Primary pigmented nodular adrenocortical disease: a case report in a 7-year-old girl

J Pediatr Endocrinol Metab. 2011;24(3-4):197-202.


Primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of Cushing syndrome in children, often occurring in association with Carney complex. We report a case of Cushing syndrome due to isolated non-familial PPNAD. The child presented with typical clinical characteristics, growth retardation and obesity. Liddle's test was positive but micronodular appearance was not evident on CT scan and MRI; selective venous sampling revealed higher cortisol concentrations in the right adrenal vein. The patient underwent a laparoscopic right adrenalectomy. Postoperatively, hypercortisolism signs disappeared but after the second year a slight increase in urinary cortisol was noted and the patient developed osteopenia. Although significant catch-up growth occurred postoperatively, height did not normalize over the next 2 years. When she entered puberty, treatment with a luteinizing-hormone-releasing hormone agonist was initiated and growth hormone was added. Almost 5 years later a left adrenalectomy was also performed. Thereafter, complete disease remission was observed, the patient's growth accelerated and her osteopenia reversed.

Publication types

  • Case Reports

MeSH terms

  • Adrenal Cortex Diseases / blood
  • Adrenal Cortex Diseases / pathology*
  • Adrenal Cortex Diseases / therapy
  • Adrenalectomy
  • Bone Diseases, Metabolic / drug therapy
  • Child
  • Cushing Syndrome / blood
  • Cushing Syndrome / pathology*
  • Cushing Syndrome / therapy
  • Female
  • Gonadotropin-Releasing Hormone / agonists
  • Growth Hormone / therapeutic use
  • Hormone Replacement Therapy
  • Humans
  • Hydrocortisone / blood
  • Hyperpigmentation / pathology
  • Remission Induction


  • Gonadotropin-Releasing Hormone
  • Growth Hormone
  • Hydrocortisone