[Primary pigmented nodular adrenocortical dysplasia (PPNAD) within the scope of Carney complex as the etiology of Cushing syndrome].

Abstract

BACKGROUND:

Primary pigmented adrenocortical dysplasia (PPNAD) represents a rare disorder of the adrenal glands and frequently occurs in patients with the so-called Carney complex. Carney complex is an autosomal dominant neoplasia syndrome including skin and mucosal lentigines, myxomas, and PPNAD.

CASE REPORT:

A 37-year-old woman suffered from several episodes of weight gain/depression and weight loss/mania indicating cyclic hypercortisolism. Finally, she developed a full-blown Cushing's syndrome (CS) treated by bilateral adrenalectomy. She had PPNAD in the setting of Carney complex.

CONCLUSION:

PPNAD may lead to different clinical manifestations: 1. subclinical hypercortisolism, 2. intermittent hypercortisolemia, and 3. full-blown CS. It can be diagnosed with the 6-day Liddle test that typically shows a paradoxical stimulation of cortisol secretion after dexamethasone administration. The treatment of choice for PPNAD is bilateral adrenalectomy in order to prevent devastating long-term effects of hypercortisolism.

• Authors: C A Koch, S R Bornstein, G P Chrousos, C A Stratakis
• Journal: Medizinische Klinik (Munich, Germany : 1983) (Med Klin (Munich)) Vol. 95 Issue 4 Pg. 224-30 (Apr 15 2000) ISSN: 0723-5003 [Print] Germany
• Vernacular Title: Primäre pigmentierte noduläre adrenokortikale Dysplasie (PPNAD) im Rahmen des Carney-Komplexes als Ursache eines Cushing-Syndroms.
• PMID: 10808306 (Publication Type: Case Reports, English Abstract, Journal Article)

Topics

• Adrenal Cortex (pathology)
• Adrenal Hyperplasia, Congenital (diagnosis, genetics)
• Adult
• Chromosome Aberrations (genetics)
• Chromosome Disorders
• Cushing Syndrome (diagnosis, genetics)
• Diagnosis, Differential
• Female
• Genes, Dominant
• Heart Neoplasms (diagnosis, genetics)
• Humans
• Myxoma (diagnosis, genetics)
• Neoplastic Syndromes, Hereditary (diagnosis, genetics)
• Skin Neoplasms (diagnosis, genetics)