Minimally Invasive Surgery (MIS) in Children and Adolescents with Pheochromocytomas and Retroperitoneal Paragangliomas: Experiences in 42 Patients.

Abstract

Pheochromocytomas (PH) and paragangliomas (PGL) are rare tumours in children accounting for about 1% of the paediatric hypertension. While minimally invasive surgical techniques are well established in adult patients with PH, the experience in children is extremely limited. To the best of our knowledge, we herewith present the largest series of young patients operated on chromaffin tumours by minimally invasive access. In the setting of a prospective study (1/2001-12/2016), 42 consecutive children and adolescents (33m, 9 f) were operated on. Thirty-seven patients (88%) suffered from inherited diseases. Twenty-six patients had PH, 11 presented retroperitoneal PGL, and five patients suffered from both. Altogether, 70 tumours (mean size 2.7cm) were removed (45 PH, 25 PGL). All operations were performed by a minimally invasive access (retroperitoneoscopic, laparoscopic, extraperitoneal). Partial adrenalectomy was the preferred procedure for PH (31 out of 39 patients). Twenty patients received α-receptor blockade preoperatively. One patient died after induction of anaesthesia due to cardiac arrest. All other complications were minor. Conversion to open surgery was necessary in two cases with PGL. Median operating time for unilateral PH was 55min, in bilateral cases 125, 143min in PGs, and 180min in combined cases. Median blood loss was 20ml (range 0-1000). Blood transfusion was necessary in two cases. Intraoperative, systolic peak pressure was 170±39mmHg with α-receptor blockade and 191±33mmHg without α-receptor blockade (p=0.41). The median post-operative hospital stay was 3days. After a mean follow-up of 8.5years, two patients presented ipsilateral recurrence (after partial adrenalectomy). All patients with bilateral PH (n=13) are steroid independent post-operatively. PH and PGL in children and adolescents should preferably be removed by minimally invasive surgery. Partial adrenalectomy provides long-term steroid independence in bilateral PH and a low rate of (ipsilateral) recurrence. α-Receptor blockade may not be necessary in these patients.