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Carney Complex
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Additional studies indicate that primary pigmented nodular adrenocortical disease (PPNAD) is a most notable endocrine tumor associated with CNC (up to 60% of cases), and cardiac myxoma (occurring in 53% of cases) represents an important cause of CNC-related death. After localization of the CNC susceptibility gene region in chromosome 17q24.2 in 1998, and subsequent characterization of the PRKAR1A gene in 2000, molecular mechanisms of CNC have become apparent [5,6].
Genodermatoses
Published in Dimitris Rigopoulos, Alexander C. Katoulis, Hyperpigmentation, 2017
The most common noncutaneous lesions found in CNC are cardiac myxomas (in 20%–40% of the patients), which may appear early in infancy, but the median age at detection is at 20 years. The most common endocrine tumor in CNC is primary pigmented nodular adrenocortical disease (PPNAD), a cause of (adrenocorticotropin-) independent overproduction of cortisol.14
Surgical pathology of the adrenal gland
Published in Demetrius Pertsemlidis, William B. Inabnet III, Michel Gagner, Endocrine Surgery, 2017
In contrast to the enlarged adrenals seen in the hyperplastic conditions discussed above, the adrenal glands are often of grossly normal size in ACTH-independent micronodular hyperplasia. Multiple small nodules, often pigmented, are scattered within an otherwise atrophic cortex. The nodules are composed of large cells with eosinophilic cytoplasm, with or without prominent brown granular pigment. Grossly pigmented cases are referred to as primary pigmented nodular adrenal cortical disease (PPNAD), a subset of which may be associated with the Carney complex [10].
Primary pigmented nodular adrenocortical disease (PPNAD) as an underlying cause of symptoms in a patient presenting with hirsutism and secondary amenorrhea: case report and literature review
Published in Gynecological Endocrinology, 2018
Ewa Cyranska-Chyrek, Dorota Filipowicz, Ewelina Szczepanek-Parulska, Marta Nowaczyk, Urszula Ambroziak, Sadegh Toutounchi, Łukasz Koperski, Tomasz Bednarczuk, Blazej Meczekalski, Marek Ruchała
The state of hypercortisolism may be caused by exogenous glucocorticoid therapy, being the most common cause, or endogenous hypersecretion of cortisol. The latter is caused either by ACTH-dependent in 80% of patients or in the ACTH-independent mechanism, which is found in the remainder [5,6]. Autonomous excess of cortisol secretion by adrenal glands, apart from frequently observed adrenocortical adenomas or carcinomas, in <1% of patients may be caused by primary pigmented nodular adrenocortical disease (PPNAD). In more than 90% of patients, PPNAD is one of the manifestations of Carney complex (CC), which is a genetic condition characterized with autosomal dominant inheritance and high risk of many neoplasms [7]. However, there were only few cases of non-familial PPNAD not associated with CC described in the literature, mainly in children [8–14].