Primary Intramedullary Spinal Cord Tumors in Children: A Single-centre Experience

Background: Primary spinal cord tumors are rare, accounting for 4 % to 8 % of all Central Nervous System (CNS) neoplasms in the pediatric population. Most of them are low grade tumors and prognosis is related to the extent of surgical resection.

Objective: The aim of this study was to evaluate the clinical outcome of a small group of patients with intramedullary spinal tumors.

Methods: We retrospectively analyzed clinical and histopathological features, treatment modalities, survival rates of children aged 0-14 years with a diagnosis of spinal tumor treated at our department during the last 15 years.

Results: A total of 93 patients with a CNS tumor were included. Four of them (4.3%) had a spinal localization: 2 males and 2 girls, 3/4 (75%) < 5 years of age at diagnosis (mean age 4.4+3.6 years). Three (75%) had cervical localization, one (25%) was localized in the lumbosacral tract. All cases underwent surgery: 2 biopsies (50%), 1 partial resection (25%) and 1 total resection (25%). All of them were low-grade gliomas: 2 (50%) pilocytic astrocytomas, 1 (25%) glioneuronal tumor and 1 ependymoma. All of the children with subtotal resection (biopsy and partial resection) underwent subsequent therapies: 2 were treated with radiotherapy (RT) and chemotherapy (CT), 1 is under medical treatment (SIOP-LGG 2004 protocol). Regimens of CT included Carboplatin-Vincristin in pilocytic astrocytomas, and Cisplatin-Temozolomide in glioneuronal tumor. At current time, all these patients had stable disease.

Conclusion: Our data support the evidence that the majority of intramedullary tumors are histologically benign and radical resection results in long-term survival. When total surgical resection is not achievable, excellent results in survival rates could be obtained with a multimodal treatment combining RT and CT.