An unusual variant of Cushing syndrome.
OBJECTIVE
To discuss the initial clinical manifestations of primary pigmented nodular adrenocortical disease.


METHODS
We present a case report of a 4-year-old boy who had the classic clinical features of Cushing syndrome. Results of hormonal investigations are reviewed, and histopathologic findings are illustrated.


RESULTS
Investigations revealed adrenocorticotropic hormone (corticotropin)-independent Cushing syndrome. Findings on magnetic resonance imaging of the pituitary gland and abdomen were within normal limits. The patient underwent bilateral adrenalectomy. The histopathologic features were consistent with primary pigmented nodular adrenocortical disease.


CONCLUSIONS
Primary pigmented nodular adrenocortical disease should be suspected in patients with corticotropin-independent Cushing syndrome who have normal findings on adrenal imaging.
Ganesh
Hosahithlu K
HK
Department of Endocrinology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India.
George
Joe
J
Vimal
Mavila V
MV
Bandgar
Tushar
T
Menon
Padma S
PS
Shah
Nalini S
NS
D000303:Adrenal Cortex Diseases / Q000175:diagnosis* / Q000473:pathology; D000324:Adrenocorticotropic Hormone / Q000378:metabolism; D002675:Child, Preschool; D003480:Cushing Syndrome / Q000150:complications* / Q000175:diagnosis / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D010902:Pituitary Gland / Q000473:pathology
D000324:Adrenocorticotropic Hormone