Original ArticleManagement of Patients with Primary Intramedullary Spinal Cord Glioblastoma
Introduction
Primary spinal cord (SC) tumors are rare entities, accounting for 2%–4% of all central nervous system tumors.1, 2 Among these, astrocytoma represent only 6%–8% of all intramedullary tumors, with 75%–90% of them being low-grade gliomas. Therefore, SC glioblastoma multiforme (GBM) is extremely rare, accounting for only 1.5% of all SC tumors.3, 4, 5, 6 These highly malignant lesions occur mainly in the cervicothoracic segments and have a slight tendency to occur in the first decades of life. A short clinical history before diagnosis is mainly associated with the natural history of intramedullary spinal cord glioblastoma (ISCGBM).7, 8 The overall survival (OS) of patients with ISCGBM is approximately 10–12 months, in contrast to a better prognosis of 14 months for its intracranial counterpart.9 ISCGBM results in death resulting from complications related to progressive SC involvement, respiratory impairment, and cerebral metastases.7, 10 Only few data exist reporting current experience about survival and progression after the introduction of temozolomide (TMZ) and novel therapeutic strategies. The aim of this paper also is to highlight the effect of new drugs on survival and tumor control.
Section snippets
Methods
The study was approved by the local ethics committee at the authors' institution. We retrospectively reviewed the medical history of all patients with intramedullary SC tumors treated at our institution during a period of 36 years. Between 2006 and 2015, 4 patients with the diagnosis of ISCGBM were identified. The medical charts were reviewed, including surgical and histologic data, treatment parameters, neurologic outcome, progression-free survival (PFS), and OS. Histologic grading of all
Results
All patients were female, and their ages ranged from 14 to 50 years at the time of surgery (mean 33.5 years). The follow-up period ranged from 10 to 46 months (mean 32.5 months). The presenting symptoms and signs were pain, hypesthesia, or paresthesia in all cases. Two patients revealed motor weakness preoperatively and only 1 patient an improvement of the neurologic status after surgery and postoperative therapy. The preoperative functional assessment, which was made in all patients, showed 1
Discussion
As a result of the extremely rare occurrence of ISCGBM the current literature is scarce. A review of the pertaining literature suggests that this entity shows a predilection in the earlier decades of life.4, 18, 19, 20 In our series, mean age was 33.5 years with one patient being younger than 18 years. Apparently, the most affected spinal areas are the cervical and thoracic region in line with the data presented here.6, 21, 22 Because complete resection of these tumors is not possible because
Conclusions
The surgical outcome of ISCGGM still remains poor. Severe disability and amelioration of the neurologic status lead to reduced quality of life; however, an aggressive multimodal and interdisciplinary treatment for the disease may be associated with longer survival.
Acknowledgments
We thank Marina Heibel for her excellent technical support.
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Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.