ACTA ENDOCRINOLOGICA (BUC)

Introduction. Pheochromocytoma is a rare clinical finding during pregnancy. Due to the variable clinical presentation it may be mistaken for preeclampsia or primary hypertension. The early antenatal diagnosis is crucial, because it reduces possible maternal and fetal complications. Pheochromocytomas are usually benign, but may also present as or develop into a malignancy. Malignancy requires evidence of metastases at non-chromaffin sites distant from that of the primary tumor. Large tumor size and malignant disease are not necessarily associated. Case. The patient, a 39 years old multipara presented at 30 weeks of gestation with labile hypertension, headache and palpitations. She had a 6 years history of chronic hypertension controlled during the pregnancy with methyldopa. Using this treatment blood pressure was maintained at 140/100 mmHg. Further biochemical and radiological investigations confirmed the diagnosis of pheochromocytoma. The patient was invasively monitored and treated with alpha-adrenoblockers. Childbirth was performed by elective cesarean section at 34 weeks with simultaneous right-sided adrenalectomy. Postoperative period was uneventful. Histological examination of 12 cm encapsulated tumor revealed trabecular type pheochromocytma with focal capsular invasion. Although the usual criteria for malignancy, such as mitotic activity, nuclear pleomorphism, are not suitable to discern benign from malignant pheochromocytomas, we considered this large tumor presumably malignant in order to provide systematic longterm follow-up. Postoperative biochemical and imagistic screening was planned to detect and treat local recurrence or metastatic tumors. Conclusions. A multidisciplinary team to diagnose and treat pheochromocytoma during pregnancy is mandatory. Careful postoperative monitoring of recurrent disease is necessary indefinitely.