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.
- Copyright © 1999 by American Heart Association
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. 168000R1 ) was in fact described by J. Aidan CarneyR2 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. 160980R1 ) discussed in our report.R3 The latter disorder, also described by Dr Carney and bearing his name, is therefore referred to as Carney complex to distinguish the 2 entities.
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.
Carney JA. The triad of gastric epithelioid leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma. Cancer. 1979;43:374–382.
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.