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Ann Thorac Surg. 2011 Nov;92(5):1877-8. doi: 10.1016/j.athoracsur.2011.03.123. Epub 2011 Oct 31.

Cardiac paraganglioma and gastrointestinal stromal tumor: a pediatric case of Carney-Stratakis syndrome.

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Department of Cardiothoracic Surgery, Birmingham Children's Hospital, Birmingham, United Kingdom.


The eponymous Carney triad reported the association of gastric leiomyosarcoma, also known as a gastrointestinal stromal tumor, extra-adrenal paraganglioma and pulmonary chondromata. Subsequently, Carney and Stratakis distinguished the inherited gastrointestinal stromal tumor with paraganglioma syndrome from the classical Carney Triad as an autosomal dominant condition in adult patients. This combination of gastrointestinal stromal tumor and cardiac paragangliomata in a child is almost unique.

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