Difference between revisions of "Acquired cystic disease-associated renal cell carcinoma"

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'''Acquired cystic disease-associated renal cell carcinoma''', abbreviated ''ACD-RCC'', is a rare kidney cancer that arises in the context of chronic renal failure.
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Acquired cystic disease-associated renal cell carcinoma - alt -- intermed mag.jpg
| Width      =
| Caption    = Acquired cystic disease-associated renal cell carcinoma. [[H&E stain]].
| Synonyms  =
| Micro      = sieve-like architecture (tubular structures/cribriforming), tumour cells with prominent nucleoli and eosinophilic cytoplasm, oxalate crystals - seen best in [[polarized light]], acquired cystic disease in background, +/-papillary structures - common minor component
| Subtypes  =
| LMDDx      = [[acquired cystic renal disease]], [[papillary renal cell carcinoma]], [[hereditary leiomyomatosis renal cell carcinoma syndrome associated renal cell carcinoma]]
| Stains    =
| IHC        = AMACR +ve, CD10 +ve, pankeratin +ve, CK7 +ve (heterogeneous), EMA -ve
| EM        =
| Molecular  =
| IF        =
| Gross      = kidney with cystic changes, thinned cortex
| Grossing  = [[total nephrectomy for tumour grossing]], [[partial nephrectomy grossing]]
| Staging    = [[kidney cancer staging]]
| Site      = [[kidney]] - see [[kidney tumours]]
| Assdx      = [[acquired cystic disease of the kidney]], [[end-stage renal disease]]
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = rare
| Bloodwork  =
| Rads      = renal mass and cysts
| Endoscopy  =
| Prognosis  =
| Other      =
| ClinDDx    =
| Tx        = nephrectomy
}}
'''Acquired cystic disease-associated renal cell carcinoma''', abbreviated '''ACD-RCC''', is a rare [[kidney cancer]] that arises in the context of [[chronic renal failure]].


It was added to the [[WHO]] classification of renal neoplasia in the [[Vancouver classification|Vancouver modification of 2012/2013]].<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>
==General==
==General==
*Arise in the context of long-standing [[end-stage renal disease]] (ESRD).<ref name=pmid23471757>{{Cite journal  | last1 = Ahn | first1 = S. | last2 = Kwon | first2 = GY. | last3 = Cho | first3 = YM. | last4 = Jun | first4 = SY. | last5 = Choi | first5 = C. | last6 = Kim | first6 = HJ. | last7 = Park | first7 = YW. | last8 = Park | first8 = WS. | last9 = Shim | first9 = JW. | title = Acquired cystic disease-associated renal cell carcinoma: further characterization of the morphologic and immunopathologic features. | journal = Med Mol Morphol | volume =  | issue =  | pages =  | month = Mar | year = 2013 | doi = 10.1007/s00795-013-0028-x | PMID = 23471757 }}</ref>
*Arise in the context of [[acquired cystic disease of the kidney]] which is seen in the context of [[end-stage renal disease]] (ESRD).<ref name=pmid23471757>{{Cite journal  | last1 = Ahn | first1 = S. | last2 = Kwon | first2 = GY. | last3 = Cho | first3 = YM. | last4 = Jun | first4 = SY. | last5 = Choi | first5 = C. | last6 = Kim | first6 = HJ. | last7 = Park | first7 = YW. | last8 = Park | first8 = WS. | last9 = Shim | first9 = JW. | title = Acquired cystic disease-associated renal cell carcinoma: further characterization of the morphologic and immunopathologic features. | journal = Med Mol Morphol | volume =  | issue =  | pages =  | month = Mar | year = 2013 | doi = 10.1007/s00795-013-0028-x | PMID = 23471757 }}</ref>
 
*May have aggressive course - including [[lymph node metastases]] and death.<ref name=pmid16434887>{{Cite journal  | last1 = Tickoo | first1 = SK. | last2 = dePeralta-Venturina | first2 = MN. | last3 = Harik | first3 = LR. | last4 = Worcester | first4 = HD. | last5 = Salama | first5 = ME. | last6 = Young | first6 = AN. | last7 = Moch | first7 = H. | last8 = Amin | first8 = MB. | title = Spectrum of epithelial neoplasms in end-stage renal disease: an experience from 66 tumor-bearing kidneys with emphasis on histologic patterns distinct from those in sporadic adult renal neoplasia. | journal = Am J Surg Pathol | volume = 30 | issue = 2 | pages = 141-53 | month = Feb | year = 2006 | doi =  | PMID = 16434887 }}</ref>


==Gross==
==Gross==
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==Microscopic==
==Microscopic==
Features:
Features:
*Tubular strucutres with cribriforming.
*Fused tubular structures/cribriforming/sieve-like architecture.<ref>{{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 }}
**Hobnail cells.
</ref>
*Oxylate crystals - '''important'''.
**Focal papillary architecture - common.<ref name=pmid16434887>{{Cite journal  | last1 = Tickoo | first1 = SK. | last2 = dePeralta-Venturina | first2 = MN. | last3 = Harik | first3 = LR. | last4 = Worcester | first4 = HD. | last5 = Salama | first5 = ME. | last6 = Young | first6 = AN. | last7 = Moch | first7 = H. | last8 = Amin | first8 = MB. | title = Spectrum of epithelial neoplasms in end-stage renal disease: an experience from 66 tumor-bearing kidneys with emphasis on histologic patterns distinct from those in sporadic adult renal neoplasia. | journal = Am J Surg Pathol | volume = 30 | issue = 2 | pages = 141-53 | month = Feb | year = 2006 | doi =  | PMID = 16434887 }}</ref>
**Tumour cells have prominent nucleoli ([[ISUP nucleolar grade]] 3) and eosinophilic cytoplasm.
*Oxalate crystals - '''important'''.
**Look somewhat like cholesterol clefts.
**Look somewhat like cholesterol clefts.
**Seen easily in [[polarized light]].
*[[Acquired cystic disease of the kidney|Acquired cystic disease]] in background - '''required'''.
**Changes of [[end-stage kidney]] (obsolete glomeruli, thyroidization, interstitial fibrosis).


DDx:
DDx:
*[[Acquired cystic renal disease]].  
*[[Acquired cystic disease of the kidney]].  
*[[Papillary renal cell carcinoma]].
*[[Papillary renal cell carcinoma]].
*[[Hereditary leiomyomatosis renal cell carcinoma syndrome associated renal cell carcinoma]].
===Images===
====Case====
<gallery>
Image: Acquired cystic disease-associated renal cell carcinoma -- very low mag.jpg | ACD-RCC - very low mag.
Image: Acquired cystic disease-associated renal cell carcinoma -- low mag.jpg | ACD-RCC - low mag.
Image: Acquired cystic disease-associated renal cell carcinoma -- intermed mag.jpg | ACD-RCC - intermed. mag.
Image: Acquired cystic disease-associated renal cell carcinoma - alt -- intermed mag.jpg | ACD-RCC - intermed. mag.
Image: Acquired cystic disease-associated renal cell carcinoma -- high mag.jpg | ACD-RCC - high mag.
Image: Acquired cystic disease-associated renal cell carcinoma -- very high mag.jpg | ACD-RCC - very high mag.
Image: Acquired cystic disease-associated renal cell carcinoma -2-- low mag.jpg | ACD-RCC - low mag.
Image: Acquired cystic disease-associated renal cell carcinoma -2-- intermed mag.jpg | ACD-RCC - intermed. mag.
Image: Acquired cystic disease-associated renal cell carcinoma -2- polarized -- intermed mag.jpg | ACD-RCC - intermed. mag.
Image: Acquired cystic disease-associated renal cell carcinoma -2-- high mag.jpg | ACD-RCC - high mag.
Image: Acquired cystic disease-associated renal cell carcinoma -2- polarized -- high mag.jpg | ACD-RCC - high mag.
Image: Acquired cystic disease-associated renal cell carcinoma -2-- very high mag.jpg | ACD-RCC - very high mag.
Image: Acquired cystic disease-associated renal cell carcinoma -2- polarized -- very high mag.jpg | ACD-RCC - very high mag.
Image: Acquired_cystic_disease-associated_renal_cell_carcinoma_-2a--_intermed_mag.gif| ACD-RCC with and without polarized light - intermed. mag.
</gallery>
====www====
*[https://www.auanet.org/education/modules/pathology/kidney-carcinomas/images/acquired_cystic-figureA_Big.jpg ACDARCC (auanet.org)].<ref name=auanet>URL: [https://www.auanet.org/education/modules/pathology/kidney-carcinomas/acquired-cystic.cfm https://www.auanet.org/education/modules/pathology/kidney-carcinomas/acquired-cystic.cfm]. Accessed on: 13 May 2015.</ref>
*[https://www.auanet.org/education/modules/pathology/kidney-carcinomas/images/acquired_cystic-figureB_Big.jpg ACDARCC - sieve-like pattern (auanet.org)].<ref name=auanet/>


==IHC==
==IHC==
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*CD68 -ve.
*CD68 -ve.
*PAX2 -ve.
*PAX2 -ve.
*EMA -ve.<ref name=Ref_Uropath113>{{Ref Uropath|113}}</ref>
*HMWCK -ve.<ref name=Ref_Uropath113>{{Ref Uropath|113}}</ref>
*Vimentin +ve/-ve.<ref name=Ref_Uropath113>{{Ref Uropath|113}}</ref>
*CAM5.2 +ve/-ve.<ref name=Ref_Uropath113>{{Ref Uropath|113}}</ref>


==See also==
==See also==
*[[Renal tumours]].
*[[Renal tumours]].
*[[Acquired cystic renal disease]].
*[[Acquired cystic renal disease]].
*[[End-stage kidney]].


==References==
==References==
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[[Category:Genitourinary pathology]]
[[Category:Genitourinary pathology]]
[[Category:Diagnosis]]
[[Category:Kidney tumours]]

Latest revision as of 23:37, 26 May 2018

Acquired cystic disease-associated renal cell carcinoma
Diagnosis in short

Acquired cystic disease-associated renal cell carcinoma. H&E stain.
LM sieve-like architecture (tubular structures/cribriforming), tumour cells with prominent nucleoli and eosinophilic cytoplasm, oxalate crystals - seen best in polarized light, acquired cystic disease in background, +/-papillary structures - common minor component
LM DDx acquired cystic renal disease, papillary renal cell carcinoma, hereditary leiomyomatosis renal cell carcinoma syndrome associated renal cell carcinoma
IHC AMACR +ve, CD10 +ve, pankeratin +ve, CK7 +ve (heterogeneous), EMA -ve
Gross kidney with cystic changes, thinned cortex
Grossing notes total nephrectomy for tumour grossing, partial nephrectomy grossing
Staging kidney cancer staging
Site kidney - see kidney tumours
Associated Dx acquired cystic disease of the kidney, end-stage renal disease
Prevalence rare
Radiology renal mass and cysts
Treatment nephrectomy

Acquired cystic disease-associated renal cell carcinoma, abbreviated ACD-RCC, is a rare kidney cancer that arises in the context of chronic renal failure.

It was added to the WHO classification of renal neoplasia in the Vancouver modification of 2012/2013.[1]

General

Gross

  • Cysts.

Microscopic

Features:

  • Fused tubular structures/cribriforming/sieve-like architecture.[4]
    • Focal papillary architecture - common.[3]
    • Tumour cells have prominent nucleoli (ISUP nucleolar grade 3) and eosinophilic cytoplasm.
  • Oxalate crystals - important.
  • Acquired cystic disease in background - required.
    • Changes of end-stage kidney (obsolete glomeruli, thyroidization, interstitial fibrosis).

DDx:

Images

Case

  • ACD-RCC - very low mag.

  • ACD-RCC - low mag.

  • ACD-RCC - intermed. mag.

  • ACD-RCC - intermed. mag.

  • ACD-RCC - high mag.

  • ACD-RCC - very high mag.

  • ACD-RCC - low mag.

  • ACD-RCC - intermed. mag.

  • ACD-RCC - intermed. mag.

  • ACD-RCC - high mag.

  • ACD-RCC - high mag.

  • ACD-RCC - very high mag.

  • ACD-RCC - very high mag.

  • ACD-RCC with and without polarized light - intermed. mag.

www

IHC

Features:[2]

  • AMACR +ve.
  • CD10 +ve.
  • Pankeratin +ve.
  • CK7 +ve (heterogeneous).

Others:[2]

  • CD68 -ve.
  • PAX2 -ve.
  • EMA -ve.[6]
  • HMWCK -ve.[6]
  • Vimentin +ve/-ve.[6]
  • CAM5.2 +ve/-ve.[6]

See also

References

  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. ↑ 2.0 2.1 2.2 Ahn, S.; Kwon, GY.; Cho, YM.; Jun, SY.; Choi, C.; Kim, HJ.; Park, YW.; Park, WS. et al. (Mar 2013). "Acquired cystic disease-associated renal cell carcinoma: further characterization of the morphologic and immunopathologic features.". Med Mol Morphol. doi:10.1007/s00795-013-0028-x. PMID 23471757.
  3. ↑ 3.0 3.1 Tickoo, SK.; dePeralta-Venturina, MN.; Harik, LR.; Worcester, HD.; Salama, ME.; Young, AN.; Moch, H.; Amin, MB. (Feb 2006). "Spectrum of epithelial neoplasms in end-stage renal disease: an experience from 66 tumor-bearing kidneys with emphasis on histologic patterns distinct from those in sporadic adult renal neoplasia.". Am J Surg Pathol 30 (2): 141-53. PMID 16434887.
  4. ↑ 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.
  5. ↑ 5.0 5.1 URL: https://www.auanet.org/education/modules/pathology/kidney-carcinomas/acquired-cystic.cfm. Accessed on: 13 May 2015.
  6. ↑ 6.0 6.1 6.2 6.3 Amin, Mahul B.; Eble, John; Grignon, David; Srigley, John. (2013). Urological Pathology (1st ed.). Wolters Kluwer. pp. 113. ISBN 978-0781782814.
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