Elsevier

World Neurosurgery

Volume 98, February 2017, Pages 198-202
World Neurosurgery

Original Article
Management of Patients with Primary Intramedullary Spinal Cord Glioblastoma

https://doi.org/10.1016/j.wneu.2016.10.075Get rights and content

Background

Primary intramedullary spinal cord glioblastomas are very rare tumors of the spinal cord. They imply a very poor prognosis because complete surgical resection is not possible as the result of the infiltrative growth of these tumors. The aim of this study is to present our data achieved with an aggressive multimodality treatment.

Methods

We retrospectively reviewed our clinical database. All patients with histologically proven intramedullary spinal cord glioblastoma treated in our department were included in this study.

Results

Four patients with intramedullary spinal cord glioblastoma were identified between 2006 and 2015, all of whom were female. Mean age at the time of surgery was 33.5 years (range 14–50 years). Tumors were located in the cervical region in 2 patients and in the thoracic region in 2 patients. All 4 patients underwent microsurgical biopsy of the tumor. After surgery, all patients received radiation and temozolomide treatment. One patient underwent additional therapy with Bevacizumab, another patient received Rapamycin and Sunitinib, and the third patient received Chlorethyl-cyclohexyl-nitroso-urea and Etoposide as additional therapy after tumor regrowth. Tumor progression occurred in a mean time of 18.2 months (6–32 months). In this series, all patients died as the result of progression of the malignancy; median survival after diagnosis was 32.5 months.

Conclusions

The surgical outcome of intramedullary spinal cord glioblastoma 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.

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.

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