# Clinical features and oncologic outcomes of primary retroperitoneal ganglioneuroma: a retrospective cohort study of 51 patients from a high-volume sarcoma center

## TL;DR

This study analyzed clinical features and outcomes of 51 patients with rare retroperitoneal ganglioneuroma tumors, showing that surgical resection at specialized centers leads to good results with low recurrence rates.

## Contribution

The study provides the largest cohort analysis of retroperitoneal ganglioneuroma, offering insights into clinical presentation, surgical outcomes, and management strategies.

## Key findings

- Most patients had nonspecific symptoms like abdominal discomfort or were diagnosed incidentally.
- Complete or subtotal resection was achieved in 88.2% of cases with low recurrence and no metastases over a median 62-month follow-up.
- Specialized centers are recommended for surgery to reduce complications and improve outcomes.

## Abstract

Retroperitoneal ganglioneuroma (RGN) is a rare, benign tumor derived from neural crest cells of the sympathetic nervous system. Due to its rarity and complex management, clinical understanding remains limited. This study aimed to analyze the clinical features and surgical outcomes of RGN.

A retrospective analysis was performed utilizing the retroperitoneal tumor database of Peking University International Hospital. Patients who underwent surgical resection for pathologically confirmed primary RGN between January 2015 and August 2024 were included. Systematic postoperative follow-up was conducted to assess outcomes.

Fifty-one consecutive patients (18 males, 33 females; median age 28 years, range 12–73) with newly diagnosed RGN were enrolled. Clinical presentations were heterogeneous: 29 cases (56.9%) were incidental findings on physical examination, 16 (31.4%) reported abdominal discomfort, 3 (5.9%) had lumbago, and single cases presented with hematuria, chest tightness, or lower limb pain. R0/R1 resection was achieved in 45 patients (88.2%). Postoperative complications occurred in 11 patients (21.6%), including gastroparesis (n = 3), pancreatic fistula (n = 3), liver function impairment (n = 2), wound infection (n = 2), and one mortality due to intestinal ischemia and necrosis. Histopathology confirmed GN in all cases. At median follow-up of 62 months (90.2% follow-up rate), no recurrences, metastases, or disease-specific deaths occurred—including in R2 resection patients (n = 6).

Retroperitoneal ganglioneuroma (RGN) is a rare benign tumor that frequently presents with nonspecific symptoms. While surgical resection remains the cornerstone of management, it is best undertaken at specialized, high-volume sarcoma centers to mitigate operative risks. When complete excision is precluded by critical vascular involvement, subtotal resection represents a judicious alternative. For selected patients with small, asymptomatic tumors—particularly those with elevated surgical risk—active surveillance is a reasonable option. Despite a generally favorable prognosis, long-term follow-up is recommended.