Large cell calcifying Sertoli cell tumour: a contemporary multi-institutional case series highlighting the diagnostic utility of PRKAR1A immunohistochemistry

William J. Anderson, Jennifer B. Gordetsky, Muhammad T. Idrees, Khaleel I. Al-Obaidy, Chia Sui Kao, Kristine M. Cornejo, Sara E. Wobker, John C. Cheville, Sara O. Vargas, Christopher D.M. Fletcher, Michelle S. Hirsch, Andrés M. Acosta

Research output: Contribution to journalArticlepeer-review

Abstract

Aims: Large cell calcifying Sertoli cell tumour (LCCSCT) is a rare testicular sex cord-stromal tumour that primarily affects young patients and is associated with Carney complex. We sought to characterise the clinicopathological features of a series of LCCSCT and evaluate the diagnostic utility of PRKAR1A immunohistochemistry (IHC). Methods and results: The LCCSCT cohort (n = 15) had a median age of 16 years (range = 2–30 years). Four patients were known to have Carney complex. PRKAR1A IHC was performed in each case. For comparison, PRKAR1A IHC was also assessed in other sex cord-stromal tumours, including Sertoli cell tumour, not otherwise specified (SCT, NOS; n = 10), intratubular large cell hyalinising Sertoli cell tumour (n = 1) and Leydig cell tumour (n = 23). Loss of cytoplasmic PRKAR1A expression was observed in all but one LCCSCT (14 of 15; 93%). PRKAR1A expression was retained in all SCTs, NOS (10 of 10; 100%), the majority of Leydig cell tumours (22 of 23; 96%) and an intratubular large cell hyalinising Sertoli cell tumour (1 of 1; 100%). One Leydig cell tumour showed equivocal staining (multifocal weak expression). Conclusions: Overall, PRKAR1A loss is both sensitive (93%) and highly specific (97%) for the diagnosis of LCCSCT. PRKAR1A loss may aid its diagnosis, particularly in sporadic cases and those that are the first presentation of Carney complex.

Original languageEnglish (US)
Pages (from-to)677-685
Number of pages9
JournalHistopathology
Volume80
Issue number4
DOIs
StatePublished - Mar 2022

Keywords

  • Carney complex
  • PRKAR1A
  • Sertoli
  • immunohistochemistry
  • testis

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology

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