Retroperitoneal Ganglioneuroma Incidentally Found in a Patient Presenting with Renal Colic

Abstract

A 21-year-old man with no significant past medical history presented to the emergency department with sudden-onset left upper and lower quadrant pain, which he described as being sharp and nonradiating. He also complained of nausea and dry heaves. Examination revealed left lower quadrant and left flank pain on palpation with slight protrusion of the left flank. There were no palpable masses. Urinalysis revealed 3+ blood. Computed tomography (CT) of the abdomen revealed a 2 mm obstructing stone in the distal left ureter (not shown), which was likely the source of the patient's pain, and a heterogenous mass in the retroperitoneum centered within the mesenteric root containing multiple coarse internal calcific deposits (Figure ​(Figure11). The mass was relatively well circumscribed and encased the inferior mesenteric artery without appreciable extrinsic mass effect (Figures ​(Figures22 and ​and33). The mass was subsequently biopsied by CT guidance, and histopathology was consistent with a ganglioneuroma (Figure ​(Figure44). The neoplasm was resected in its entirety. Figure 1 Axial noncontrast CT image demonstrates an oval mass in the retroperitoneum (short arrows) containing multiple coarse internal calcific deposits (long arrow). The internal attenuation of the mass is slightly less than that of the visualized musculature, ... Figure 2 Axial postcontrast CT image shows the well-circumscribed nature of the mass (red arrows). The mass is seen anterior to the abdominal aorta (yellow arrow) and inferior vena cava (dotted yellow arrow). The portal venous phase of the contrast demonstrates ... Figure 3 Coronal postcontrast images through the mass better delineate the encasement of the inferior mesenteric artery from its origin (arrows) without luminal compromise. Again seen is the well-circumscribed nature of the mass and internal calcific deposits. ... Figure 4 Photomicrographs of the specimen showing (a) a prominent background of wavy collagen fibers and Schwann cells and (b) mature ganglion cells (arrow), consistent with a ganglioneuroma. Ganglioneuromas are a rare neoplasm of neural crest cell origin. They are typically benign but can undergo malignant trasnformation in rare instances. The retroperitoneum and posterior mediastinum are the two most common locations for a ganglioneuroma. Patients of all ages are affected, but it is classically seen in adolescents and young adults. Patients are usually asymptomatic but can present with vague abdominal pain. Prognosis is usually excellent after complete surgical excision of the mass.