Diagnostic and surgical management of a retroperitoneal ganglioneuroma: a case report

Retroperitoneal ganglioneuromas are rare, benign, well-differentiated tumors originating from neural crest cells. Peak incidence occurs between 20 and 40 years of age. Their preoperative diagnosis remains challenging due to non-specific clinical presentation and radiological overlap with malignant retroperitoneal neoplasms, such as sarcomas. Reporting such cases contributes to refined diagnostic pathways and surgical strategies. We report the case of a 24-year-old North African woman with no significant medical, family, or psychosocial history, who presented with chronic right iliac fossa pain evolving over 12 months. Physical examination revealed a firm, non-tender, poorly mobile right-sided abdominal mass measuring approximately 10 × 9 × 8 cm. Laboratory evaluation, including urinary and plasma catecholamines/metanephrines, was normal. Contrast-enhanced computed tomography (CECT) demonstrated a well-circumscribed retroperitoneal mass measuring 10 × 9 × 8 cm compressing the inferior vena cava (IVC) without invasion. Magnetic resonance imaging (MRI) and positron emission tomography (PET) were deemed non-essential given the clear encapsulating features on CECT. Endoscopic ultrasound (EUS)-guided transduodenal core biopsy with immunohistochemical (IHC) analysis established the preoperative diagnosis of ganglioneuroma. Complete surgical excision (R0) was performed via midline laparotomy with right colon mobilization and Kocher maneuver, preserving vascular integrity. Histopathological and IHC examination confirmed an encapsulated ganglioneuroma (S-100+, CD56+, chromogranin+, synaptophysin+). Postoperative course was uneventful (Clavien–Dindo grade 0). At 3-year follow-up, the patient remained asymptomatic without local recurrence. Accurate radiological and histological assessment is paramount. While active surveillance is emerging for small asymptomatic lesions, complete R0 surgical excision remains the curative standard for large or symptomatic tumors, yielding excellent long-term prognosis even when closely adjacent to major vascular structures.