Abstract
We report two patients, a 28-year-old female (case 1) and her 53-year-old father (case 2), with multiple endocrine neoplasia (MEN) type IIa (Stipple's syndrome). A family history revealed the presence of Sipple's syndrome in the uncle and grandfather of the female patient. The two patients had no symptoms, and their blood counts and routine blood chemistry tests results were normal. Howeve, the levels of serum calcitonin, adrenaline, and noradrenaline were high in both cases, while the carcinoembryonic antigen level was elevated in case 1. A CT scan demonstrated bilateral thyroid tumors with calcification and a hemiadrenal tumor in both cases. The patients were diagnosed as having Sipple's syndrome because an aspiration biopsy of the thyroid tumor revealed medullary carcinoma. A hemilateral adrenalectomy followed by a total thyroidectomy were performed, without intraoperative hypertensive crisis, hypotension or arrhythmia.
Histopathological examination revealed the presence of medullary carcinoma in the thyroid glands and pheochromocytomas in the adrenal gland. The levels of serum adrenaline and noradrenaline were within the normal range in both cases after surgery. The two patients were discharged without complications.