Carney's complex

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Carney syndrome

An autosomal dominant multiple neoplasia and lentiginosis syndrome, first described as the association of:
(1) primary pigmented nodular adrenocortical disease (PPNAD), a pituitary-independent, primary form of hyperadrenocortisolism;
(2) lentigines, freckles, and blue nevi of skin and mucosae; and
(3) nonendocrine and endocrine tumours, including myxomas of skin, heart, breast, and other sites; tumours of thyroid, ductal adenoma of breast; psammomatous melanotic schwannoma; growth hormone-producing pituitary adenoma; testicular Sertoli-cell tumour; and acromegaly due to somatomammotroph hyperplasia and growth hormone-releasing, hormone-independent adenoma.

Molecular pathology
Most cases (70%) of Carney syndrome are due to an inactivating mutation in PRKAR1A on chromosome 17q23-q24, which is thought to encode a tumour-suppressor gene.

Carney complex (≥ 3 of the following)
Cardiac myxoma
Cutaneous myxoma
Mammary myxoid fibroma 
Spotty mucocutaneous pigmentation
Lentigines—blue, junctional or compound nevi
Primary pigmented nodular adrenocortical disease
Testicular tumour—large cell calcifying Sertoli cell tumours, often bilateral/multicentric
Pituitary hGH-secreting tumour—acromegaly or gigantism
An autosomal dominant [MIM 160980] clinical triad of young adult onset, which consists of spotty mucocutaneous Lentigenes, cutaneous and cardiac—Atrial Myxomas—and Blue nevi, endocrine hyperactivity, multifocal myxoid fibroadenomas of breast, adrenocortical hyperplasia and calcifying Sertoli cell tumours of testes
Segen's Medical Dictionary. © 2012 Farlex, Inc. All rights reserved.

Carney's complex

Cardiology A variable clinical complex of mucocutaneous, visceral, and endocrine disorders, defined by ≥ 2 of the following
Carney's complex
Cardiac myxoma
Cutaneous myxoma
Mammary myxoid fibroma
Spotty mucocutaneous pigmentation
Lentigines, blue, junctional or compound nevi
Primary pigmented nodular adrenocortical disease
testicular tumor
large cell calcifying Sertoli cell tumors, often bilateral/multicentric
Pituitary hGH-secreting tumor
–Acromegaly or gigantism (MCP 1986; 61:165)
McGraw-Hill Concise Dictionary of Modern Medicine. © 2002 by The McGraw-Hill Companies, Inc.
References in periodicals archive ?
Psammomatous melanotic schwannoma: a distinctive, heritable tumor with special associations, including cardiac myxoma and the Cushing syndrome.
The DDx of cardiac involvement in ECD is a cardiac myxoma. Pulmonary involvement associated with ECD occurs in 43% of patients.
As observed, acute heart failure may be the first clinical manifestation of a giant cardiac myxoma in a patient with normal coronary arteries; therefore, timely echocardiographic evaluation and surgical removal of the mass is recommended to prevent dangerous complications.
Cardiac myxoma is the most frequently reported primary cardiac tumor, but hematologic disease, in rare cases, has been known to orginate in the heart [4].
The final diagnosis was ventricular cardiac myxoma. Postoperative course was uneventful, and the patient was discharged home well; as of writing, he has experienced complete resolution of prior symptoms and subsequent echocardiograms have been normal, without evidence of recurrence.
Primary cardiac myxoma (CM) is the most common type of primary cardiac tumor with an estimated incidence around 0.5-1.0 per million cases per year [1].
Furthermore, the PPAR/miRNA axis was shown to be a crucial regulator of cardiac myxoma (CM), a predominant cardiac tumour especially in the young [94].
Cardiac myxoma is the most common cause of tumour embolism to the brain in addition to being a thrombotic source and trigger for atrial fibrillation.
Metastatic cardiac myxoma. Ann Thorac Surg 2001; 72:623-5.
Cardiac myxoma is the most frequent tumor of the heart [1].
Symptoms from a cardiac myxoma are more pronounced when the myxomas are left-sided, racemosus, and over 5 cm in diameter [6].
* We reported a large left atrial cardiac myxoma and paradoxical pulmonary emboli to the upper lobe of the left lung from a patent large foramen ovale subsequently.