48,460
edits
Difference between revisions of "Biphasic hyalinizing psammomatous renal cell carcinoma"
Jump to navigation
Jump to search
Biphasic hyalinizing psammomatous renal cell carcinoma (view source)
Revision as of 17:57, 25 March 2024
, 25 Marchno edit summary
| (7 intermediate revisions by the same user not shown) | |||
| Line 1: | Line 1: | ||
'''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 | | {{ 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== | ==General== | ||
| Line 11: | Line 45: | ||
*#Small cells. | *#Small cells. | ||
*#Large cells. | *#Large cells. | ||
*Hyaline stroma. | |||
*[[Psammoma bodies]]. | *[[Psammoma bodies]]. | ||
DDx: | DDx: | ||
*[[TFEB renal cell carcinoma]]. | *[[TFEB renal cell carcinoma]]. | ||
*[[Papillary renal cell carcinoma]]. | |||
*[[FH-deficient renal cell carcinoma]]. | |||
==IHC== | ==IHC== | ||
Features:<ref name=pmid32217839/> | Features:<ref name=pmid32217839/> | ||
*HNF-1beta +ve | *[[HNF-1beta]] +ve | ||
*PAX8 +ve | *[[PAX8]] +ve | ||
*CK7 +ve | *[[CK7]] +ve | ||
*EMA +ve. | *EMA +ve. | ||
*AMACR +ve. | *AMACR +ve. | ||
| Line 26: | Line 63: | ||
*Melan A -ve. | *Melan A -ve. | ||
*HMB-45 -ve. | *HMB-45 -ve. | ||
*GATA3 -ve. | *[[GATA3]] -ve. | ||
*Chromogranin A -ve. | *Chromogranin A -ve. | ||
*Synaptophysin -ve. | *Synaptophysin -ve. | ||
*FH normal. | *FH normal. | ||
*SDHB 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== | ||