Multifocal ganglioneuromas are characterized by the presence of multiple benign neuroepithelial tumor nodules and are less common than solitary tumors. A small percentage of ganglioneuromas present with a fatty appearance. Only a few cases of multifocal ganglioneuromas have been reported, due to both their rarity and minimal symptomatic presentation; therefore, generalizations about risk factors and predictive markers are very difficult. Here, we report a case of multifocal retroperitoneal ganglioneuroma with an infiltrative appearance on computed tomography (CT). The tumor demonstrated slow growth on multiple imaging studies and was associated with abdominal and flank pain. The aggressive appearance eventually led to surgical resection 18 months after the initial incidental finding on CT. Postsurgical analysis of the tumor on imaging was crucial in revealing its nodularity and infiltration, as well as for clarifying its retroperitoneal location inseparable from the adrenal gland. Histology demonstrated Schwann cells and ganglion cells without atypia or increased cellularity, and with no mitosis or necrosis seen. Our case highlights the consideration of ganglioneuroma with fatty infiltration in the differential diagnosis of a fatty tumor in the mediastinum or retroperitoneum. Additionally, our report differentiates multifocal ganglioneuroma with fatty infiltration from lipomatous ganglioneuroma on radiology and histopathology.
Keywords: Case report; Computed tomography; Ganglioneuroma; Retroperitoneum.
© 2023 The Author(s). Published by S. Karger AG, Basel.