All issues > Volume 30(10); 1987

Case Report

J Korean Pediatr Soc. 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

Abstract

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.

Keywords :Peripheral neuroepithelioma; Cushing’s syndrome; Ectopic ACTH syndrome