RESUMO
A 26-year-old male, with a family history of Paraganglioma Syndrome 4 (PGL4) presented with an 18-month history of paroxysmal headaches, a one-month history of frequent diaphoresis, anxiety attacks and unintentional weight loss of one stone in 2 months. Physical examination and vital parameters were normal. Laboratory studies showed significant elevation of plasma normetanephrines and 3-methoxytyramine while DNA molecular analysis confirmed pathogenic mutation in the SDHB gene and genetic transmission of PGL4. Imaging studies demonstrated a left para-aortic mass in the mid-abdomen and a mediastinal paraganglioma between the root of aorta and origin of the main pulmonary artery, encroaching the right ventricle. After adequate alpha blockade, the patient underwent a combined sequential hand-assisted laparoscopic resection of the abdominal tumour followed by midline sternotomy and resection of the second lesion at the root of the aorta, complicated by the need for emergency cardiopulmonary bypass due to perforation of right ventricular wall.
