Glioblastoma multiforme of the conus medullaris with leptomeningeal dissemination that presented as intracranial hypertension: A case report and literature review

Yu Hu; Wumeng Yin; Junpeng Ma; Jiagang Liu; Siqing Huang; Haifeng Chen

doi:10.4103/glioma.glioma_15_21

CASE REPORT

Year : 2021 | Volume : 4 | Issue : 3 | Page : 57-61

Glioblastoma multiforme of the conus medullaris with leptomeningeal dissemination that presented as intracranial hypertension: A case report and literature review

Yu Hu¹, Wumeng Yin², Junpeng Ma¹, Jiagang Liu¹, Siqing Huang¹, Haifeng Chen¹
¹ Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China
² West China college of Stomatology, Sichuan University, Chengdu, Sichuan Province, China

Date of Submission	22-Sep-2021
Date of Decision	11-Oct-2021
Date of Acceptance	15-Oct-2021
Date of Web Publication	11-Nov-2021

Correspondence Address:
Dr. Haifeng Chen
Department of Neurosurgery, West China Hospital, Sichuan University, No. 37, Guo Xue Xiang, Chengdu, Sichuan Province
China

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/glioma.glioma_15_21

Abstract

Spinal glioblastoma multiforme (GBM) that originates from the conus medullaris is rare as only 28 cases have been reported. It is highly aggressive and usually initially presents with low back pain, sensory and motor impairment of the lower extremities, and bladder dysfunction. We herein report a unique case of GBM in the conus medullaris with leptomeningeal dissemination that initially presented with increased intracranial pressure without hydrocephalus. The patient was first diagnosed with tuberculous meningitis and received antituberculosis therapy, but the symptoms did not resolve and even worsened. Subsequent radiological imaging clearly disclosed an intramedullary lesion at the T12 level with the progression of leptomeningeal enhancement. Subtotal resection of the intramedullary lesion was performed, and pathological examination revealed the presence of GBM. To our knowledge, our case was the first with holocordal and intracranial leptomeningeal dissemination that initially presented with intracranial hypertension in the absence of hydrocephalus. Awareness of this unusually lethal condition is significant for proper diagnosis, timely treatment, and consideration of clinical prognosis.

Keywords: Case report, conus medullaris, intracranial hypertension, leptomeningeal dissemination, spinal glioblastoma multiforme, tuberculous meningitis

How to cite this article:
Hu Y, Yin W, Ma J, Liu J, Huang S, Chen H. Glioblastoma multiforme of the conus medullaris with leptomeningeal dissemination that presented as intracranial hypertension: A case report and literature review. Glioma 2021;4:57-61

How to cite this URL:
Hu Y, Yin W, Ma J, Liu J, Huang S, Chen H. Glioblastoma multiforme of the conus medullaris with leptomeningeal dissemination that presented as intracranial hypertension: A case report and literature review. Glioma [serial online] 2021 [cited 2023 Oct 1];4:57-61. Available from: http://www.jglioma.com/text.asp?2021/4/3/57/330196

Introduction

Classified as a Grade IV astrocytoma by the World Health Organization, primary spinal glioblastoma multiforme (GBM) is a rare but highly malignant central nervous system tumor.^[1],[2] It accounts for only 0.2% of all central nervous system GBMs and 1.5% of all spinal cord tumors.^{[2],[3],[4],[5]} Spinal GBMs usually occur in the cervical and thoracic spine and rarely arise from the lumbar spine.^[6],[7],[8] Approximately, 14% of spinal GBMs originating from the conus medullaris have been documented in the literature.^[9] Frequently, patients with conus GBMs initially present with low back pain, sensory and motor impairment of one or both lower limbs, and bladder dysfunction. Despite vigorous treatments, these tumors progress rapidly, and patients' survival time ranges from weeks to months. Herein, we report an extremely rare case of a 47-year-old woman with spinal GBM of the conus medullaris that initially presented with intracranial hypertension in the absence of hydrocephalus.

Case Report

A 47-year-old Chinese woman presented with headaches associated with intermittent vomiting for 2 weeks prior. Neurologic examination revealed bilateral papilledema and right lower extremity motor weakness. Magnetic resonance (MR) imaging of the spine and brain revealed a minimally enhancing intramedullary lesion of the conus medullaris in association with holocordal and focal intracranial leptomeningeal enhancement [Figure 1]. Cerebrospinal fluid (CSF) examinations revealed an opening pressure of 20 cm of water, 20 mononuclear cells, a protein concentration of 12.9 g/L (the normal range is 0.15–0.45 g/L), normal glucose level, and a chloride concentration of 103.4 mM (the normal range is 120–130 mM). Results of a tuberculosis interferon-gamma release assay were positive. Tuberculous meningitis was tentatively diagnosed, and antituberculosis therapy was initiated. Despite aggressive medical treatment, the increased intracranial pressure persisted, and she developed seizures. Further radiologic examination was warranted and clearly identified an intramedullary lesion at the T12 level with the progression of leptomeningeal enhancement [Figure 2]. The patient underwent a T12 laminectomy for subtotal tumor resection, and pathological examination revealed a GBM. Chemotherapy and radiotherapy were suggested, but the patient declined and died 6 months after surgery. IRB review was waived owing to the retrospective nature of this case report.

Figure 1: Magnetic resonance imaging of the spine and brain reveal an intramedullary lesion of the conus medullaris with leptomeningeal dissemination. The lesion (arrows) is hypointense signal on sagittal T1 (A), hyperintense signal on sagittal T2 (B), and minimal enhancement on sagittal (C), as well as axial (D) T1-weighted gadolinium-enhanced sequences. Brain magnetic resonance imaging demonstrates subtle leptomeningeal enhancement in the area surrounding the brainstem, sylvian fissures, and tentorium, without hydrocephalus (E, arrows)

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Figure 2: Magnetic resonance imaging performed 2 weeks after antituberculosis therapy. Spinal sagittal (A) and axial (B) T1-weighted images with contrast enhancement clearly reveal an intramedullary contrast-enhancing lesion of the conus medullaris with marked enhancement of the holocordal leptomeninges (arrows). Brain magnetic resonance imaging shows obvious leptomeningeal enhancement (C)

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The ethical approval and written consent were waived by our institutional review board owing to the retrospective nature of this case report.

Discussion

We reviewed the publications of GBM in the conus medullaris from a comprehensive literature research of the electronic databases in PubMed. The key search terms involved spinal, conus medullaris, GBM, intracranial, metastasis, and leptomeningeal. Only English articles were included as the eligibility criteria without other irrelevant publications through further identification. GBM is a rare pathological type of primary intramedullary spinal cord gliomas, and to our knowledge, only 28 cases of conus GBM have been documented [Table 1]. It is consistent with a population-based analysis of primary glioblastomas of the spinal cord from 1973 through 2007.^[10] The average age of onset is 26.5 years (ranging from 4 to 62 years), with eight patients older than 40 years, including the present case.^{[1],[2],[6],[7],[8],[11],[12],[13],[14],[15],[16],[17],[18],[19],[20],[21],[22],[23],[24],[25],[26],[27],[28]} The ratio of afflicted men-to-women is 19:10, revealing an obvious male predominance.

Table 1: Summary of glioblastoma multiforme of the conus medullaris

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The initial clinical presentations of GBM of the conus medullaris are usually nonspecific and include back pain, sensory and motor impairment of the lower extremities, and bladder dysfunction.^[5] Six cases (20.7%, including our patient) developed symptoms of intracranial hypertension, and this was due to hydrocephalus in five cases.^{[2],[11],[15],[21]} The probable causes of hydrocephalus in cases of spinal GBM are elevation of CSF protein concentration, occlusion of CSF channels, arachnoiditis, and intratumoral hemorrhage.^[7] Our case was unique because the patient initially presented with the symptoms of increased intracranial pressure without hydrocephalus. This might have been caused by elevated protein concentration in CSF that resulted in hemodynamic interruption that may progress to hydrocephalus in a later stage.^{[4],[28],[29]}

Radiographical investigations always reveal an ill-defined intramedullary lesion with heterogeneous enhancement. Enhanced MR imaging of the whole central nervous system should also be performed due to the high potential for CSF dissemination. Leptomeningeal dissemination has been reported in up to 58% of cases of spinal GBM. Leptomeningeal involvement of conus GBM has been reported in five cases (including our patient). Specifically, the majority of clinical manifestations of leptomeningeal dissemination are cranial nerve palsy, focal neurological dysfunction, elevated intracranial pressure, hydrocephalus, and pseudomeningitis, which can be misdiagnosed as tuberculous meningitis as in the present case.^[30] Leptomeningeal spread has been considered an incurable end-stage complication.^[9],[31]

Due to the rarity of this malignant tumor, the standard therapeutic strategy for this disease remains to be settled. Currently, resection of spinal cord lesions seems to be the only treatment option that can achieve a longer survival period.^[26],[32] Adjuvant chemotherapy and radiotherapy can usually improve the survival rate in low-grade invasive astrocytoma of the spinal cord. However, uniformly poor treatment outcomes of GBM of the conus medullaris have been reported. The overall survival time of patients afflicted with primary GBM in the conus medullaris ranges from 4 to 16 months with an average of 13.7 months after the diagnosis.^[7],[33] The survival rate tends to decrease with the leptomeningeal spread of the disease.^{[34],[35],[36]} Four previously reported cases and our patients with leptomeningeal dissemination have exhibited shorter survival periods of only 4.94 months on average (ranging from 2 to 9 months).^[30],[37] The prognosis of patients with conus GBM is mainly determined by meningeal seeding and/or cerebral metastases, which may indicate a rapidly evolving threat to life.

Although GBM of the conus medullaris that presents as intracranial hypertension is extremely rare, awareness of this unusual condition is crucial to its timely treatment and consideration of the clinical prognosis. Further clinical and basic scientific research is the keystone to learn more about the pathophysiology of this disease, estimate its progression, and develop effective treatment strategies to enhance the survival benefits.

Financial support and sponsorship

Nil.

Institutional review board statement

IRB review was waived owing to the retrospective nature of this case report.

Declaration of patient consent

The authors certify that they have obtained the patient consent form. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that her name and initial will not be published and due efforts will be made to conceal her identity.

Conflicts of interest

There are no conflicts of interest.

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