Large Asymptomatic Retroperitoneal Ganglioneuroma Displacing Major Abdominal Organs and Vessels in an Adult

Patient: Female, 29-year-old Final Diagnosis: Ganglioneuroma Symptoms: Asymptomatic • incidental finding Medication: — Clinical Procedure: — Specialty: Surgery OBJECTIVE: Rare disease BACKGROUND: Ganglioneuromas (GNs) are benign neuroblastic tumors. These extra-cranial solid tumors are common in childhood but unusual in adults. Patients with GNs typically do not have any symptoms and the tumors usually are incidental findings. However, if a GN is large enough to compress adjacent organs, complications can occur. Furthermore, even in patients who have incomplete resection of a GN, long-term survival rates are high. After a GN is seen on imaging, the diagnosis usually is made with a biopsy and treatment is with surgery alone. CASE REPORT: A 29-year-old woman was referred to General Surgery from the Gynecology Clinic for an incidental finding of an abdominal mass on routine ultrasound for secondary infertility and admitted for an investigation. Abdominal and pelvic computed tomography (CT) and magnetic resonance imaging showed a retroperitoneal mass that measured 6.318×22 cm arising from the paravertebral region with intraspinal extension. The mass was displacing the patient’s thoracic aorta, abdominal inferior vena cava, and ureters. A CT-guided biopsy revealed a GN. Debulking surgery was performed and a small amount of residual tumor was left in the paravertebral nerve roots. The patient recovered well with no complications. The diagnosis of GN was confirmed with pathology, which was reviewed by the Tumor Board; the Board agreed that only follow-up in the Surgery Clinic was needed. During the patient’s last visit, 10 months after surgery, a follow-up CT scan showed that the residual tumor was stable. CONCLUSIONS: GNs are benign abdominal and retroperitoneal tumors that are typically asymptomatic and detected incidentally. Surgical resection is the treatment of choice and even when it is incomplete, the prognosis for patients is excellent.