Difference between revisions of "Mixed epithelial and stromal tumour of the kidney"
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'''Mixed epithelial and stromal tumour of the kidney''', abbreviated '''MEST''', is a rare benign [[kidney tumour]]. | {{ Infobox diagnosis | ||
| Name = {{PAGENAME}} | |||
| Image = Mixed epithelial stromal tumour of kidney -- intermed mag.jpg | |||
| Width = | |||
| Caption = Mixed epithelial stromal tumour of kidney. [[H&E stain]]. | |||
| Synonyms = [[mixed epithelial and stromal tumour family]] (term recommended in WHO 2016 classification) | |||
| Micro = cysts lined by simple epithelium with hobnailing, stroma has an ovarian look (basophilic, spindle cells) | |||
| Subtypes = on a spectrum with [[cystic nephroma, adult type]] | |||
| LMDDx = [[cystic nephroma, pediatric type]], [[tubulocystic renal cell carcinoma]] | |||
| Stains = | |||
| IHC = ER +ve, PR +ve, CD10 +ve | |||
| EM = | |||
| Molecular = | |||
| IF = | |||
| Gross = renal mass, predominantly solid | |||
| Grossing = [[total nephrectomy for tumour grossing]], [[partial nephrectomy grossing]] | |||
| Site = [[kidney]] - see ''[[kidney tumour]]'' | |||
| Assdx = | |||
| Syndromes = | |||
| Clinicalhx = | |||
| Signs = | |||
| Symptoms = | |||
| Prevalence = rare | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = benign | |||
| Other = | |||
| ClinDDx = | |||
| Tx = | |||
}} | |||
'''Mixed epithelial and stromal tumour of the kidney''', abbreviated '''MEST''', is a rare benign [[kidney tumour]] in the [[WHO]] classification of renal neoplasia. | |||
Turbiner ''et al.''<ref name=pmid17414095>{{Cite journal | last1 = Turbiner | first1 = J. | last2 = Amin | first2 = MB. | last3 = Humphrey | first3 = PA. | last4 = Srigley | first4 = JR. | last5 = De Leval | first5 = L. | last6 = Radhakrishnan | first6 = A. | last7 = Oliva | first7 = E. | title = Cystic nephroma and mixed epithelial and stromal tumor of kidney: a detailed clinicopathologic analysis of 34 cases and proposal for renal epithelial and stromal tumor (REST) as a unifying term. | journal = Am J Surg Pathol | volume = 31 | issue = 4 | pages = 489-500 | month = Apr | year = 2007 | doi = 10.1097/PAS.0b013e31802bdd56 | PMID = 17414095 }}</ref> | In the 2016 World Health Organization classification of renal neoplasia, this tumour was lumped with ''[[cystic nephroma, adult type]]'' and the recommended term is '''''mixed epithelial and stromal tumour family'''''.<ref name=pmid26935559>{{Cite journal | last1 = Moch | first1 = H. | last2 = Cubilla | first2 = AL. | last3 = Humphrey | first3 = PA. | last4 = Reuter | first4 = VE. | last5 = Ulbright | first5 = TM. | title = The 2016 WHO Classification of Tumours of the Urinary System and Male Genital Organs-Part A: Renal, Penile, and Testicular Tumours. | journal = Eur Urol | volume = 70 | issue = 1 | pages = 93-105 | month = 07 | year = 2016 | doi = 10.1016/j.eururo.2016.02.029 | PMID = 26935559 }}</ref> | ||
Turbiner ''et al.''<ref name=pmid17414095>{{Cite journal | last1 = Turbiner | first1 = J. | last2 = Amin | first2 = MB. | last3 = Humphrey | first3 = PA. | last4 = Srigley | first4 = JR. | last5 = De Leval | first5 = L. | last6 = Radhakrishnan | first6 = A. | last7 = Oliva | first7 = E. | title = Cystic nephroma and mixed epithelial and stromal tumor of kidney: a detailed clinicopathologic analysis of 34 cases and proposal for renal epithelial and stromal tumor (REST) as a unifying term. | journal = Am J Surg Pathol | volume = 31 | issue = 4 | pages = 489-500 | month = Apr | year = 2007 | doi = 10.1097/PAS.0b013e31802bdd56 | PMID = 17414095 }}</ref> came-up with a cheeky term for the the tumour family (''[[renal epithelial and stromal tumour]]'', abbreviated ''REST'') that didn't catch on. | |||
This kidney tumour should '''not''' be confused with ''mixed epithelial-stromal tumour of seminal vesicle''.<ref name=pmid25664946>{{Cite journal | last1 = Reikie | first1 = BA. | last2 = Yilmaz | first2 = A. | last3 = Medlicott | first3 = S. | last4 = Trpkov | first4 = K. | title = Mixed epithelial-stromal tumor (MEST) of seminal vesicle: a proposal for unified nomenclature. | journal = Adv Anat Pathol | volume = 22 | issue = 2 | pages = 113-20 | month = Mar | year = 2015 | doi = 10.1097/PAP.0000000000000057 | PMID = 25664946 }}</ref> | |||
==General== | ==General== | ||
| Line 8: | Line 42: | ||
*Benign. | *Benign. | ||
*Prevalence: males > females. | *Prevalence: males > females. | ||
==Gross== | |||
*Solid and cystic mass - predominantly solid. | |||
==Microscopic== | ==Microscopic== | ||
| Line 18: | Line 55: | ||
Notes: | Notes: | ||
*Parenchymal elements (e.g. glomueruli, tubules) are not found in the septa between the cysts. | *Parenchymal elements (e.g. glomueruli, tubules) are not found in the septa between the cysts. | ||
*May extend into the renal vein.<ref name=pmid27383188>{{Cite journal | last1 = Xie | first1 = WL. | last2 = Lian | first2 = JY. | last3 = Li | first3 = B. | last4 = Tian | first4 = XY. | last5 = Li | first5 = Z. | title = Mixed epithelial and stromal tumor of kidney with renal vein extension: an unusual case report and review of literature. | journal = Histol Histopathol | volume = 32 | issue = 4 | pages = 361-369 | month = Apr | year = 2017 | doi = 10.14670/HH-11-800 | PMID = 27383188 }}</ref> | |||
*The WHO 2016 classification considers it to be on a spectrum with ''[[cystic nephroma, adult type]]''.<ref name=pmid26935559/> | |||
DDx: | DDx: | ||
*[[Tubulocystic renal cell carcinoma]]. | *[[Cystic nephroma, pediatric type]] - more cystic. | ||
*[[Tubulocystic renal cell carcinoma]] - has high grade nucleoli. | |||
*[[Papillary renal cell carcinoma]] with MEST-like stroma.<ref name=pmid31862520>{{cite journal |authors=Rogala J, Kojima F, Alaghehbandan R, Agaimy A, Martinek P, Ondic O, Ulamec M, Sperga M, Michalova K, Pivovarcikova K, Pitra T, Hora M, Ferak I, Marečková J, Michal M, Hes O |title=Papillary renal cell carcinoma with prominent spindle cell stroma - tumor mimicking mixed epithelial and stromal tumor of the kidney: Clinicopathologic, morphologic, immunohistochemical and molecular genetic analysis of 6 cases |journal=Ann Diagn Pathol |volume=44 |issue= |pages=151441 |date=February 2020 |pmid=31862520 |doi=10.1016/j.anndiagpath.2019.151441 |url=}}</ref> | |||
===Images=== | ===Images=== | ||
<gallery> | <gallery> | ||
Image: | Image: Mixed epithelial stromal tumour of kidney -- low mag.jpg | MEST - low mag. | ||
Image: | Image: Mixed epithelial stromal tumour of kidney -- intermed mag.jpg | MEST - intermed. mag. | ||
Image: Mixed epithelial stromal tumour of kidney -- high mag.jpg | MEST - high mag. | |||
Image: Mixed epithelial stromal tumour of kidney - alt -- high mag.jpg | MEST - high mag. | |||
Image: Mixed epithelial stromal tumour of kidney -- very high mag.jpg | MEST - very high mag. | |||
</gallery> | </gallery> | ||
==IHC== | ==IHC== | ||
Features:<ref name=pmid17454754>{{Cite journal | last1 = Mai | first1 = KT. | last2 = Elkeilani | first2 = A. | last3 = Veinot | first3 = JP. | title = Mixed epithelial and stromal tumour (MEST) of the kidney: report of 14 cases with male and PEComatous variants and proposed histopathogenesis. | journal = Pathology | volume = 39 | issue = 2 | pages = 235-40 | month = Apr | year = 2007 | doi = 10.1080/00313020701230799 | PMID = 17454754 }} | Features:<ref name=pmid17454754>{{Cite journal | last1 = Mai | first1 = KT. | last2 = Elkeilani | first2 = A. | last3 = Veinot | first3 = JP. | title = Mixed epithelial and stromal tumour (MEST) of the kidney: report of 14 cases with male and PEComatous variants and proposed histopathogenesis. | journal = Pathology | volume = 39 | issue = 2 | pages = 235-40 | month = Apr | year = 2007 | doi = 10.1080/00313020701230799 | PMID = 17454754 }}</ref> | ||
</ref> | |||
*ER +ve. | *ER +ve. | ||
*PR +ve. | *PR +ve. | ||
*CD10 +ve. | *CD10 +ve. | ||
Others: | |||
*[[PAX8]] +ve (8 of 8 tumours<ref>{{Cite journal | last1 = Karafin | first1 = M. | last2 = Parwani | first2 = AV. | last3 = Netto | first3 = GJ. | last4 = Illei | first4 = PB. | last5 = Epstein | first5 = JI. | last6 = Ladanyi | first6 = M. | last7 = Argani | first7 = P. | title = Diffuse expression of PAX2 and PAX8 in the cystic epithelium of mixed epithelial stromal tumor, angiomyolipoma with epithelial cysts, and primary renal synovial sarcoma: evidence supporting renal tubular differentiation. | journal = Am J Surg Pathol | volume = 35 | issue = 9 | pages = 1264-73 | month = Sep | year = 2011 | doi = 10.1097/PAS.0b013e31822539a1 | PMID = 21836481 }}</ref>). | |||
*AMACR +ve stromal & epithelial cells - based on a case report.<ref name=pmid27865685>{{Cite journal | last1 = Horsanali | first1 = MO. | last2 = Yilmaz | first2 = Y. | last3 = Ozer | first3 = K. | last4 = Kose | first4 = O. | last5 = Cakalagaoglu | first5 = F. | last6 = Etit | first6 = D. | title = Mixed Epithelial and Stromal Tumor of the Kidney: Two Case Reports of a Rare Benign Kidney Tumor. | journal = Clin Genitourin Cancer | volume = 15 | issue = 1 | pages = e55-e59 | month = Feb | year = 2017 | doi = 10.1016/j.clgc.2016.10.009 | PMID = 27865685 }}</ref> | |||
*Inhibin -ve/+ve (42% +ve in 14 cases<ref name=pmid17414095>{{Cite journal | last1 = Turbiner | first1 = J. | last2 = Amin | first2 = MB. | last3 = Humphrey | first3 = PA. | last4 = Srigley | first4 = JR. | last5 = De Leval | first5 = L. | last6 = Radhakrishnan | first6 = A. | last7 = Oliva | first7 = E. | title = Cystic nephroma and mixed epithelial and stromal tumor of kidney: a detailed clinicopathologic analysis of 34 cases and proposal for renal epithelial and stromal tumor (REST) as a unifying term. | journal = Am J Surg Pathol | volume = 31 | issue = 4 | pages = 489-500 | month = Apr | year = 2007 | doi = 10.1097/PAS.0b013e31802bdd56 | PMID = 17414095 }}</ref>). | |||
*Calretinin +ve (69% +ve in 14 cases<ref name=pmid17414095/>). | |||
==See also== | ==See also== | ||
Latest revision as of 15:50, 3 March 2020
| Mixed epithelial and stromal tumour of the kidney | |
|---|---|
| Diagnosis in short | |
 Mixed epithelial stromal tumour of kidney. H&E stain. | |
|
| |
| Synonyms | mixed epithelial and stromal tumour family (term recommended in WHO 2016 classification) |
|
| |
| LM | cysts lined by simple epithelium with hobnailing, stroma has an ovarian look (basophilic, spindle cells) |
| Subtypes | on a spectrum with cystic nephroma, adult type |
| LM DDx | cystic nephroma, pediatric type, tubulocystic renal cell carcinoma |
| IHC | ER +ve, PR +ve, CD10 +ve |
| Gross | renal mass, predominantly solid |
| Grossing notes | total nephrectomy for tumour grossing, partial nephrectomy grossing |
| Site | kidney - see kidney tumour |
|
| |
| Prevalence | rare |
| Prognosis | benign |
Mixed epithelial and stromal tumour of the kidney, abbreviated MEST, is a rare benign kidney tumour in the WHO classification of renal neoplasia.
In the 2016 World Health Organization classification of renal neoplasia, this tumour was lumped with cystic nephroma, adult type and the recommended term is mixed epithelial and stromal tumour family.[1]
Turbiner et al.[2] came-up with a cheeky term for the the tumour family (renal epithelial and stromal tumour, abbreviated REST) that didn't catch on.
This kidney tumour should not be confused with mixed epithelial-stromal tumour of seminal vesicle.[3]
General
- Rare - approximately 1.5% of renal neoplasms.[4]
- Benign.
- Prevalence: males > females.
Gross
- Solid and cystic mass - predominantly solid.
Microscopic
Features:
- Cysts lined by simple epithelium with hobnailing - key feature.
- Stroma has an ovarian look:
- Blue (basophilic).
- Spindle cells.
Notes:
- Parenchymal elements (e.g. glomueruli, tubules) are not found in the septa between the cysts.
- May extend into the renal vein.[5]
- The WHO 2016 classification considers it to be on a spectrum with cystic nephroma, adult type.[1]
DDx:
- Cystic nephroma, pediatric type - more cystic.
- Tubulocystic renal cell carcinoma - has high grade nucleoli.
- Papillary renal cell carcinoma with MEST-like stroma.[6]
Images
MEST - low mag.
MEST - intermed. mag.
MEST - high mag.
MEST - high mag.
MEST - very high mag.
IHC
Features:[4]
- ER +ve.
- PR +ve.
- CD10 +ve.
Others:
- PAX8 +ve (8 of 8 tumours[7]).
- AMACR +ve stromal & epithelial cells - based on a case report.[8]
- Inhibin -ve/+ve (42% +ve in 14 cases[2]).
- Calretinin +ve (69% +ve in 14 cases[2]).
See also
References
- ↑ 1.0 1.1 Moch, H.; Cubilla, AL.; Humphrey, PA.; Reuter, VE.; Ulbright, TM. (07 2016). "The 2016 WHO Classification of Tumours of the Urinary System and Male Genital Organs-Part A: Renal, Penile, and Testicular Tumours.". Eur Urol 70 (1): 93-105. doi:10.1016/j.eururo.2016.02.029. PMID 26935559.
- ↑ 2.0 2.1 2.2 Turbiner, J.; Amin, MB.; Humphrey, PA.; Srigley, JR.; De Leval, L.; Radhakrishnan, A.; Oliva, E. (Apr 2007). "Cystic nephroma and mixed epithelial and stromal tumor of kidney: a detailed clinicopathologic analysis of 34 cases and proposal for renal epithelial and stromal tumor (REST) as a unifying term.". Am J Surg Pathol 31 (4): 489-500. doi:10.1097/PAS.0b013e31802bdd56. PMID 17414095.
- ↑ Reikie, BA.; Yilmaz, A.; Medlicott, S.; Trpkov, K. (Mar 2015). "Mixed epithelial-stromal tumor (MEST) of seminal vesicle: a proposal for unified nomenclature.". Adv Anat Pathol 22 (2): 113-20. doi:10.1097/PAP.0000000000000057. PMID 25664946.
- ↑ 4.0 4.1 Mai, KT.; Elkeilani, A.; Veinot, JP. (Apr 2007). "Mixed epithelial and stromal tumour (MEST) of the kidney: report of 14 cases with male and PEComatous variants and proposed histopathogenesis.". Pathology 39 (2): 235-40. doi:10.1080/00313020701230799. PMID 17454754.
- ↑ Xie, WL.; Lian, JY.; Li, B.; Tian, XY.; Li, Z. (Apr 2017). "Mixed epithelial and stromal tumor of kidney with renal vein extension: an unusual case report and review of literature.". Histol Histopathol 32 (4): 361-369. doi:10.14670/HH-11-800. PMID 27383188.
- ↑ Rogala J, Kojima F, Alaghehbandan R, Agaimy A, Martinek P, Ondic O, Ulamec M, Sperga M, Michalova K, Pivovarcikova K, Pitra T, Hora M, Ferak I, Marečková J, Michal M, Hes O (February 2020). "Papillary renal cell carcinoma with prominent spindle cell stroma - tumor mimicking mixed epithelial and stromal tumor of the kidney: Clinicopathologic, morphologic, immunohistochemical and molecular genetic analysis of 6 cases". Ann Diagn Pathol 44: 151441. doi:10.1016/j.anndiagpath.2019.151441. PMID 31862520.
- ↑ Karafin, M.; Parwani, AV.; Netto, GJ.; Illei, PB.; Epstein, JI.; Ladanyi, M.; Argani, P. (Sep 2011). "Diffuse expression of PAX2 and PAX8 in the cystic epithelium of mixed epithelial stromal tumor, angiomyolipoma with epithelial cysts, and primary renal synovial sarcoma: evidence supporting renal tubular differentiation.". Am J Surg Pathol 35 (9): 1264-73. doi:10.1097/PAS.0b013e31822539a1. PMID 21836481.
- ↑ Horsanali, MO.; Yilmaz, Y.; Ozer, K.; Kose, O.; Cakalagaoglu, F.; Etit, D. (Feb 2017). "Mixed Epithelial and Stromal Tumor of the Kidney: Two Case Reports of a Rare Benign Kidney Tumor.". Clin Genitourin Cancer 15 (1): e55-e59. doi:10.1016/j.clgc.2016.10.009. PMID 27865685.