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.