Retroperitoneal paraganglioma in a child: resection and vena caval reconstruction

Abstract.

Retroperitoneal paragangliomas (PG) are tumors of neuroectodermal origin rarely seen in children. A 10-year-old boy presented with back pain and a palpable mass secondary to a large right-sided retrocaval PG inferomedial to the kidney. Preoperative chemotherapy and perioperative embolisation were instigated to reduce tumor vascularity. En-bloc resection of the inferior vena cava below the left renal vein and above the caval bifurcation was achieved. Caval reconstruction was completed using a complex autograft of anterior uninvolved vena cava and right renal vein. At 4-year follow-up, the child remains tumor-free with normal caval flow and patency.