후신경모세포종에 합병된 이소성 부신피질자극 호르몬 증후군 1예
A case of ectopic adrenocorticotropic hormone syndrome with olfactory neuroblastoma
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초록
요약하면 본 증례는 후신경모세포종에 병발한 이소성 ACTH 증후군에 대한 보고로서 국내에서는 첫 보고이다. 고 코르티솔 혈증에 의한 면역 기능 약화로 주폐포자충 폐렴 및 거대세포바이러스 폐렴에 이환되었으며 부신의 스테로이드 합성 억제 및 TMP/SMX 투약 후 회복되었다. 이소성 ACTH 증후군에서 원인 질환의 조속한 치료와 더불어 내인성 코르티솔 과잉 상태의 해결이 필요함을 시사하는 증례이다.
Abstract
We here present the case of a 37-year-old woman who suffered from adrenocorticotrophic hormone (ACTH)-dependent Cushing's syndrome due to an olfactory neuroblastoma. The patient's clinical course was complicated by co-infection with cytomegalovirus and Pneumocystis jirovechi, which improved with appropriate antimicrobial therapy and suppression of endogenous steroidogenesis with ketoconazole and octreotide. Radiation to the mass in the nasal cavity resulted in reduction of tumor size and remission of the Cushing's syndrome. This is the first report of the ectopic ACTH syndrome resulting from olfactory neuroblastoma in Korea.(Korean J Med 71:309-315, 2006)
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