Primary Pigmented Nodular Adrenocortical Dysplasia

Summary

A 29-year-old female patient presented clinical features of Cushing's syndrome. Biochemical tests suggested the presence of an autonomously functioning adrenocortical neoplasm. However, the adrenal glands were normal on MRI. Diagnostic adrenalectomy revealed a slightly enlarged organ studded externally and on multiple cut surfaces by small yellow and brown nodules. Microscopically, the nodules were composed of enlarged cortical cells with eosinophilic cytoplasm and nuclear pleomorphism, without mitotic figures. The morphology was consistent with primary pigmented nodular adrenocortical dysplasia (PPNAD). Additional right adrenalectomy was performed for curative treatment of Cushing's syndrome. The pathogenesis of PPNAD is not yet established, though, a defect on chromosome 16 was recently suggested. Since our patient had a malignant melanoma earlier in her history, the PPNAD may be a manifestation of the Carney complex.