PRIMARY PIGMENTED NODULAR ADRENOCORTICAL DISEASE AS CAUSE OF CUSHING'S SYNDROME ASSOCIATED WITH CARNEY COMPLEX
Autori:
Sažetak
Summary
Summary. We report a 11 year old girl and two 14 year old boys with Cushing’s syndrome due to primary pigmented nodu- lar adrenocortical disease (PPNAD). In these patients, hypercortisolism is a consequence of autonomous cortisol secretion from adrenal glands and is ACTH-independent. Besides PPNAD, the girl had lentigines, spotty pigmentation on her bucal mucosa and lips and she also had schwannoma. One of the reported boys had prolactinoma. Considering this, those two patients fulfill the criteria for Carney complex which is a type of multiple endocrine neoplasia syndromes inherited in an autosomal dominant trait. The other boy had PPNAD but no other obvious signs of Carney complex were noticed. Family study didn’t reveal any clinical or laboratory signs of Carney complex in our patients’ first relatives. All of our patients under- went bilateral adrenalectomy (in one of the boys laparoscopic surgery was performed). Glucocorticoid and mineralo- corticoid substitution has been started. Adrenal glands were macroscopically normal but pathohistological analysis con- firmed the diagnosis of PPNAD.
