A CASE REPORT OF SIPPLE'S SYNDROME

Abstract

We experienced a case of Sipple's syndrome which was suggestive of familial occurorence. A 27-year-old man was admitted to the hospital because of hypertention and palpitation. He had been aware of palpitation in the morning since the age of about 17 and was under treatment of diabetes on an ambulant base for about 5 years. Examinations showed increases in the values of serum adrenalin, noradrenalin, calcitonin and urine catecholamine. Ultrasonogram and CT scan revealed a 4.1×3.2cm mass in the right adrenal gland and a 1.3×7cm mass in the left thyroid. A diagnosis of Sipple's syndrome was made and right adrenalectomy for the adrenal tumor and left hemithyroidectomy with modified neck dissection were performed. Histological examination revealed benign pheochromocytoma in the adrenal gland and medullary carcinoma of the thyroid. His postoperative course was uneventful and currently he is under routine check-up. Blood examination of his family members revealed elevations in serum calcitonin and CEA levels for his brother and sister and medullary carcinoma of the thyroid was detected in both of them. Since Sipple's syndrome is a hereditary autosomal dominant disease, it is mandotory to follow up the family periodically by routine blood examinations including serum CEA and calcitonin.