Spinal Cord Diffuse Leptomeningeal Glioneuronal Tumor Presenting without Leptomeningeal Dissemination

Background and Importance: Diffuse leptomeningeal glioneuronal tumor (DLGNT) represents a provisional entity in the 2016 World Health Organization classification of tumors; it is characterized by a widespread leptomeningeal growth and oligodendroglial-like cytology. To this day, 4 pediatric patients have been reported to present with an isolated spinal cord tumor in the absence of leptomeningeal dissemination. Gross total resection (GTR) was achieved in only 1 patient. We present the clinical and technical nuances of this unique type of tumor, as well as the second reported case of GTR in a patient with DLGNT. Clinical Presentation: A 4-year-old boy presented to the emergency department after an episode of flaccid paralysis of bilateral lower extremities. MRI showed an intramedullary spinal cord tumor centered at T8. The patient was taken to the operative room, where a laminectomy and tumor resection were performed; cystic and solid tumor components were identified. Pathology report was consistent with DLGNT. After achieving GTR, patient is free of recurrence after a 15-month follow-up. Conclusion: No standard treatment for DLGNT has been identified. Current literature report surgery and chemotherapy with variable success rates. DLGNT presenting as an isolated intramedullary tumor is an uncommon condition which progression appears to be halted when treated promptly. Identifying solid and cystic components of this tumor is crucial for achieving GTR.