Abstract. Ganglioneuromas are slow growing, clinically silent benign tumors for which surgery is considered to be the standard treatment. However, surgical excision in cases where surrounding structures are involved can be challenging. The present study reports a novel technique of ex vivo excision for the management of a retroperitoneal ganglioneuroma in a 21-year old patient, that appeared to be inoperable using standard surgical resection. Preoperative investigations revealed a large tumor with encasement of the origins of the superior mesenteric artery (SMA) and bilateral renal arteries. Initially, to prevent the need to explant the liver, the distal SMA (with takeoff of the replaced common hepatic artery) was anastomosed to the splenic artery. The bulk of the tumor along with the bilateral kidneys was mobilized from the retroperitoneum, and the aorta and inferior vena cava (IVC) were cross-clamped above and below the tumor and divided. The two kidneys were dissected free of the tumor at the back-table and were auto-transplanted in a standard technique following the reconstruction of the aorta and IVC. The patient tolerated surgery well and a one-year postoperative follow-up did not show any sign of tumor recurrence. Although technically demanding, ex vivo resection and auto-transplantation of the involved organs can be introduced as a final option for the treatment of tumors that are un-resectable using standard surgical techniques.
IntroductionGanglioneuromas (GNs) are rare, slow-growing benign tumors arising from the sympatho-adrenal neuroendocrine system (1,2). GNs typically occur in children and adolescents, with up to 60% of patients <20 years old at the time of diagnosis. Additionally, females are more likely to be affected than males (3). GNs are the benign ends of a wide spectrum of peripheral neuroblastic tumors, which also include neuroblastoma, ganglioneuroblastoma and ganglioneuroblastoma intermixed (2,4). GN can be diagnosed de novo in healthy patients or in specific cases result from spontaneous or chemo-or radiotherapy induced maturation of less-differentiated neuroblastic tumors (neuroblastoma and ganglioneuroblastoma) (1,5). In addition, certain studies have presented an association between GNs and specific familial diseases including neurofibromatosis type 2 and multiple endocrine neoplasia type 2 (6,7). While GNs can occur anywhere along the sympathetic nervous system, the two most common locations of occurrence are the posterior mediastinum and retroperitoneum (6). Retroperitoneal tumors may originate from the adrenal glands or extra-adrenal tissues (6). There are a limited number of studies reporting tumor occurrence in uncommon locations, including the tongue, bladder, uterus and skin (8-10).Although complete surgical excision is considered to be the treatment of choice in the management of these tumors, occasionally it can be challenging and in specific cases (such as when large tumors are involving the surrounding vital organs) it is not a feasible option. The current case report pre...