Key result
Sequential bilateral adrenalectomy successfully resolved persistent ACTH-independent Cushing's syndrome caused by primary pigmented nodular adrenocortical disease in a young male with Carney complex.
Case Report (n=1)
No
Highlights the diagnostic challenge of primary pigmented nodular adrenocortical disease (PPNAD) and Carney complex in patients presenting with ACTH-independent Cushing's syndrome.
May support adrenalectomy for refractory PPNAD-related Cushing's; hypothesis-generating from single case report.
INTRODUCTION: We present a rare variety of adrenocorticotrophic hormone (ACTH)-independent Cushing's syndrome known as primary pigmented nodular adrenocortical disease (PPNAD). CLINICAL PICTURE: The patient initially underwent unilateral adrenalectomy for what was thought to be a left adrenal adenoma. OUTCOME: Partial resolution of symptoms and demonstrable persistent hypercortisolism after surgery prompted further evaluation with findings leading to the diagnosis of Carney complex. A review of the adrenal histology was consistent with PPNAD. CONCLUSION: This entity of PPNAD, which has rarely been reported in Asians, forms part of the Carney complex. The diagnosis may not be simple and straightforward, as illustrated in this patient.
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Lim et al. (2006) conducted a case report in Persistent ACTH-independent Cushing's Syndrome (PPNAD / Carney complex) (n=1). Bilateral adrenalectomy was evaluated on Resolution of hypercortisolism. Sequential bilateral adrenalectomy successfully resolved persistent ACTH-independent Cushing's syndrome caused by primary pigmented nodular adrenocortical disease in a young male with Carney complex.
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