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April 30, 2026Cancer Genetics

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Population

A 33-year-old female patient with Carney complex features and a novel PRKAR1A variant

Design

Case report

Key result

A novel variant in the PRKAR1A gene was identified in a 33-year-old female patient presenting with Carney complex and malignant melanocytic psammomatous schwannoma.

Authors

MAMaría José Vázquez AresCRCamila RolónGMGraciela Mercado

Discussion

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Overview

Alerts clinicians to PRKAR1A variants in Carney complex with schwannomas; extends spectrum but leaves causality open.

Key Points

  • This report aims to describe a case of malignant psammomatous melanotic schwannoma in the context of Carney complex and its genetic underpinnings.
  • Case presentation of a 33-year-old female patient
  • Investigation of clinical signs including cutaneous and mucosal lentiginosis and blue nevus
  • Genetic analysis identifying a novel variant in the PRKAR1A gene.
  • Patient presented multiple tumor types including cutaneous myxoma and malignant melanocytic schwannoma.
  • Identification of a novel PRKAR1A gene variant associated with severe manifestations of Carney complex.
  • Penetrance of Carney complex attributed to PRKAR1A mutations is nearly 100%.

Study Design

Type

Case Report (n=1)

Structured PICO

P
Population
33-year-old female patient with Carney complex, presenting with cutaneous and mucosal lentiginosis, blue nevus, malignant melanocytic psammomatous schwannoma, cutaneous myxoma and a novel variant in the PRKAR1A gene

Reports a novel PRKAR1A gene variant in a patient with Carney complex and malignant psammomatous melanotic schwannoma.

Cite This Study

Ares et al. (2026) conducted a case report in Carney complex (n=1). A novel variant in the PRKAR1A gene was identified in a 33-year-old female patient presenting with Carney complex and malignant melanocytic psammomatous schwannoma.

synapsesocial.com/papers/6a025df493868c48f309378ehttps://doi.org/10.1016/j.cancergen.2026.04.005
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