Large functional retroperitoneal paraganglioma: A surgical challenge

Abstract

Paraganglioma is relatively rare retroperitoneal tumors. If functional retroperitoneal paragangliomas are misdiagnosed, surgical intervention can precipitate intraoperative hypertensive crises which may have serious consequences. We present a case of a 40-year-old female who presented with a large functional right-sided retroperitoneal paraganglioma encasing the inferior vena cava (IVC). The patient underwent paraganglioma excision with IVC resection with right nephrectomy. Such complex multivisceral resections require surgical expertise and are feasible at high-volume centers experienced in performing retroperitoneal surgeries.

Figure 1

Contrast-enhanced computed tomography images (a and b) Large well-defined heterogeneously enhancing retroperitoneal mass (*) with lobulated margins and nonenhancing necrotic areas noted within on the right side and seen crossing midline towards the left side. It measures approximately 13.6 cm × 14.5 cm × 11 cm. Antero-superiorly, the mass shows loss of fat planes with the head and the proximal part of the body of the pancreas and the second and the third parts of the duodenum (yellow arrows). Posteriorly, it is seen compressing the right kidney. The left renal vein is encased by the mass (blue arrow). Medially, it is abutting the abdominal aorta with angle of contact of 90°–180° (green arrows). It is completely encasing infrahepatic and renal inferior vena cava. (c) Three-dimensional reconstructed computed tomography angiogram showing multiple dilated tortuous collaterals in the retroperitoneum surrounding the lesion, likely secondary to chronic inferior vena cava obstruction (red arrows)

Figure 2

Intraoperative picture of the retroperitoneal mass after a complete Cattell–Braasch’s maneuver has been performed, where the right colonic mesentery is seen medially (*). The retroperitoneal mass is in close relation with the duodenum and the pancreas. A leash of large collateral vessels is seen close to the mass near its lower end (white arrows)

Figure 3

(a) Post-resection surgical bed. Lower divided end of the inferior vena cava (yellow arrow) can be seen along with the other structures in the right retroperitoneal bed. (b) Picture of the final specimen