Laparoscopic Excision of a Familial Paraganglioma

Abstract

Pheochromocytomas are rare neuroendocrine tumors that secrete catecholamines and usually arise from the adrenal medulla. Catecholamine-producing tumors that arise from extra-adrenal chromaffin tissue are referred to as paragangliomas, or extra-adrenal pheochromocytomas. Contrary to the traditional "Rule of Tens," as many as 25% of pheochromocytomas occur in hereditary tumor syndromes, such as multiple endocrine neoplasia-2, von Hippel-Lindau disease, neurofibromatosis-1, or hereditary or familial paraganglioma syndrome. Surgical resection remains the only curative therapy for pheochromocytomas, and advances in minimally invasive techniques have shown laparoscopic adrenalectomy to be safe and effective. Paragangliomas are extremely rare tumors, especially in children, and the role of laparoscopy in their treatment is evolving. This case report and the accompanying video demonstrate that the laparoscopic approach to retroperitoneal paraganglioma resection provides excellent exposure of the tumor and surrounding structures. Given proper patient preparation and perioperative monitoring, laparoscopic exploration and resection of paragangliomas can be safely and successfully accomplished in children. In addition, genetic analysis should be considered for all patients presenting with these tumors.