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Carney triad.

J Aidan Carney1

  • 1Department of Laboratory Medicine and Pathology and College of Medicine, Mayo Clinic, Rochester, MN 55905, USA. Carney.Aidan@mayo.edu

Frontiers of Hormone Research
|May 9, 2013
PubMed
Summary
This summary is machine-generated.

Carney triad is a rare genetic disorder causing tumors in the stomach, lungs, and adrenal glands. Gastric tumors are malignant, while others are benign, impacting patient prognosis.

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Area of Science:

  • Endocrinology
  • Oncology
  • Genetics

Background:

  • Carney triad is a rare multitumoral syndrome of unknown etiology.
  • First described in 1977, it affects the stomach, lungs, paraganglionic system, adrenal cortex, and esophagus.
  • The disorder primarily affects young women and is typically not familial.

Purpose of the Study:

  • To summarize the clinical characteristics and outcomes of Carney triad.
  • To highlight the variable expression and organ involvement in affected patients.
  • To discuss the prognostic implications of gastric sarcoma metastases.

Main Methods:

  • Review of existing literature and case reports.
  • Analysis of clinical data from approximately 150 identified cases.
  • Long-term follow-up data analysis from a cohort described in 1999.

Main Results:

  • Multifocal tumors develop in affected organs, with most patients having tumors in two organs.
  • Gastric tumors are malignant and prone to metastasis, while lung, adrenal, and esophageal tumors are benign.
  • Long-term follow-up indicates a chronic, indolent condition, with outcomes dependent on gastric sarcoma metastasis.

Conclusions:

  • Carney triad is a complex syndrome with variable organ involvement and tumor behavior.
  • Gastric sarcoma behavior significantly influences patient prognosis.
  • Understanding the syndrome's characteristics is crucial for patient management and long-term care.