Abstract
Background
Ganglioneuroblastoma (GNB) with spinal canal involvement is exceptionally rare and encompasses anatomically heterogeneous tumors ranging from predominantly intraspinal lesions to paraspinal or mediastinal dumbbell tumors with foraminal extension. Evidence regarding their clinical behavior and management remains limited.Methods
A systematic review was conducted according to PRISMA guidelines. PubMed, Scopus, and Web of Science were searched from inception through April 30, 2026. Histologically confirmed GNBs with clinically relevant spinal canal involvement were included. Patient-level demographic, anatomical, pathological, treatment, and outcome data were descriptively synthesized.Results
Fourteen publications comprising 18 independent patients were included. Twelve patients (66.7%) were younger than 18 years, and thoracic involvement occurred in 13 (72.2%). Anatomical compartments were heterogeneous, including intramedullary, intradural extramedullary, extradural, and multicompartmental lesions. Recurrence or progression was documented in 4 patients (22.2%). Follow-up ranged from 1.7 to 228 months (median, 7 months), precluding reliable estimates of long-term prognosis.Case presentation
A 4-year-old girl presented with progressive lower-extremity weakness, sphincter dysfunction, and severe spinal cord compression from a cervicothoracic dumbbell GNB with posterior mediastinal and intraspinal components. Bone marrow involvement, MYCN amplification, and INRGSS stage M established high-risk disease. Combined neurosurgical-thoracic resection was followed by induction chemotherapy, radiotherapy, temozolomide, isotretinoin, and dinutuximab beta. Repeat bone marrow evaluation showed no persistent involvement. At the latest follow-up in April 2026, she was neurologically intact without clinical, biochemical, or radiological evidence of residual or recurrent disease.Conclusion
GNB with spinal canal involvement represents a rare, anatomically and biologically heterogeneous presentation. Management should integrate neurological urgency, anatomical characteristics, metastatic staging, and biological risk within an individualized multidisciplinary strategy.