Update on the optimal management of patients with vascular extension of pheochromocytoma

Abstract

Aim Vascular extension of pheochromocytoma is extremely rare but highly resectable. The aim of this study was to review the available information in the literature for patients with this condition. Methods A systematic literature search produced individual data for 29 treated patients with vascular extension of pheochromocytoma mainly to the inferior vena cava. Results Out of 29 reported cases, 26 have been treated surgically. Nine of these patients required cardiopulmonary bypass. Two patients died untreated and one patient declined surgical treatment and discharged to follow-up. In 24 patients including nine with primarily missed diagnosis, surgery was documented as curative. Conclusion Surgeons need to be aware of this extremely rare characteristic feature of pheochromocytoma and indicated medical work-up in order to properly diagnose and manage this challenging group of patients.