From the Urologic Service, Massachusetts General Hospital, and the Department of Surgery, Harvard Medical School, Boston, Massachusetts
ABSTRACT -A case of Sipple’s syndrome, a triad of medullary carcinoma of the thyroid, pheochromocytoma, and parathyroid hyperplasia is presented. The cause, laboratory diagnosis, and operative management of the syndrome are discussed. The need fw thorough endocrine screening of patients with pheochromocytoma and family screening of patients with the full syndrome is stressed.
Sipple’s syndrome, a triad of medullary carcinoma of the thyroid, pheochromocytoma, and parathyroid disease, is a rarely occurring entity transmitted as an autosomal dominant trait with a high degree of penetrance. Sipple’s’ original report in 1961 associating medullary carcinoma of the thyroid and pheochromocytoma in 6 patients has been more recently followed by numerous reports associating parathyroid adenomas or hyperplasia with this syndrome2-4 leading to the present classification of the three entities as the multiple endocrine adenoma (neoplasia) syndrome type II. The case presented here represents many of the salient features of the syndrome and emphasizes the need for thorough preoperative endocrine evaluation by the urologist of all patients presenting with signs and symptoms of pheochromocytoma. Case Report A forty-seven-year-old white male, referred to the Massachusetts General Hospital with hypertension, was well until seventeen years prior to admission when he was noted to have renal calculi and had a left nephrolithotomy. Ten years prior to admission the patient was started on hydralazine for hypertension, and he did well until he had a right pyelolithotomy in 1971. Two months prior to
admission the patient was admitted to his local hospital with hypertension, anorexia, and weight loss. At that time his blood pressure was 290/140 mm. Hg with .a blood sugar of approximately 1,000 mg. per 100 ml. and a twenty-four-hour urinary vanilmandelic acid of 95.5 mg. (normal