Carney Complex

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Cardiovasc Surg. 2002 Jun;10(3):264-75.
Carney's syndrome: complex myxomas. Report of four cases and review of the literature.
Edwards A, Bermudez C, Piwonka G, Berr ML, Zamorano J, Larrain E, Franck R, Gonzalez M, Alvarez E, Maiers E.
Centro Cardiovascular, Hospital Clinico Universidad de Chile, Av. Santos Dumont 999, Santiago, Chile.

Cardiac myxomas are rare tumors. They usually appear as a sporadic isolated condition in the left atrium of middle-aged women with no other coincidental pathology. Carney and others have described in young people a special complex group of cardiac myxomas associated to a distinctive complex pathology, giving identity to the "Syndrome Myxoma" or "Carney's Syndrome". Four additional cases of this syndrome, treated from 1977 to 1999 at the Hospital Clinico de la Universidad de Chile are presented here with a comprehensive review of the literature, accumulating 100 cases. The main features of our cases include the presence of malignant non cardiac tumors, a familial trend, follow-up of 23 years and an iterative recurrence in the elder case. To date all patients are tumor free. Reviewing the literature, patients with Carney's Syndrome were younger, with a mean age of 26 years and female predominance (62%). Cardiac myxomas affected the four chambers of the heart: 64% the left atrium; 44% the right atrium; 14% the left ventricle and 12% the right ventricle. They were multiple tumors in 41% and involved more than one chamber in 31%, being synchronous or metachronous. There was a marked familial trend (52%), a high incidence of recurrence (20%), with more than one occurring in half the cases. Extra-cardiac involvement consisted of: 68% pigmented skin lesions, 40% cutaneous myxomas, 37% adrenal cortical disease, 27% myxoid mammary fibroadenoma and 34% male patients with testes tumors. A low percentage had pituitary adenoma, melanotic schwannomas and thyroid disease. The diagnosis is made when two or more of these criteria are present. In agreement with these findings the four chambers of the heart should be examined at surgery for atypical myxoma locations, right atriotomy and combined superior-transseptal approach improve exposure of the cavities, careful screening of the first degree family members should be conducted, and closed short and long term follow up controls are important. Complex myxoma appears as a multi-systemic disorder, occasionally having an ominous prognosis and malignant potentiality, and is still undergoing investigation for better understanding and identification.

J Cardiol. 2003 Feb;41(2):97-102.
Complex myxoma detected by syncope: a case report
Ejima K, Uchida T, Hen Y, Nishio Y, Nomoto F, Uchida Y, Mun J, Satoh T, Fujimori K, Tanino S, Hirosawa K, Agematsu K, Nanaumi M, Sugimoto K, Koizumi J, Ohkado A, Shiikawa A.
Department of Cardiology, Sendai Cardiovascular Center, Hondacho 21-1, Izumi-ku, Sendai 981-3107.

A 23-year-old man was admitted to our hospital for evaluation of syncope and intracardiac masses. Echocardiography revealed three masses in the right ventricle and one in the left ventricle. The largest mass, 4 by 5 cm, occupied the right ventricular outflow tract and prolapsed through the pulmonary valve orifice. Right ventricular systolic pressure was 65 mmHg. A soft mass, 4 by 5 cm, was found on the left subcostal abdominal wall and multiple pigmented spots on the face and trunk. Histological examination of the resected tumors, including the abdominal soft mass, were consistent with myxoma. The combination of multiple cardiac and abdominal wall myxomas and pigmented skin lesions in this young patient is considered to be a diagnostic feature of Carney complex.

Tex Heart Inst J. 2003;30(1):80-2.
Comment in: Tex Heart Inst J. 2004;31(1):106-7.
Familial cardiac myxoma: Carney's complex.
Mahilmaran A, Seshadri M, Nayar PG, Sudarsana G, Abraham KA.
Institute for Cardiac Treatment & Research, Southern Railway Hospital, Ayanavaram, Chennai 600023, India.

We report a case of Carney's complex in a 12-year-old boy who had the characteristic features of multiple cutaneous tumors, pigmentation, and biatrial myxoma. His large right atrial myxoma almost occluded the tricuspid valve and presented a life-threatening emergency. Surgery saved his life, but recurrence of myxoma was noted on follow-up. The familial nature of the condition is highlighted by the case of the patient's 44-year-old mother, who also presented with features of Carney's complex: multiple cutaneous tumors and a tiny, asymptomatic, left atrial myxoma, which was detected during routine echocardiographic screening.

Ann Cardiol Angeiol (Paris). 1992 Apr;41(4):177-83.
Myxoma of the right atrium. Apropos of 10 surgically treated cases
Loire R, Delaye J.
Hopital Cardiologique, Lyon.

In a series of 100 patients with intracardiac myxoma, the lesion was in the right atrium in 10 cases. The authors attempt to define the particular features of right atrial myxoma in terms of clinical aspects, outcome and pathology. The diagnostic difficulty raised by complex clinical pictures (including right ventricular failure, pseudopericardial forms and forms with a predominance of systemic problems) has now been resolved by imaging and, above all echocardiography. Consequences for the right side of the heart may involve the pulmonary artery with myxomatous emboli causing multiple fusiform aneurysms and pulmonary hypertension, or, more rarely, the tricuspid (with need for valve replacement), vena cava (Budd-Chiari syndrome) or atrial septum (right/left shunt). Alongside systemic problems due to interleukin 6, erythrocyte abnormalities are a new feature. Frequent etiological inclusion in a Carney complex explains recurrences and multi-cavity forms. The dominant pathological feature is tumor calcifications, no doubt as a result of the long presymptomatic phase of myxoma.

Arch Mal Coeur Vaiss. 1991 Sep;84(9):1365-8.
Echocardiographic aspects of multiple myxoma in Carney's syndrome
Agostini D, Scanu P, Galateau F, Breut C, Valette B, Mabire JP, Lorier E, Khayat A, Grollier G, Potier JC.
Service de soins intensifs de cardiologie, CHU Cote de Nacre, Caen.

In Carney's syndrome, the association of cardiac myxomas, spotty pigmentation and endocrine over activity, the myxomas are usually multiple and have atypical locations. The authors report a case in which an accurate diagnosis of these multiple myxomas was made by transoesophageal echocardiography, although transthoracic echocardiography had missed the diagnosis.

Am J Ophthalmol. 1991 Jun 15;111(6):699-702.
The Carney complex with ocular signs suggestive of cardiac myxoma.
Kennedy RH, Flanagan JC, Eagle RC Jr, Carney JA.
Department of Ophthalmology, University of Texas-Southwestern Medical Center, Dallas 75235-9057.

We treated a patient who had ophthalmic findings of the Carney complex that led to a search for and the discovery of asymptomatic cardiac myxoma. Substantial morbidity and mortality are associated with the complex because of the occurrence of cardiac myxoma. Facial and eyelid lentigines, conjunctival and caruncle pigmentation and eyelid pigmentation may precede signs or symptoms of cardiac myxoma. A study of the patient's primary relatives disclosed manifestations of the complex transmitted in a manner consistent with mendelian autosomal dominant inheritance.

Arch Mal Coeur Vaiss. 1990 Jan;83(1):121-4.
Association of cutaneous myxoma, recurrent cardiac myxoma and Cushing's syndrome (Carney's complex). Description of a case and review of the literature
Champagnac D, Gayet C, Mornex R, Termet H, Pierrard B, Milon H.
Service de cardiologie, hopital de la Croix-Rousse, Lyon.

The authors report the case of a rare clinical syndrome which has recently been called Carney's complex or "myxomas, spotty pigmentation and endocrine overactivity". Three components of this complex are described: cutaneous myxomas, Cushing's syndrome of unpredictable evolution treated by bilateral adrenalectomy for multiple adrenal adenoma and left atrial myxoma which recurred twice. The authors review the literature and discuss the practical implications of this new syndrome which may be familial. The role of echocardiography, the key investigation for the detection of the myxoma and follow-up of these patients who have a high risk of recurrence, is underlined.

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