Mature neuroblastic tumors with spinal cord compression: report of five pediatric cases

Neuroblastic tumors cause spinal cord compression when they arise primarily in the spinal canal or invade it through the radicular foramen. Whereas neuroblastomas (NB) are relatively common and are generally treated with chemotherapy, mature neuroblastic tumors (MNT), which include intermixed ganglioneuroblastomas (iGNB) and ganglioneuromas (GN), are less common and the role of surgery is more prominent. Because MNT are rare and have been separated only recently from NB, their clinical and radiological features as well as the role of surgery are poorly defined.

In order to increase our knowledge on MNT, we reviewed our database for cases operated for spinal cord compression in our department since the introduction of magnetic resonance imaging (MRI). We treated four cases of NB and one case of iGNB presenting primarily with spinal cord compression.

MNT represented 10% of spinal tumors and 1.6% of all tumors of the nervous system in our pediatric neurosurgical practice. The neurological and oncological outcomes were generally favorable after surgical resection, followed by orthotic treatment. In one case with neurofibromatosis type 1, the tumor was inoperable and the child died of tumor progression several years later.

GN, and some iGNB, are chemo-insensitive and can only be cured by surgical removal. Surgery is an emergency in case of rapidly progressing paraplegia and can be challenging because the tumor is often hard and hemorrhagic. In case of subtotal removal, tumor remnants can stay stable without oncological treatment.