Endocrine Abstracts

Introduction: Pheochromocytoma multisystem crisis (PMC) is the most fulminant clinical expression of pheochromocytoma. The appropriate timing and judgment for the start of surgery and the adequate preoperative medical treatment are unclear. We report a case of PMC successfully treated with elective adrenalectomy after doxazosin blockade. The importance of an early biochemical diagnosis is discussed.

Case report: A 47-year-old healthy man consulted for sudden onset dyspnea and thoracic discomfort. In the Emergency Department, his level of consciousness upon arrival was 12 (Glasgow Coma Scale) and he was found to be in acute respiratory distress with severe hypoxemia, requiring intubation in Intensive Care Unit. His blood pressure was 165/110 mmHg and his body temperature, 39.9 °C. Electrocardiography revealed atrial flutter. The laboratory data showed polycythemia, severe renal and hepatic failure, rhabdomyolysis and hyperglycemia. All infectious screens were negative. A blood sample drawn on the patient’s arrival at the hospital showed high serum norepinephrine (>758 pg/ml; normal <370). Epinephrine and dopamine concentrations were normal. An abdominal CT scan revealed a 3 cm. Mass, located in the right adrenal gland and a MIBG scan was strongly positive. After reaching clinical stability, elective right laparoscopic adrenalectomy was performed under previous α-blockade with doxazosin. Pathology examination revealed a 3 cm. Pheochromocytoma without evidence of malignant involvement, necrosis or haemorrhage. Six months after, he is asymptomatic and his level of catecholamines is normal.

Conclusions: Most reported PMC cases requiring emergency surgery or died were mixed producers of epinephrine and norepinephrine or epinephrine only. The high rate of initial suspicion allowed us an early identification of amine secreted by the tumour and it allowed us to plan a specific therapeutic strategy. Future studies with a major number of cases are needed; nevertheless our observation suggests that pure norepinephrine secretor tumours may have a more favourable clinical course and this knowledge could help to identify candidates to elective surgery after doxazosin blockade.