Abstract
Association of Gushing's syndrome caused by primary adrenocortical nodular dysplasia (PAND) with atrial myxoma was recently reported by Schweizer-Cagianut, Salomon and Hedinger in a Swiss family. We have recently studied a female who presented at age 13 with clinical signs of Gushing's syndrome. Serum cortisol was markedly elevated, and there was no suppression with low and high doses of dexamethasone. Arteriogram indicated an enlarged left adrenal gland. On surgical removal of the left adrenal, PAND was found. After a one year remission Cushing's syndrome recurred, and the other adrenal was excised, also revealing PAND. Three years later a systolic ejection murmur with a mid-systolic click was detected. Echocardiogram revealed a large right atrial mass. Serum hyaluronic acid level was 30.8 μg/ml (normal 0-5). Two myxomas, one attached to the RA septum and the other to the septal leaflet of the tricuspid valve, were removed, with recovery of the patient. A genetic basis for the association of PAND with atrial myxoma is postulated. Determination of serum hyaluronic acid levels is helpful in the diagnosis of atrial myxomas.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Fisher, E., Matalon, R., Manaligod, J. et al. 450 ASSOCIATION OF CUSHING'S SYNDROME CAUSED BY PRIMARY ADRENOCORTICAL NODULAR DYSPLASIA WITH ATRIAL MYXOMA. Pediatr Res 19, 185 (1985). https://doi.org/10.1203/00006450-198504000-00480
Issue Date:
DOI: https://doi.org/10.1203/00006450-198504000-00480