LAUSR

Pheochromocytomatosis is a rare but dramatic complication of pheochromocytoma surgery. In this study, we collected and analysed our experience with the surgical treatment of cases of this rare condition. In total 9 patients were operated on between December 2001 and August 2022 at the Kliniken Essen-Mitte due to pheochromocytomatosis. There were 5 female and 4 male patients, mean age of 58 ± 18 (range: 22 to 86). The data was prospectively collected and retrospectively analysed. All patients were initially operated on due to pheochromocytoma (2 with SDHB-Syndrome and 1 with MEN-IIa-Syndrome). In 4 cases an intraoperative tumour capsule injury during the initial surgery was described. The operation due to pheochromocytomatosis was performed minimally invasive in 8 cases, 1 patient was operated on by open approach. The mean time between the primary surgery and the pheochromocytomatosis operation was 107 ± 60 months. Operation time was 246 + 119 minutes. Follow-up data were available for 8 patients (mean follow-up time 12 years). 2 patients were cured at the time of the last follow-up. 7 patients developed recurrent disease: loco-regional recurrence in 6 cases and distant metastasis in 3 cases. 4 patients with loco-regional recurrence were reoperated within 54 ± 50 months (range 10–137 months). By the follow-up, 3 patients died. Pheochromocytomatosis is a potentially lethal complication of pheochromocytoma surgery. Even if the initial pheochomocytomatosis could be managed minimally invasive, the recurrence rate is extremely high.