Laparoscopic unilateral adrenalectomy in children for isolated primary pigmented nodular adrenocortical disease (PPNAD): case report and literature review.

摘要

Primary pigmented nodular adrenocorti-cal disease (PPNAD) is a pituitary-independent, primary adrenal form of hypercortisolism, characterized by small or normal-sized adrenal glands containing multiple cortical nodules; it is a rare syndrome and its main endocrine manifestation is adrenocorticotropic hormone-independent Cushing's syndrome. ACTH-independent adrenocortical causes of Cushing's syndrome account for up to 15% of all cases of Cushing's syndrome in children older than 7 years. In younger children, ACTH-independent Cushing's syndrome may account for as many as half of all Cushing's syndrome cases, mostly due to adrenal tumors [1]. PPNAD may occur in an isolated form or in association with a multiple neoplasia syndrome, the Carney complex (32% of cases of PPNAD to 90% of cases, in some series).