Carney Complex

Autosomal dominant syndrome characterized by cardiac and cutaneous MYXOMAS; LENTIGINOSIS (spotty pigmentation of the skin), and endocrinopathy and its associated endocrine tumors. The cardiac myxomas may lead to SUDDEN CARDIAC DEATH and other complications in Carney complex patients. The gene coding for the PRKAR1A protein is one of the causative genetic loci (type 1). A second locus is at chromosome 2p16 (type 2).
Also Known As:
Carney Complex, Type 1; Carney Complex, Type I; Carney Myxoma Endocrine Complex; Complex, Carney; Complex, Carney Myxoma-Endocrine; LAMB Syndromes; Myxoma-Endocrine Complex, Carney; NAME Syndromes; Syndrome, Carney; Syndrome, LAMB; Syndrome, NAME; Syndromes, LAMB; Syndromes, NAME; Carney Complex, Type 2; Carney Complex, Type II; Carney Myxoma-Endocrine Complex; Carney Syndrome; LAMB Syndrome; Myxoma, Spotty Pigmentation, and Endocrine Overactivity; NAME Syndrome
Networked: 141 relevant articles (0 outcomes, 8 trials/studies)

Disease Context: Research Results

Related Diseases

1. Acromegaly
2. Pituitary Neoplasms (Pituitary Adenoma)
3. Gigantism
4. Multiple Endocrine Neoplasia (Multiple Endocrine Adenomatosis)
5. Neurilemmoma (Schwannoma)


1. Stratakis, Constantine A: 35 articles (02/2016 - 06/2002)
2. Kirschner, Lawrence S: 13 articles (03/2014 - 10/2002)
3. Bertherat, Jérôme: 9 articles (11/2015 - 12/2002)
4. Stratakis, C A: 9 articles (11/2005 - 03/2000)
5. Carney, J Aidan: 7 articles (09/2014 - 12/2002)
6. Groussin, Lionel: 7 articles (01/2011 - 12/2002)
7. Bertagna, Xavier: 6 articles (11/2015 - 12/2002)
8. Horvath, Anelia: 6 articles (03/2012 - 05/2006)
9. Nesterova, Maria: 6 articles (03/2012 - 01/2008)
10. Bourdeau, Isabelle: 6 articles (06/2009 - 06/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Carney Complex:
1. Cyclic AMP-Dependent Protein Kinases (cAMP-Dependent Protein Kinase)IBA
2. Growth Hormone (Somatotropin)IBA
3. aryl hydrocarbon receptor-interacting proteinIBA
01/01/2011 - "A genetic defect is evident in some cases; known genetic changes include: multiple endocrine neoplasia type 1 (MEN1); Carney complex; McCune-Albright syndrome; and, more recently identified, aryl hydrocarbon receptor-interacting protein (AIP). "
01/01/2011 - "To date, the number of molecular genetic factors unequivocally linked to pituitary tumours can be counted on the fingers of one hand: (1) GNAS1 activation in acromegaly; (2) the MENIN and p27Kip1 (CDKN1B) mutations associated with multiple endocrine neoplasia type 1; (3) mutations of PRKA1RA with loss of 17q22-24 in Carney complex, and (4) aryl hydrocarbon receptor interacting protein gene mutations in 15% of familial isolated pituitary adenomas and 50% of familial isolated acromegaly. "
03/01/2008 - "GNAS1 activation and the mutations associated with multiple endocrine neoplasia type 1 and Carney complex, aryl hydrocarbon receptor interacting protein gene mutations, and a narrowing region of chromosome 11q13 in familial isolated acromegaly together account for such a small proportion of pituitary adenomas that the pituitary adenoma pathogenic epiphany is surely yet to come."
01/01/2014 - "The genetic alterations responsible for the formation of familial pituitary syndromes include the MEN1 gene, responsible for about 80% of MEN1 cases, the regulatory subunit of the protein kinase A, PRKAR1A, responsible for about 70% of Carney complex cases, and AIP, the gene coding the aryl hydrocarbon receptor interacting protein, responsible for about 20% of FIPA cases. "
02/01/2016 - "Prior studies have identified gigantism as a feature of a number of monogenic disorders, including mutations in the aryl hydrocarbon receptor interacting protein gene, multiple endocrine neoplasia types 1 and 4, McCune Albright syndrome, Carney complex, and the paraganglioma, pheochromocytoma, and pituitary adenoma association because of succinate dehydrogenase defects. "
4. Hydrocortisone (Cortisol)FDA LinkGeneric
04/10/2011 - "Both Carney complex and isolated primary pigmented nodular adrenocortical disease are caused by germline PRKAR1A mutations; somatic mutations of this gene that regulates cAMP-dependent protein kinase are also found in cortisol-producing adenomas, and abnormalities of PKA are present in most cases of massive macronodular adrenocortical disease. "
01/01/2006 - "Carney complex (CNC) is a unique multiple endocrine neoplasia syndrome (MIM 160980) which is characterized by unusual biochemical features (chronic hypersomatotropinemia and paradoxical responses of cortisol production to glucocorticoids) and multi-tissue involvement. "
12/01/2014 - "Recent studies have identified L206R mutations in the alpha catalytic subunit of protein kinase A (PRKACA) in cortisol-producing adrenocortical adenomas and amplification of the beta catalytic subunit of protein kinase A PRKACB in acromegaly associated with Carney complex. "
06/01/2002 - "Cortisol-producing hyperplasia of the adrenal glands is caused by two distinct syndromes, both of which have been directly or indirectly associated with protein kinase A signaling: (i) primary pigmented nodular adrenocortical disease (PPNAD) (a micronodular form of bilateral adrenal hyperplasia), either isolated (rarely) or in the context of Carney complex, is caused (in most cases) by mutations of the PRKAR1A gene; and (ii) ACTH-independent macronodular adrenal hyperplasia (AIMAH), or massive macronodular adrenal disease (MMAD), has been associated with aberrant (ectopic) expression, and presumably regulation, of various G protein-coupled receptors. "
5. Succinate Dehydrogenase (Fumarate Reductase)IBA
6. ProlactinIBA
7. Hecht syndromeIBA
8. Myosins (Myosin)IBA
9. Adrenocorticotropic Hormone (ACTH)FDA Link
10. Pendred syndromeIBA

Therapies and Procedures

1. Adrenalectomy
2. Thoracotomy
3. Epidural Analgesia
4. Chinese Traditional Medicine (Traditional Chinese Medicine)
5. Lasers (Laser)