ESPE Abstracts (2016) 86 P-P2-78

Primary Pigmented Nodular Adrenocortical Disease (PPNAD) Justifying a Pediatric Case of ACTH-independent Cushing Syndrome (CS)

Liana Capeloa,b, Stefânia Vieiraa,b, Elisa Cordeiro Apolinárioa & Cristiane Kopaceka


aHospital da Criança Santo Antônio, Porto Alegre, Brazil; bUniversidade de Ciências da Saúde de Porto Alegre, Porto Alegre, Brazil


Background: CS is characterized by excess glucocorticoid excess, either endogenous or exogenous. In children younger than 7 years, the adrenal origin is the most common cause. From this age until adulthood predominates Cushing’s disease (central cause). We can divide CS in ACTH-dependent and independent according to laboratory findings. When ACTH-independent, etiologies are related to benign or malignant diseases of the adrenal or McCune Albright syndrome.

Case report: Two-year-old boy with progressive weight gain, acne, axillary odor, hirsutism, and early aggressiveness for about 9 months. At physical exam: full moon facies, facial acne, hirsutism, mild virilization, behavioral changes, tachycardia and hypertension. Initial investigation showed ACTH-independent CS. Despite an adrenal 5 mm lesion finding at CT, PPNAD was suspected. Initial investigation for Carney complex was normal (skin, testis, heart, thyroid, pituitary). Initially left adrenalectomy was performed followed by the removal of the right adrenal after histopathological examination by intraoperative frozen that suggested architectural changes of the adrenal. He received hydrocortisone protocol intraoperatively and was maintained with stress dose, with subsequent gradual reduction. The clinical outcome was favorable, with improvement of acne and tissue infiltration. He was monitored as an outpatient with hydrocortisone dose of 14 mg/m2 per day and fludrocortisone 0.1 mg/day.

Conclusion: PPNAD is a benign disease of the adrenal, with bilateral involvement, which is responsible for <2% of all cases of endogenous CS. In more than 90% of the time, PPNAD is related to the Carney Complex, an autosomal dominant disease that coexists with multiple tumors. The diagnosis of CS is challenging, in the case of a child is even more. Although a rare and benign disease, PPNAD should be remembered as an important diagnosis of ACTH-independent CS.

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