primary pigmented nodular adrenocortical dysplasia , PPNAD (Abkürzung)
carney complex

It may occur as part of the Carney complex, and is sometimes the first sign.

Approximately 7% of all cardiac myxomas are associated with Carney complex.

For patients with the Carney complex, prognosis depends on how often heart, skin, or other tumors recur.

Both types of Carney complex are autosomal dominant.

Epithelioid blue nevi are a cutaneous condition most commonly seen in patients with the Carney complex.

Less commonly, the molecular pathogenesis of Carney complex is a variety of genetic changes at chromosome 2p16.

Carney complex.

Boikos SA, Stratakis CA: Carney complex: the first 20 years.

Although both syndromes were described by J. Aiden Carney, the Carney complex is entirely different.

Boikos SA, Stratakis CA: Carney complex: pathology and molecular genetics.

Other rare childhood cancers include multiple endocrine neoplasia syndromes and Carney complex, skin cancer, chordoma, and cancer of unknown primary site.

Mutation of PRKAR1A leads to the Carney complex, associating multiple endocrine tumors.

Functional null mutations in this gene cause Carney complex (CNC), an autosomal dominant multiple neoplasia syndrome.

The Carney complex is an autosomal dominant syndrome caused by mutations in the PPKAR1A gene, located in chromosome 17.

Ryan MW, Cunningham S, Xiao SY: Maxillary sinus melanoma as the presenting feature of Carney complex.

The encoded protein is a type 1A regulatory subunit of protein kinase A. Inactivating germline mutations of this gene are found in 70% of people with Carney complex.

Stratakis CA, Kirschner LS, Carney JA: Clinical and molecular features of the Carney complex: diagnostic criteria and recommendations for patient evaluation.

Carney complex and its subsets LAMB syndrome and NAME syndrome are autosomal dominant conditions comprising myxomas of the heart and skin, hyperpigmentation of the skin (lentiginosis), and endocrine overactivity.

Myxomas are rare, benign tumors which may be part of an inheritedsyndrome called Carney complex (see Multiple Endocrine Neoplasia Syndromes (MEN) and Carney Complex section below for more information).

Although not officially categorized as multiple endocrine neoplasia syndromes, Von Hippel-Lindau disease and Carney complex are two other autosomal dominant endocrine tumor syndromes with features that overlap the clinical features of the MEN syndromes.