초록 열기/닫기 버튼
Carney complex는 피부의 색소침착과 여러 양성 종양(점액종, 내분비 종양)이 상염색체 우성으로 표현되는 질환이다. 저자들은 다발성 척추 골절로 내원한 젊은 남자에서 쿠싱양 외형을 관찰하여 시행한 검사상 PPNAD 발견하였고, 환자의 입술에 색소 침착을 관찰하여 Carney complex 진단하였다. 이에 좌측 부신절제술 이후 우측 부신절제술 단계적으로 시행하고 조직학적으로 확진된 예를 문헌고찰과 함께 보고하는 바이다.
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. (Korean J Med 2011;80:S239-S244)
키워드열기/닫기 버튼
Carney complex, Cushing syndrome, Pigmentation
