Introduction: Primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of ACTH-independent Cushings syndrome and has characteristic gross and microscopic pathologic findings.
Case presentation: We report a case of PPNAD in a 11-year-old girl. She was admitted to our hospital with a chief complaint of rapid weight gain in 1.5 years. She also had hypertension and the signs of Cushing syndrome. Examination associated with laboratory tests detected hypertension and ACTH- independent cushing syndrome. Images of bilateral nodular adrenal hyperplasia were revealed by abdominal CT Scan. Total bilateral adrenalectomy following by renal hormones therapy was the choice of treatment. After 3 months of surgery, improvement of hypertension, weight gain and cushing syndrmome were noted. In addition, there was normalization of adrenal hormones levels.
Conclusions: Primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of ACTH-independent Cushings syndrome. Total bilateral adrenalectomy followed by hormone therapy is the optimal treatment. Assessment of cushing syndromes improvement associated with periodic evaluation of CNC should be performed.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology