Difference between revisions of "Biphasic hyalinizing psammomatous renal cell carcinoma"

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'''Biphasic hyalinizing psammomatous renal cell carcinoma''', abbreviated '''BHP RCC''', is a rare type of [[renal cell carcinoma]] with mutations in neurofibromin 2.<ref name=pmid32217839>{{cite journal |vauthors=Argani P, Reuter VE, Eble JN, Vlatkovic L, Yaskiv O, Swanson D, Dickson BC, Antonescu CR, Matoso A, Gagan J, Palsgrove DN |title=Biphasic Hyalinizing Psammomatous Renal Cell Carcinoma (BHP RCC): A Distinctive Neoplasm Associated With Somatic NF2 Mutations |journal=Am J Surg Pathol |volume=44 |issue=7 |pages=901–916 |date=July 2020 |pmid=32217839 |pmc=7350624 |doi=10.1097/PAS.0000000000001467 |url=}}</ref>
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      =
| Width      =
| Caption    =
| Synonyms  =
| Micro      = biphasic cytomorphology (small cells and large cells), hyaline stroma, [[psammoma bodies]]
| Subtypes  =
| LMDDx      = [[TFEB renal cell carcinoma]], [[papillary renal cell carcinoma]], [[FH-deficient renal cell carcinoma]]
| Stains    =
| IHC        = merlin loss of staining, [[HNF-1beta]] +ve, [[PAX8]] +ve, [[CK7]] +ve, EMA +ve, AMACR +ve, WT1 -ve, Melan A -ve, HMB-45 -ve, [[GATA3]] -ve
| EM        =
| Molecular  = neurofibromin 2 mutations
| IF        =
| Gross      =
| Grossing  =
| Staging    =
| Site      =
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence =
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  =
| Other      =
| ClinDDx    =
| Tx        =
}}
'''Biphasic hyalinizing psammomatous renal cell carcinoma''', abbreviated '''BHP RCC''', is a rare type of [[renal cell carcinoma]] with mutations in neurofibromin 2.<ref name=pmid32217839>{{cite journal |authors=Argani P, Reuter VE, Eble JN, Vlatkovic L, Yaskiv O, Swanson D, Dickson BC, Antonescu CR, Matoso A, Gagan J, Palsgrove DN |title=Biphasic Hyalinizing Psammomatous Renal Cell Carcinoma (BHP RCC): A Distinctive Neoplasm Associated With Somatic NF2 Mutations |journal=Am J Surg Pathol |volume=44 |issue=7 |pages=901–916 |date=July 2020 |pmid=32217839 |pmc=7350624 |doi=10.1097/PAS.0000000000001467 |url=}}</ref>
 
It is not recognized as a subtype of RCC by the WHO of 2022.<ref name=pmid35971742/>
 
==General==
*Evolving entity.
*Very rare.
*Prevalence: male > female.
 
==Microscopic==
Features:<ref name=pmid32217839/>
*Biphasic cytomorphology:
*#Small cells.
*#Large cells.
*Hyaline stroma.
*[[Psammoma bodies]].
 
DDx:
*[[TFEB renal cell carcinoma]].
*[[Papillary renal cell carcinoma]].
*[[FH-deficient renal cell carcinoma]].
 
==IHC==
Features:<ref name=pmid32217839/>
*[[HNF-1beta]] +ve
*[[PAX8]] +ve
*[[CK7]] +ve
*EMA +ve.
*AMACR +ve.
*WT1 -ve
*Melan A -ve.
*HMB-45 -ve.
*[[GATA3]] -ve.
*Chromogranin A -ve.
*Synaptophysin -ve.
*FH normal.
*SDHB normal.
 
Others:
*Merlin - loss of staining (12 of 13 cases<ref name=pmid35971742>{{cite journal |authors=Collins K, Hwang M, Antic T, Paintal A, Argani P, Matoso A, Gopinath A, Baskovich B, Mehra R, Williamson SR, Idrees MT, Barletta JA, Anderson WJ, Hirsch MS, Hornick JL, Acosta AM |title=Merlin immunohistochemistry is useful in diagnosis of tumours within the spectrum of biphasic hyalinizing psammomatous renal cell carcinoma |journal=Histopathology |volume=81 |issue=5 |pages=577–586 |date=November 2022 |pmid=35971742 |doi=10.1111/his.14731 |url=}}</ref>).
 
==Molecular==
*Neurofibromin 2 (NF2) gene mutations.<ref name=pmid35971742/>
 
==See also==
*[[Renal cell carcinoma, unclassified]].
*[[Kidney tumours]].


==References==
==References==

Latest revision as of 17:57, 25 March 2024

Biphasic hyalinizing psammomatous renal cell carcinoma
Diagnosis in short
LM biphasic cytomorphology (small cells and large cells), hyaline stroma, psammoma bodies
LM DDx TFEB renal cell carcinoma, papillary renal cell carcinoma, FH-deficient renal cell carcinoma
IHC merlin loss of staining, HNF-1beta +ve, PAX8 +ve, CK7 +ve, EMA +ve, AMACR +ve, WT1 -ve, Melan A -ve, HMB-45 -ve, GATA3 -ve
Molecular neurofibromin 2 mutations

Biphasic hyalinizing psammomatous renal cell carcinoma, abbreviated BHP RCC, is a rare type of renal cell carcinoma with mutations in neurofibromin 2.[1]

It is not recognized as a subtype of RCC by the WHO of 2022.[2]

General

  • Evolving entity.
  • Very rare.
  • Prevalence: male > female.

Microscopic

Features:[1]

  • Biphasic cytomorphology:
    1. Small cells.
    2. Large cells.
  • Hyaline stroma.
  • Psammoma bodies.

DDx:

IHC

Features:[1]

  • HNF-1beta +ve
  • PAX8 +ve
  • CK7 +ve
  • EMA +ve.
  • AMACR +ve.
  • WT1 -ve
  • Melan A -ve.
  • HMB-45 -ve.
  • GATA3 -ve.
  • Chromogranin A -ve.
  • Synaptophysin -ve.
  • FH normal.
  • SDHB normal.

Others:

  • Merlin - loss of staining (12 of 13 cases[2]).

Molecular

  • Neurofibromin 2 (NF2) gene mutations.[2]

See also

References

  1. ↑ 1.0 1.1 1.2 Argani P, Reuter VE, Eble JN, Vlatkovic L, Yaskiv O, Swanson D, Dickson BC, Antonescu CR, Matoso A, Gagan J, Palsgrove DN (July 2020). "Biphasic Hyalinizing Psammomatous Renal Cell Carcinoma (BHP RCC): A Distinctive Neoplasm Associated With Somatic NF2 Mutations". Am J Surg Pathol 44 (7): 901–916. doi:10.1097/PAS.0000000000001467. PMC 7350624. PMID 32217839. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7350624/.
  2. ↑ 2.0 2.1 2.2 Collins K, Hwang M, Antic T, Paintal A, Argani P, Matoso A, Gopinath A, Baskovich B, Mehra R, Williamson SR, Idrees MT, Barletta JA, Anderson WJ, Hirsch MS, Hornick JL, Acosta AM (November 2022). "Merlin immunohistochemistry is useful in diagnosis of tumours within the spectrum of biphasic hyalinizing psammomatous renal cell carcinoma". Histopathology 81 (5): 577–586. doi:10.1111/his.14731. PMID 35971742.
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