Retroperitoneal Ganglioneuroma: an Uncommon Presentation and Literature Review

Case Report: A female patient, 18-year-old presenting lumbar pain and progressive strength loss of lower limbs that had started 4 months ago sought emergency medical assistance in September 2016. She presented on neurologic examination paraparesis, asymmetric strength loss of the lower limbs, more accentuated on the right. Also presented with cauda equine symptoms. Magnetic Resonance Image shows a large expansive neoplastic lesion occupying lumbar canal was seen from the middle of L1 vertebral body to the lower border of L4 vertebral body. Predominantly extradural presenting areas of mild postcontrast heterogeneous and peripheral enhancement. The dural sac was compressed and displaced anterolaterally to the left. The patient underwent surgical intervention with the neurosurgery and general surgery services. A combined anterior and posterior approach was performed to remove the lesion. The Histopathological study showed a solid and compactor tumor lesion. Microscopic analysis revealed biphasic neoplasia composed of scattered ganglion cells and grouped between abundant axonal processes. Immunohistochemical reactions revealed strong marking by synaptophysin in ganglion cells. These characteristics reveal a diagnosis of Ganglioneuroma.

Discussion: Ganglioneuromas occurs in children and young adults. The middle age at diagnosis is 7 years. The Most common locations for Ganglioneuromas are the posterior mediastinum, retroperitoneum, adrenal gland and neck. Retroperitoneal Ganglioneuromas are usually asymptomatic until they reach large size. The symptoms are caused for compression of neighboring structures as back pain, vomiting, constipation, weight loss and urinary symptoms. The treatment of this lesion type is essentially a surgical procedure.

Conclusion: Retroperitoneal Ganglioneuroma is a rare benign tumor that is diagnosed typically in children and young adults. Usually asymptomatic, but the symptoms are typically associated with large tumors sizes. Spinal symptoms can occur for compression or invasion of dural sac. The Management is mainly surgical.