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[Carney's triad].

J Cuilleret, A Baril, S Boucheron

    Presse Medicale (Paris, France : 1983)
    |January 21, 1984
    PubMed
    Summary
    This summary is machine-generated.

    Carney's triad involves rare tumors: gastric leiomyoblastoma, paraganglioma, and pulmonary chondroma. Early detection of these associated tumors is crucial due to paraganglioma's severity.

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    Area of Science:

    • Oncology
    • Endocrinology
    • Pathology

    Background:

    • Carney's triad is a rare syndrome characterized by gastric leiomyoblastoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma.
    • This triad predominantly affects young females, often with blood group A, and can present incompletely with only two tumors.
    • The syndrome is linked to neural crest pathology, suggesting a common histogenetic origin.

    Observation:

    • A case report details a young woman with gastric leiomyoblastoma and a subsequent pelvic paraganglioma.
    • The patient later died from metastatic paraganglioma, complicated by malignant hypertension.
    • A review of 16 previously published cases is included.

    Findings:

    • Paragangliomas within Carney's triad are particularly severe and prone to metastasis.

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  • The study highlights the importance of a systematic search for other triad components when one tumor is identified.
  • A histogenetic link to neural crest pathology is proposed for the tumors in Carney's triad.

    • Implications:

    • Early diagnosis and vigilant monitoring are essential for patients with any component of Carney's triad.
    • Understanding the neural crest origin may lead to novel diagnostic or therapeutic strategies.
    • This research underscores the critical need for comprehensive patient evaluation to manage the risks associated with Carney's triad.