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Case of Peripheral Neuroepithelioma with Ectopic ACTH Syndrome.

Journal of the Korean Pediatric Society 1987;30(10):1152-1160.
Published online October 31, 1987.
Case of Peripheral Neuroepithelioma with Ectopic ACTH Syndrome.
O Y Lee1, K S Lee1, H I Chung1, H J Park1, M J Shin1, K J Cho2, J G Chi2
1Department of Pediatrics, Seoul Red Cross Hospital, Seoul, Korea
2Department of Patholgy, College of Medicine, Seoul National University, Seoul, Korea
이소성 ACTH 증후군을 보인 말초성 신경 상피종 1례
이옥엽1, 이경숙1, 정해일1, 박호진1, 신미자1, 조경자2, 지제근2
1서울적십자병원 소아과
2서울대학교 의과대학 병리학교실
Peripheral neuroepithelioma is a rare and controversial neoplasm that may occur at any age. The authors observed the course of a 1-year-old Korean female infant who presented with an enlarging mass in the right hand and manifested hepatic and pulmonary metastases at the time of diagnosis. She also presented with characteristic Cushingoid features, such as moon face, buffalo hump, and truncal obesity. Laboratory studies revealed hyperglycemia, hypokalemia, hypercortisolemia, and elevated serum ACTH levels. Unfortunately she died 1 month after diagnosis without any trial of the treatment. At autopsy, there was no involvement of adrenal glands or sympathetic ganglia or pituitary gland. Tumor revealed peripheral neuroepithelioma with multiple distant metastases to both lungs, chest wall, mediastinum, pleura, diaphragm, liver, pancereas and abdominal cavity. To our knowlege this case is considered the first reported instance of the ectopic ACTH syndrome due to a peripheral neuroepithelioma.
Key Words: Peripheral neuroepithelioma, Cushing’s syndrome, Ectopic ACTH syndrome

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