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 | {{ 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> | ||
Clincal: | Clincal: | ||
*Typical age (years) 50s.<ref name=pmid22534244/> | *Typical age (years): 50s.<ref name=pmid22534244/> | ||
*Age range 40-93 years. | **Age range: 40-93 years. | ||
==Gross== | ==Gross== | ||
| Line 20: | Line 57: | ||
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]]. | ||
*[[Mixed epithelial and stromal tumour of the kidney]]. | *[[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== | ==IHC== | ||
| Line 31: | Line 82: | ||
*CAM5.2 +ve. | *CAM5.2 +ve. | ||
*AE1/AE3 +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
- May be on a spectrum with clear cell papillary renal cell carcinoma.[1][3]
Clincal:
- Typical age (years): 50s.[4]
- Age range: 40-93 years.
Gross
Features:[4]
- Tan-brown renal mass +/-cystic component.
Microscopic
- Leiomyomatous capsule of variable thickness - key feature.
- Tubular structures with:
- Apical snouts.
- Clear cells with basal nuclei and mild nuclear atypia.
- Clear cell papillary renal cell carcinoma - considered to be the same entity by WHO classification (see above).
- Clear cell renal cell carcinoma.
- Papillary renal cell carcinoma.
- Angiomyolipoma.
- Mixed epithelial and stromal tumour of the kidney.
- Tuberous sclerosis-associated renal cell carcinoma - may have a RAT-like morphology.[7]
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
- Possibly TCEB1 mutation - see TCEB1-mutated renal cell carcinoma.
See also
References
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 4.0 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.
- ↑ 5.0 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.
- ↑ 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.
- ↑ 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.