Primary Retroperitoneal Ganglioneuroma: A Retrospective Cohort Study of 32 Patients

Abstract

Purpose: To investigate the clinical characteristics, diagnosis, differential diagnosis, therapy options, and outcomes of retroperitoneal ganglioneuroma.Methods: In this retrospective study, we collected and analyzed the clinical data of 32 patients diagnosed with retroperitoneal ganglioneuroma and admitted to Peking Union Medical College Hospital from October 2012 to August 2019.Results: Among our 32 cases with retroperitoneal ganglioneuroma, the male-to-female ratio was 1:3 and the mean age was 35. Only 25% of the cases presented with abdominal pain while more than 65% had no specific symptoms. The masses could be found through physical examination in only five patients. Most of the tumors are located near the renal area. They were usually single and displayed an embedded growth pattern with diameters <10 cm, clear borders, and soft texture. For radiological imaging, the majority of tumors demonstrated soft tissue density with mild-to-moderate enhancement on CT imaging and showed hypoecho with moderate blood flow signals in ultrasound. No significantly abnormal laboratory examinations were found in most patients. Of all the 32 patients, 2 chose surveillance after biopsy due to difficulties in operation, while others chose surgical resection. The mean follow-up time was 15.8 months among 26 patients. The tumor remained stable in the surveillance cases. Residual tumors were found in four cases receiving operations with no progress and discomfort. No recurrence was seen in all patients.Conclusions: The retroperitoneal ganglioneuroma is a benign tumor without specific clinical manifestations or significant laboratory findings. Typically, it is shown as low density with a clear border and an embedded growth pattern in radiological imaging. The overall prognosis is good. Surgery is an effective approach with possible severe complications. Incomplete resection or surveillance can be considered for some cases where complete resection is difficult to achieve.

A huge retroperitoneal ganglioneuroma in a middle‑aged patient: Report of a diagnostically challenging case with review of the literature

“…These findings, however, are not diagnostic and are insufficient to make an accurate diagnosis (20). Misdiagnosis of GN as sarcoma has been reported via CT scan in certain cases due to irregular mixed density (9). Workup of the initial CT of the patient of the present study was also suggestive of sarcoma.…”

“…Less commonly, GN may involve the medulla of the adrenal glands, the parapharyngeal region and the visceral ganglia (3). Although most studies indicate a higher prevalence among females (9,12), other studies have indicated no gender preference (13). The current case was a male with a huge GN presenting in the retroperitoneum.…”

“…Surgical removal of GN is curative in most cases, with a low incidence of recurrence (2). Xiao et al (9) demonstrated that among 32 patients with GN, two patients chose surveillance instead of an operation and the tumor remained stable during follow-up in both cases. Among the other 30 patients, four had the tumor incompletely resected and they still did not exhibit any signs of progression or malignancy during follow-up (9).…”

“…On rare occasions, the presence of adipose cells has been detected in these tumors (8). This may result in the diagnostic confusion of GN via imaging and cytologic diagnostic approaches with other, more aggressive tumors, such as liposarcoma, leading to inappropriate management (9).…”

The Role of CT in the Staging and Follow-Up of Testicular Tumors: Baseline, Recurrence and Pitfalls

“…Ganglioneuroma is a rare benign tumor arising from the neural crest cells from the sympathetic nervous system, typically seen as a well-circumscribed retroperitoneal mass, surrounding vessels without compression or occlusion, with a low density (approximately 30HU), weak enhancement, and may contain some punctate calcifications [ 70 ].…”