Published online Sep 6, 2019. doi: 10.12998/wjcc.v7.i17.2617
Peer-review started: April 4, 2019
First decision: July 30, 2019
Revised: August 7, 2019
Accepted: August 20, 2019
Article in press: August 20, 2019
Published online: September 6, 2019
Ganglioneuroma (GN) is a rare neurogenic tumor that accounts for about 0.1%-0.5% of all tumors of the nervous system. It originates from neural crest cells. GN has no specific clinical symptoms or laboratory findings, which leaves it easily overlooked and misdiagnosed as other tumors. Retroperitoneal GN with very large volume and vascular penetration is extremely rare.
We present the imaging and pathological findings of a giant retroperitoneal GN in a child. A 4-year-old boy had suffered from postprandial vomiting for more than 6 mo with no precipitating factors. Abdominal computerized tomographic examination showed a giant cystic mass in the retroperitoneal area. After injection of contrast agent, the mass showed heterogeneous enhancement. Surgery with local excision of the mass was performed to address the embedded abdominal blood vessels, and the histopathological and immunohistochemical diagnosis of the mass was GN. Postprandial vomiting was relieved, and no complications occurred after the operation.
In the diagnosis of giant retroperitoneal hypodense masses in children, GN should be considered if the mass presents delayed enhancement, punctate calcification, and vascular embedding but no invasion. Pathology is the golden standard for the diagnosis of GN, and surgical excision is the optimal treatment for GN.
Core tip: Ganglioneuroma (GN) is a rare benign neurogenic tumor originating from the sympathetic nerve chain. Although it often occurs in the retroperitoneum, we report a case of giant GN causing postprandial vomiting and enclosing all major abdominal vessels, which is clinically rare. Surgical resection is the optimal choice for the treatment of GN at present. Pathology is the gold standard for the diagnosis of GN, and the presence of mucous matrix and ganglion cells are pathological features of GN.