ESPE Abstracts (2018) 89 P-P3-013

Case of Primary Pigmented Nodular Adrenocortical

Loan Huynh & Huyen Tran


Children’s Hospital 1, Ho Chi Minh, Vietnam


Introduction: Primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of ACTH-independent Cushing’s 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 Cushing’s syndrome. Total bilateral adrenalectomy followed by hormone therapy is the optimal treatment. Assessment of cushing syndrome’s improvement associated with periodic evaluation of CNC should be performed.

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