Analysis of Eight Sipple's Syndrome Patients and Review of Eighty-two Cases from the Japanese Literature

Abstract

Eight Sipple's syndrome patients from four families have been reviewed. One family had the largest number of members with pheochromocytoma and/or medullary thyroid carcinoma. A total of 82 cases (47 females and 35 males) collected from the Japanese literature during the period 1960-1989, are also reviewed. The ages ranged from 22 to 73 (median 41) years, 13%, being over the age of 60 years (elderly patients). Pheochromocytoma occurred bilaterally in 72% of cases. With Sipple's syndrome, a relatively high urinary excretion of the epinephrine fraction and a high content of epinephrine in the tumor tissues were thought to be characteristic. Seventy-three patients underwent adrenalectomy with a successful outcome for 62 (85%). There needs to be careful periodic follow-up after unilateral adrenalectomy. Medullary thyroid carcinoma (MTC) also occurred bilaterally and was multicentric in 66% of cases, and was often found to be metastasized to the cervical lymph nodes at the time of surgery (35%). Parathyroid disease was found in 22% of cases: parathyroid adenoma in nine, hyperplasia in 10. Fourteen patients (17%) died of surgical complications, hypertensive crisis caused by excessive catecholamine release and/or widespread MTC metastases.