The International Journal of Romanian Society of Endocrinology / Registered in 1938

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  • Case Report

    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.