Neoplasia endócrina múltipla IIa e gestaçäo / Multiple endocrine neoplasia type IIa and pregnancy

ABSTRACT

Multiple endocrine neoplasia typella (Sipple Syndrome) is characterized by the presence of medullary thyroid carcinoma (MTC), pheochromocytoma and parathyroid hyperplasia. Although this is a rare tumor, its study is extremely relevant because its autosomal dominant type of inheritance requires familial screening. This is a report of a 35-year old pregnant woman with signs and symptoms involving arterial hypertyension, cephalea and palpitation. Her medical history included right adrenalectomy due to a phenochromocytoma and aspirative needle biopsy of thyroid sugesting MTC. In this pregnancy the patient received Prazosin and was later submitted to left adrenalectomy at 23 weeks of gestation. Histopathologic examination reveaaled a pheochromocytoma. The clinical picture became acute again stating on the 31 st week of gestation, requiring the use of Prazosin. Gestation was interrupted by cesarian section during yhe 34t week due to fetal sulfering. The infant's condition at birth was regular and recovery was good. During the late postpartum period, the patient was submited to toal thyroidectomy and left inferior parathyroidectomy. Histopathologic examination revealed MCT and parathyroid hyperplasia