임상경과에 따라 코르티솔 분비능의 변화를 보인 부신피질선암 1예 (A Case of Adrenocortical Carcinoma showing Variable Cortisol Production during the Clinical Course)

서지정보

· 발행기관 : 대한내분비학회
· 수록지 정보 : Endocrinology and Metabolism / 21권 / 5호 / 419 ~ 423페이지
· 저자명 : 구윤희, 최형진, 김진택, 윤지완, 이은경, 조화영, 강미연, 이지선, 조영민, 김성연

초록

저자들은 코르티솔을 분비하는 부신피질선암을 진단 받고 수술을 시행한 후 비기능성 종양으로 재발하였다가, 비기능성 종양으로 수개월간 치료한 뒤 종양이 진행함에 따라 다시 코르티솔을 분비하는 능력을 획득한 부신피질선암의 예를 경험하였다. 본 증례에서 기능성이 변화하게 된 원인은 내분비 종양 내에 존재하는 기능성이 다른 클론 간의 우월성 등에 따른 것으로 판단된다. 부신피질선암은 종양이 진행하면서 분화도가 바뀔 수 있으므로, 이에 따른 적절한 평가 및 치료가 필요함을 시사하는 증례이다.

영어초록

Patients with adrenocortical carcinoma (ACC) present with evidence of excessive adrenal steroid hormone in approximately 60% of cases, in which rapidly progressing Cushing's syndrome with or without virilization is the most frequent presentation. Some patients experience an increase or a decline in cortisol production through the progression of their ACC. We report on an unusual case of a cortisol-producing ACC, and the patient presented with a decline in cortisol production, followed by an increase in cortisol production, through the progression of the tumor. A 65-year-old woman who manifested with facial edema and weight gain was diagnosed with Cushing's syndrome, caused by cortisol producing ACC. The patient was treated with adrenalectomy. However, 8 months later, a metastatic hepatic tumor of recurred ACC was detected. At that time, the hormonal evaluation revealed that the liver mass did not produce any hormones. The patient was treated with metastatectomy. Four months later, a relapsed tumor was detected. Increased cortisol production was observed at that time. We speculate there was a change in the clonal dominance within the ACC and this change might cause such a difference. This is the first case report of ACC that showed variable hormone production during progression. (J Kor Endocrinol Soc 21:419~423, 2006)

참고자료

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