Sporadic superficial angiomyxomas demonstrate loss of PRKAR1A expression

Farhaan Hafeez, Andrew C. Krakowski, Christine G. Lian, Rosalynn M. Nazarian, Joseph J. Maleszewski

Research output: Contribution to journalArticlepeer-review


Superficial angiomyxomas are cutaneous mesenchymal tumours that typically present clinically as slow-growing, solitary, asymptomatic nodules that can occur at any age. Histopathologically, these dermal and subcutaneous tumours are characterized by abundant myxoid stroma, numerous thin-walled and often arbourising blood vessels, and spindled to stellate fibroblast-like cells. While usually sporadic, superficial angiomyxomas can occasionally be associated with Carney complex (CNC), an autosomal dominant disorder characterized by inactivating germline mutations in the 1-alpha regulatory subunit of protein kinase A (PRKAR1A) and various clinical manifestations, including cardiac myxomas, facial lentigines, epithelioid blue naevi, endocrinopathies and psammomatous melanotic schwannomas. In this study, we sought to characterize the presence or absence of PRKAR1A expression by immunohistochemistry (IHC) in sporadic superficial angiomyxomas based on our observations in an index case. In total, PRKAR1A immunohistochemical expression was determined in 15 sporadic superficial angiomyxoma cases retrieved from the surgical pathology archives. IHC demonstrated that the lesional cells in 12 cases (80%) were non-reactive to antibodies against PRKAR1A. This study provides evidence in support of a role for PRKAR1A in the development of clinically non-syndromic superficial angiomyxomas. Together with previous studies, this report demonstrates that PRKAR1A may play an important role in the development of a variety of myxomatous mesenchymal tumours.

Original languageEnglish (US)
Pages (from-to)1001-1003
Number of pages3
Issue number6
StatePublished - May 2022


  • Carney complex
  • cutaneous myxoma
  • dermatopathology
  • soft tissue pathology
  • superficial angiomyxoma

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology


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