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Circulation. 1999;100:e150

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(Circulation. 1999;100:e150.)
© 1999 American Heart Association, Inc.


Circulation Electronic Pages

Carney Complex

Christian Gaissmaier, MD

Ulm, Germany


*    Introduction
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*Introduction
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To the Editor:

In their interesting article "Identification of a novel genetic locus for familial cardiac myxomas and Carney Complex "(Circulation. 1998;98:2560–2566), Casey et al report on the genetics of cardiac myxomas in patients with Carney complex. Some of your readers, including myself, may ask why the syndrome has been termed Carney "complex" (rather than "Carney syndrome"). A search revealed that J.A. Carney already published a syndrome that today bears his name: the triad of pulmonary chondroma, gastric epithelioid leiomyosarcoma, and functioning extra-adrenal paraganglioma1 is listed in "Jablonski’s Dictionary of Syndromes and Eponymic Diseases"2 as Carney syndrome.


*    References
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  1. Carney JA. The triad of gastric epithelioid leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma. Cancer. 1979;43:374–382.[Medline] [Order article via Infotrieve]
  2. Jablonski S. Jablonski’s Dictionary of Syndromes and Eponymic Diseases. 2nd ed. Melbourne, Fla: Krieger Publishing Company; 1991:101.

Response

Craig T. Basson, MD, PhD; Mairead Casey, BA; Caroline Mah, BA

Cardiology Division, Department of Medicine, Weill Medical College of Cornell University, New York, NY

Andrew D. Merliss, MD

MeritCare Heart Services Fargo, ND

Lawrence S. Kirschner, MD, PhD; Susan E. Taymans, PhD; Constantine A. Stratakis, MD, PhD

National Institute of Child Health and Development, Bethesda, Md

Alfred E. Denio, MD

Center for Arthritis and Rheumatic Disease Virginia Beach, Va

Bruce Korf, MD, PhD

Department of Genetics Children’s Hospital, Boston, Mass

Alan D. Irvine, MRCP

Department of Dermatology Royal Victoria Hospital, Belfast, Northern Ireland

Anne Hughes, PhD

Department of Medical Genetics Queen’s University, Belfast, Northern Ireland

J. Aidan Carney, MD, PhD

Department of Pathology Mayo Clinic, Rochester, Minn


*    Introduction 
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*Introduction 
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We appreciate Dr Gaissmaier’s comments, which highlight a difficulty created by eponymic nomenclature. The triad of gastric leiomyosarcoma, pulmonary chondroma, and extra-adrenal paraganglioma (OMIM No. 1680001 ) was in fact described by J. Aidan Carney2 and is referred to as Carney syndrome. This triad, though, is nonfamilial and is distinct from the familial disorder of cardiac myxomas, spotty pigmentation, and endocrinopathy (OMIM No. 1609801 ) discussed in our report.3 The latter disorder, also described by Dr Carney and bearing his name, is therefore referred to as Carney complex to distinguish the 2 entities.


*    References 
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up arrowIntroduction 
*References 
 

  1. Online Mendelian Inheritance in Man, OMIM (TM). Center for Medical Genetics, Johns Hopkins University (Baltimore, Md) and National Center for Biotechnology Information, National Library of Medicine (Bethesda, Md). World Wide Web URL: http://www.ncbi.nlm.nih.gov/omim/ 1997.
  2. Carney JA. The triad of gastric epithelioid leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma. Cancer. 1979;43:374–382.
  3. Casey M, Mah C, Merliss AD, Kirschner LS, Taymans SE, Denio AE, Korf B, Irvine AD, Hughes A, Carney JA, Stratakis CA, Basson CT. Identification of a novel genetic locus for familial cardiac myxomas and Carney complex. Circulation. 1998;98:2560–2566.[Abstract/Free Full Text]




This Article
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