Carney's complex

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 new cutaneous marker for Carney's complex. Arch Dermatol.
Psammomatous melanotic schwannoma: an additional component of Carney's complex: report of a case.
Psammomatous melanotic schwannoma arising in the dermis in a patient with Carney's complex. Histopathology.
INTRODUCTION: Superficial angiomyxoma is a rare benign tumour of dermis and subcutis which was first described by Carney and colleagues in 1986 as a cutaneous myxoma of carney's complex. [1] Later, in 1988 Allen et al [2] described as a cutaneous tumour histologically similar to cutaneous myxoma but without associated with carney's complex.
When the lesion is associated with Carney's complex (i.e., Cutaneous myxomas, cardiac myxomas, spotty pigmentation and endocrine over activity), they are often multiple and frequently occur in the eyelid or externalear.
CONCLUSION: A rare case of superficial angiomyxoma of neck without associated carney's complex in a 21 years old male patient.
Detailed cardiac and dermatological evaluation did not show other components of Carney's complex. Isolated eyelid myxoma is an extremely rare condition with a tendency towards local recurrence following incomplete excision.
Carney's complex is the term used if myxomas are associated with spotty pigmentation and endocrine overactivity.3 Ophthalmic manifestations of Carney's complex are eyelid lentigines, conjunctival or caruncular pigmentation and eyelid myxoma.4 Solitary myxomas are frequently noted in skeletal and cardiac muscle, intestine, conjunctiva and subcutaneous tissue.5 Eyelid myxomas are reported to occur in 10% of cases with Carney's complex and are usually associated with cardiac myxomas and isolated eyelid myxomas are very
A detailed cutaneous and cardiac evaluation was within normal limits thus negative for Carney's complex.
(1) Carney's complex, a neuroendocrine-cardiac syndrome is a common cause of familial recurrent cardiac myxomas.
Familial recurrent atrial myxoma: Carney's complex. Clinical Cardiology 2011; 34(2): 83-6