Unravelling the mystery in a case of persistent ACTH-independent Cushing's syndrome.
BACKGROUND
We present a rare variety of adrenocorticotrophic hormone (ACTH)-independent Cushing's syndrome known as primary pigmented nodular adrenocortical disease (PPNAD).


METHODS
The patient initially underwent unilateral adrenalectomy for what was thought to be a left adrenal adenoma.


RESULTS
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.


CONCLUSIONS
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.
Lim
Ling Choo
LC
Department of Medicine, Alexandra Hospital, Singapore.
Tan
Leonard H C
LH
Rajasoorya
C
C
D000311:Adrenal Glands / Q000000981:diagnostic imaging; D000315:Adrenalectomy; D000308:Adrenocortical Hyperfunction / Q000503:physiopathology; D000324:Adrenocorticotropic Hormone / Q000378:metabolism; D000328:Adult; D015519:Bone Density; D003480:Cushing Syndrome / Q000175:diagnosis* / Q000378:metabolism / Q000503:physiopathology; D005260:Female; D006801:Humans; D014057:Tomography, X-Ray Computed
D000324:Adrenocorticotropic Hormone