pages = 1412-20 | month = Jul | year = 2013 | doi = 10.1016/j.humpath.2012.11.019 | PMID = 23434146 }}</ref>
*May be on a spectrum with [[clear cell papillary renal cell carcinoma]].<ref name=pmid24025519/><ref name=pmid23434146>{{Cite journal  | last1 = Petersson | first1 = F. | last2 = Grossmann | first2 = P. | last3 = Hora | first3 = M. | last4 = Sperga | first4 = M. | last5 = Montiel | first5 = DP. | last6 = Martinek | first6 = P. | last7 = Gutierrez | first7 = ME. | last8 = Bulimbasic | first8 = S. | last9 = Michal | first9 = M. | title = Renal cell carcinoma with areas mimicking renal angiomyoadenomatous tumor/clear cell papillary renal cell carcinoma. | journal = Hum Pathol | volume = 44 | issue = 7 | pages = 1412-20 | month = Jul | year = 2013 | doi = 10.1016/j.humpath.2012.11.019 | PMID = 23434146 }}</ref>
*First descried in 2000.


Clincal:
Clincal:
*Usually middle age (40s and 50s).
*Typical age (years): 50s.<ref name=pmid22534244/>
**Age range: 40-93 years.


==Gross==
==Gross==
Line 13: Line 50:


==Microscopic==
==Microscopic==
Features:<ref name=pmid22534244>{{Cite journal  | last1 = Singh | first1 = C. | last2 = Kendi | first2 = AT. | last3 = Manivel | first3 = JC. | last4 = Pambuccian | first4 = SE. | title = Renal angiomyoadenomatous tumor. | journal = Ann Diagn Pathol | volume = 16 | issue = 6 | pages = 470-6 | month = Dec | year = 2012 | doi = 10.1016/j.anndiagpath.2012.01.006 | PMID = 22534244 }}</ref>
Features:<ref name=pmid22534244>{{Cite journal  | last1 = Singh | first1 = C. | last2 = Kendi | first2 = AT. | last3 = Manivel | first3 = JC. | last4 = Pambuccian | first4 = SE. | title = Renal angiomyoadenomatous tumor. | journal = Ann Diagn Pathol | volume = 16 | issue = 6 | pages = 470-6 | month = Dec | year = 2012 | doi = 10.1016/j.anndiagpath.2012.01.006 | PMID = 22534244 }}</ref><ref name=pmid19020896>{{Cite journal  | last1 = Michal | first1 = M. | last2 = Hes | first2 = O. | last3 = Nemcova | first3 = J. | last4 = Sima | first4 = R. | last5 = Kuroda | first5 = N. | last6 = Bulimbasic | first6 = S. | last7 = Franco | first7 = M. | last8 = Sakaida | first8 = N. | last9 = Danis | first9 = D. | title = Renal angiomyoadenomatous tumor: morphologic, immunohistochemical, and molecular genetic study of a distinct entity. | journal = Virchows Arch | volume = 454 | issue = 1 | pages = 89-99 | month = Jan | year = 2009 | doi = 10.1007/s00428-008-0697-3 | PMID = 19020896 }}</ref>
*Leiomyomatous capsule of variable thickness - '''key feature'''.
*Leiomyomatous capsule of variable thickness - '''key feature'''.
*Clear cell with basal nuclei and mild [[nuclear atypia]].
*Tubular structures with:
**Apical snouts.
**Clear cells with basal nuclei and mild [[nuclear atypia]].


DDx:<ref name=pmid22534244/><ref>{{Cite journal  | last1 = Petersson | first1 = F. | last2 = Branzovsky | first2 = J. | last3 = Martinek | first3 = P. | last4 = Korabecna | first4 = M. | last5 = Kruslin | first5 = B. | last6 = Hora | first6 = M. | last7 = Peckova | first7 = K. | last8 = Bauleth | first8 = K. | last9 = Pivovarcikova | first9 = K. | title = The leiomyomatous stroma in renal cell carcinomas is polyclonal and not part of the neoplastic process. | journal = Virchows Arch | volume = 465 | issue = 1 | pages = 89-96 | month = Jul | year = 2014 | doi = 10.1007/s00428-014-1591-9 | PMID = 24838683 }}</ref>
DDx:<ref name=pmid22534244/><ref>{{Cite journal  | last1 = Petersson | first1 = F. | last2 = Branzovsky | first2 = J. | last3 = Martinek | first3 = P. | last4 = Korabecna | first4 = M. | last5 = Kruslin | first5 = B. | last6 = Hora | first6 = M. | last7 = Peckova | first7 = K. | last8 = Bauleth | first8 = K. | last9 = Pivovarcikova | first9 = K. | title = The leiomyomatous stroma in renal cell carcinomas is polyclonal and not part of the neoplastic process. | journal = Virchows Arch | volume = 465 | issue = 1 | pages = 89-96 | month = Jul | year = 2014 | doi = 10.1007/s00428-014-1591-9 | PMID = 24838683 }}</ref>
*[[Clear cell papillary renal cell carcinoma]] - considered to be the same entity by WHO classification (see above).
*[[Clear cell renal cell carcinoma]].
*[[Clear cell renal cell carcinoma]].
*[[Papillary renal cell carcinoma]].
*[[Papillary renal cell carcinoma]].
*[[Angiomyolipoma]].
*[[Angiomyolipoma]].
*[[Renal mixed epithelial and stromal tumour]].
*[[Mixed epithelial and stromal tumour of the kidney]].
*[[Tuberous sclerosis-associated renal cell carcinoma]] - may have a RAT-like morphology.<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>
 
===Images===
<gallery>
Image: Clear cell tubulopapillary RCC with smooth muscle stroma -- low mag.jpg | CCTPRCC with SMS - low mag.
Image: Clear cell tubulopapillary RCC with smooth muscle stroma -- intermed mag.jpg | CCTPRCC with SMS - intermed. mag.
 
Image: Clear cell tubulopapillary RCC with smooth muscle stroma - a1 -- intermed mag.jpg | CCTPRCC with SMS - intermed. mag.
Image: Clear cell tubulopapillary RCC with smooth muscle stroma - a2 -- intermed mag.jpg | CCTPRCC with SMS - intermed. mag.
 
Image: Clear cell tubulopapillary RCC with smooth muscle stroma - b -- intermed mag.jpg | CCTPRCC with SMS - intermed. mag.
Image: Clear cell tubulopapillary RCC with smooth muscle stroma - b -- high mag.jpg | CCTPRCC with SMS - high mag.
</gallery>
 
==IHC==
Features:<ref name=pmid19020896/>
*CK7 +ve.
*CK20 +ve.
*CAM5.2 +ve.
*AE1/AE3 +ve.
 
==Molecular==
*Possibly TCEB1 mutation - see ''[[TCEB1-mutated renal cell carcinoma]]''.


==See also==
==See also==
*[[Kidney tumours]].
*[[Kidney tumours]].
*[[Tuberous sclerosis]].
*[[Vancouver classification]].
*[[TCEB1-mutated renal cell carcinoma]].


==References==
==References==

Latest revision as of 22:41, 9 August 2015

Renal angiomyoadenomatous tumour
Diagnosis in short

Renal cell carcinoma with clear cells and leiomyomatous bands. H&E stain.
Synonyms renal cell carcinoma with angioleiomyoma-like stroma, renal cell carcinoma associated with prominent angioleiomyoma-like proliferation
LM leiomyomatous capsule of variable thickness, tubular structures with (1) apical snouts, and (2) clear cells with basal nuclei and mild nuclear atypia
LM DDx clear cell renal cell carcinoma, papillary renal cell carcinoma, (epithelioid) angiomyolipoma, mixed epithelial and stromal tumour, tuberous sclerosis-associated renal cell carcinoma
IHC CK7 +ve, CK20 +ve, CAM5.2 +ve
Gross tan-brown mass +/-cystic component
Grossing notes partial nephrectomy grossing, total nephrectomy for tumour grossing
Site kidney - see kidney tumours
Associated Dx possibly related to clear cell papillary renal cell carcinoma - the WHO lumps this entity with it
Prevalence extremely rare
Prognosis good

Renal angiomyoadenomatous tumour, abbreviated RAT, is an uncommon kidney tumour not recognized by the WHO classification of renal neoplasia.

The Vancouver modification of the WHO classification[1] lumps it with clear cell papillary renal cell carcinoma (also known as clear cell tubulopapillary renal cell carcinoma).

Synonyms are renal cell carcinoma with angioleiomyoma-like stroma and renal cell carcinoma associated with prominent angioleiomyoma-like proliferation.[2]

It may be one and the same with TCEB1-mutated renal cell carcinoma.

General

Clincal:

  • Typical age (years): 50s.[4]
    • Age range: 40-93 years.

Gross

Features:[4]

  • Tan-brown renal mass +/-cystic component.

Microscopic

Features:[4][5]

  • Leiomyomatous capsule of variable thickness - key feature.
  • Tubular structures with:

DDx:[4][6]

Images

  • CCTPRCC with SMS - low mag.

  • CCTPRCC with SMS - intermed. mag.

  • CCTPRCC with SMS - intermed. mag.

  • CCTPRCC with SMS - intermed. mag.

  • CCTPRCC with SMS - intermed. mag.

  • CCTPRCC with SMS - high mag.

IHC

Features:[5]

  • CK7 +ve.
  • CK20 +ve.
  • CAM5.2 +ve.
  • AE1/AE3 +ve.

Molecular

See also

References

  1. ↑ 1.0 1.1 Srigley, JR.; Delahunt, B.; Eble, JN.; Egevad, L.; Epstein, JI.; Grignon, D.; Hes, O.; Moch, H. et al. (Oct 2013). "The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia.". Am J Surg Pathol 37 (10): 1469-89. doi:10.1097/PAS.0b013e318299f2d1. PMID 24025519.
  2. ↑ Williamson, SR.; Cheng, L.; Eble, JN.; True, LD.; Gupta, NS.; Wang, M.; Zhang, S.; Grignon, DJ. (Feb 2015). "Renal cell carcinoma with angioleiomyoma-like stroma: clinicopathological, immunohistochemical, and molecular features supporting classification as a distinct entity.". Mod Pathol 28 (2): 279-94. doi:10.1038/modpathol.2014.105. PMID 25189644.
  3. ↑ Petersson, F.; Grossmann, P.; Hora, M.; Sperga, M.; Montiel, DP.; Martinek, P.; Gutierrez, ME.; Bulimbasic, S. et al. (Jul 2013). "Renal cell carcinoma with areas mimicking renal angiomyoadenomatous tumor/clear cell papillary renal cell carcinoma.". Hum Pathol 44 (7): 1412-20. doi:10.1016/j.humpath.2012.11.019. PMID 23434146.
  4. ↑ 4.0 4.1 4.2 4.3 Singh, C.; Kendi, AT.; Manivel, JC.; Pambuccian, SE. (Dec 2012). "Renal angiomyoadenomatous tumor.". Ann Diagn Pathol 16 (6): 470-6. doi:10.1016/j.anndiagpath.2012.01.006. PMID 22534244.
  5. ↑ 5.0 5.1 Michal, M.; Hes, O.; Nemcova, J.; Sima, R.; Kuroda, N.; Bulimbasic, S.; Franco, M.; Sakaida, N. et al. (Jan 2009). "Renal angiomyoadenomatous tumor: morphologic, immunohistochemical, and molecular genetic study of a distinct entity.". Virchows Arch 454 (1): 89-99. doi:10.1007/s00428-008-0697-3. PMID 19020896.
  6. ↑ Petersson, F.; Branzovsky, J.; Martinek, P.; Korabecna, M.; Kruslin, B.; Hora, M.; Peckova, K.; Bauleth, K. et al. (Jul 2014). "The leiomyomatous stroma in renal cell carcinomas is polyclonal and not part of the neoplastic process.". Virchows Arch 465 (1): 89-96. doi:10.1007/s00428-014-1591-9. PMID 24838683.
  7. ↑ 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.
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