A Case of Carney Complex with Multiple Spinal Fractures / 대한내과학회지

ABSTRACT

Carney complex is an autosomal dominant syndrome characterized by multiple neoplasias, including myxomas and endocrine tumors, spotty pigmentation, and schwannomas. Primary pigmented nodular adrenal dysplasia (PPNAD) is a cause of Cushing syndrome, independently of adrenocorticotropic hormone. A 21-year-old man was referred for evaluation with multiple spinal compression fractures and adenomas affecting both adrenal glands. Plasma and urinary cortisol levels were elevated and were not suppressed by the administration of a high dose of dexamethasone. The patient was diagnosed with Carney complex manifesting as Cushing syndrome due to PPNAD with multiple pigmented skin lesions. Left adrenalectomy was performed because the nodule on the left adrenal gland was markedly larger than the other nodules. Subsequently, right adrenalectomy was also performed. Pathological analysis revealed that the adrenal glands contained multiple dark brown and black nodules. We describe herein our recent experience of a case of Carney complex manifesting as Cushing syndrome due to PPNAD, with lentigines on the lips, and review the published literature on this subject.