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.[1]

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.

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/.