Laparoscopic resection of pediatric interaortocaval large paraganglioma

Abstract

Paragangliomas (PGL) are extremely rare tumors, especially in children. While minimally invasive surgical techniques are well established in adult patients, the evidence in children is extremely limited. PGL have a rich blood flow and are located around large vessels; thus, operative exposure and resection are often difficult due to proximity to major vascular structures including renal vessels, vena cava, and aorta. In particular, surgery for interaortocaval large PGL is extremely challenging. In this report, we presented a case of a child with hormonally active interaortocaval large PGL that was resected laparoscopically. A 14-year-old girl was transferred to the emergency room due to acute chest pain and vomiting after falling from a vaulting box. At presentation, her blood pressure was 218/137 mmHg and enhanced computed tomography of her abdomen revealed a 6.5-cm hypervascular tumor with intratumoral hemorrhage in the interaortocaval area. The patient's plasma catecholamine and 24-h urinary catecholamine level were found to be elevated. 131 I-MIBG scan showed increased uptake in the tumor. The definitive diagnosis of PGL was made and we conducted elective laparoscopic excision of the retroperitoneal tumor. Careful and meticulous dissection was needed to separate the tumor from the surrounding vessels and tissues using a sealing device. Intraoperative hypertension was controlled using phentolamine, and the surgeon closely communicated with the anesthesiologist intraoperatively. Complete resection of the tumor was achieved laparoscopically. Total blood loss was 175 mL and the operating time was 422 min. Six months later, she remained well with no evidence of recurrence. The laparoscopic approach gave excellent exposure and observation of the tumor's relationship to surrounding structures. Our findings suggest that laparoscopic tumor removal for interaortocaval large PGL is a feasible and safe procedure in children with the improvement of laparoscopic surgical skills and devices.