Endocrine Abstracts

Introduction: Primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of Cushing’s syndrome. Total bilateral adrenalectomy is mostly chosen for treatment because pathological changes usually grow out in both adrenal glands among PPNAD cases. Meanwhile total adrenalectomy may result in life-threatening acute adrenal insufficiency. In this report, two PPNAD patients were examined to check the laterality of hormone secretion from both glands by adrenal venous sampling (AVS) ahead of surgical intervention, with a view to clarify the distribution of adrenal regions which could be spared during surgery.

Case report: Two females, aged 24 and 25 years, showed typical clinical features and laboratory findings of ACTH independent Cushing’s syndrome. Imaging tests showed bilateral adrenal nodules and bilateral radioiodine uptake. Besides, other examinations revealed the history of atrial myxoma or the existence of breast tumor, which were characteristics of Carney complex. Although these findings validated the diagnosis of PPNAD, we investigated hormonal laterality to know whether there would be any way to avoid total adrenalectomy and symptoms due to cortisol deficiency. Cortisol, aldosterone, epinephrine, norepinephrine levels were measured by AVS including ACTH stimulation. The laterality of cortisol concentration varied from 1.3–1.5 times to 1.2–1.6 times, respectively before and after ACTH administration. The predominance of aldosterone and others were ipsilateral in the first case, but contralateral in the second case. The results seemed inadequate to prove the significant laterality of hormone secretion. Taking all these findings into consideration, both patients underwent laparoscopic bilateral complete adrenalectomy. Histopathological analyses diagnosed both as PPNAD and also revealed that some normal steroid synthetase expressions still remained in non-nodular area.

Conclusion: We have shown that AVS gave us meaningful information to elucidate the pathophysiologic mechanism of PPNAD. Further studies are waited to determine the optimum treatment for bilateral functional adrenal tumor.

Declaration of interest: The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research project.

Funding: This research did not receive any specific grant from any funding agency in the public, commercial or not-for-profit sector.