July - September 2020, Volume 16, Issue 3

Bavadiya G, Roy A, Sarkar KK, Shekhda KM, Chatterjee A, Shah C, Chakrabarty A

Primary Pigmented Nodular Adrenocortical Disease (PPNAD) Presenting as Cushing Syndrome in a Child and Review of Literature

Acta Endo (Buc) 2020, 16 (3): 362-365
doi: 10.4183/aeb.2020.362

Cushing syndrome in the paediatric age group is very difficult to diagnose due to atypical presenting features in children. Primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of ACTH-independent Cushing syndrome in children and it has characteristic gross and microscopic pathologic features. We report a case of PPNAD in a 16-year-old boy who was evaluated in our hospital with chief complaints of poor height velocity and rapid weight gain for 2-3 years before presentation. Proper evaluation showed ACTH-independent Cushing syndrome with normal imaging. Total bilateral adrenalectomy was performed followed by hormones replacement. 6 months after surgery, significant acceleration of height velocity was noticed. Patient also lost body weight and developed secondary sexual characteristics.

Keywords: Cushing syndrome, Primary Pigmented Nodular Adrenocortical Disease, Height velocity

Correspondence: Kalyan Mansukhbhai Shekhda D.N.B. (Medicine), M.R.C.P. (UK), Southend University Hospital NHS Foundation Trust, Diabetes and Endocrinology, Prittlewell chase, Westcliff-on-Sea, SS0 0RY, United Kingdom of Great Britain and Northern Ireland, E-mail: kalokly@gmail.com