Sipple症候群の2例

Abstract

Two cases of Sipple's syndrome are reported. The first case, a 36-year-old woman, was admitted because of paroxysmal hypertensive crises, headache, palpitation and sweating. There was a thumb's head sized mass in the right lobe of the thyroid gland but no abdominal mass. Endocrine examination showed a conspicuous rise in serum calcitonin and in plasma and urinary catecholamine level. In addition, serum adrenocorticotropic hormone, prolactin and parathormone values were high. CT-scan could detect a right large suprarenal mass lesion, and additional selective left inferior phrenic arteriography revealed left small suprarenal vascular mass. Based on these data, a diagnosis of medullary carcinoma of the thyroid gland (MCT) and bilateral adrenal pheochromocytoma was made. Two months after bilateral adrenalectomy, total thyroidectomy was performed. Histologic examination showed pheochromocytoma and MCT in both lobes. The provocation test by gastrin revealed on elevated serum calcitonin level in one of her children. The second case, a 26-year-old female, was admitted with right large abdominal mass. She noticed her abdominal mass in the third trimester, but had never had a history of hypertension. A right large abdominal mass and hypertension were found in her postpartum course. Physical examination revealed a hard mass in the left lobe of the thyroid gland. Findings from the hormonal studies, e.g., serum calcitonin, plasma and urinary catecholamine values, suggested MCT and pheochromocytoma. An aortogram demonstrated a large mass that occupied the right suprarenal region and CT-scan detected several masses on the left side. On trans-abdominal exploration, bilateral pheochromocytomas were found and removed. Total thyroidectomy was performed at an interval of four weeks. The pathology reports confirmed bilateral pheochromocytoma and MCT in both lobes of the thyroid gland. 42 cases of Sipple's syndrome in Japan were reviewed and discussed.