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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. | *Typical age (years): 50s.<ref name=pmid22534244/> | ||
*Age range 40-93 years. | **Age range: 40-93 years. | ||
==Gross== | ==Gross== | ||
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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== | ||
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*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== |
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