Abstract
Objective To discuss the surgical treatment of primary pigmented nodular adrenocortical disease(PPNAD). Methods twenty-four cases of PPNAD were treated in our hospital from January 2005 to December 2017. Clinical data of these patients were reviewed. It included 8 males and 16 females with a mean age of 23 years old (range 14 to 58). 23 cases presented with typical symptoms of Cushing syndrome, 1 case presented with hypertension. Eight cases could be diagnosed with Carney complex. All cases were confirmed as ACTH-independent Cushing syndrome. Adrenal imaging showed bilateral multiple nodules in 11 cases, unilateral multiple nodules in 4 cases, unilateral single mass or nodule in 3 cases, normal adrenals in 6 cases. Results All of the 24 cases received laparoscope unilateral adrenalectomy or laparoscope unilateral mass resection. After the operation, 8 cases underwent secondary operation because of symptom recurrence and the elevated 24-hour urinary free cortisol. Among them, 5 cases received contralateral subtotal adrenalectomy, 3 cases received contralateral total adrenalectomy. Seven cases with a slightly elevated 24-hour urinary free cortisol but a good recovery of Cushing symptoms were followed-up. Nine cases recovered well after the first operation and they didn′t undergo secondary surgical treatment, but 1 of the 9 cases needed glucocorticoid replacement. Conclusions Bilateral adrenalectomy followed with long-term glucocorticoid replacement is the standard treatment of PPNAD. Unilateral adrenalectomy or subtotal adrenalectomy may be suitable for elected patients, but appropriate criteria need to be explored. Key words: Cushing syndrome; Primary pigmented nodular adrenocortical disease(PPNAD); Carney complex
Published Version
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