Circulation. 1999;100:e150
(Circulation. 1999;100:e150.)
© 1999 American Heart Association, Inc.
Circulation Electronic Pages |
Carney Complex
Christian Gaissmaier, MD
Ulm, Germany
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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;98:25602566), 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 "Jablonskis
Dictionary of Syndromes and Eponymic Diseases"2 as
Carney syndrome.
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References
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Carney JA. The triad of gastric epithelioid
leiomyosarcoma, functioning extra-adrenal paraganglioma, and
pulmonary chondroma. Cancer. 1979;43:374382.[Medline]
[Order article via Infotrieve]
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Jablonski S. Jablonskis 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 Childrens Hospital,
Boston, Mass
Alan D. Irvine, MRCP
Department of Dermatology Royal Victoria Hospital,
Belfast, Northern Ireland
Anne Hughes, PhD
Department of Medical Genetics Queens University,
Belfast, Northern Ireland
J. Aidan Carney, MD, PhD
Department of Pathology Mayo Clinic,
Rochester, Minn
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Introduction
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We appreciate Dr Gaissmaiers comments, which highlight
a difficulty
created by eponymic nomenclature. The triad of
gastric leiomyosarcoma,
pulmonary chondroma, and extra-adrenal
paraganglioma (OMIM No.
168000
1 ) was in fact described by
J. Aidan
Carney
2 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.
160980
1 ) 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.
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References
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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.
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Carney JA. The triad of gastric epithelioid
leiomyosarcoma, functioning extra-adrenal paraganglioma, and
pulmonary chondroma. Cancer. 1979;43:374382.
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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;98:25602566.[Abstract/Free Full Text]