|Year : 2021 | Volume
| 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|
Dr. Haifeng Chen
Department of Neurosurgery, West China Hospital, Sichuan University, No. 37, Guo Xue Xiang, Chengdu, Sichuan Province
Source of Support: None, Conflict of Interest: None
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 2021 Dec 3];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., It accounts for only 0.2% of all central nervous system GBMs and 1.5% of all spinal cord tumors.,,, Spinal GBMs usually occur in the cervical and thoracic spine and rarely arise from the lumbar spine.,, Approximately, 14% of spinal GBMs originating from the conus medullaris have been documented in the literature. 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)|
Click here to view
|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)|
Click here to view
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. 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.,,,,,,,,,,,,,,,,,,,,,, The ratio of afflicted men-to-women is 19:10, revealing an obvious male predominance.
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. Six cases (20.7%, including our patient) developed symptoms of intracranial hypertension, and this was due to hydrocephalus in five cases.,,, The probable causes of hydrocephalus in cases of spinal GBM are elevation of CSF protein concentration, occlusion of CSF channels, arachnoiditis, and intratumoral hemorrhage. 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.,,
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. Leptomeningeal spread has been considered an incurable end-stage complication.,
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., 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., The survival rate tends to decrease with the leptomeningeal spread of the disease.,, 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)., 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
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.
| References|| |
Cacchione A, Mastronuzzi A, Cefalo MG, Colafati GS, Diomedi-Camassei F, Rizzi M, et al.
Pediatric spinal glioblastoma of the conus medullaris: A case report of long survival. Chin J Cancer 2016;35:44.
Andrews AA, Enriques L, Renaudin J, Tomiyasu U. Spinal intramedullary glioblastoma with intracranial seeding. Report of a case. Arch Neurol 1978;35:244-5.
Yanamadala V, Koffie RM, Shankar GM, Kumar JI, Buchlak QD, Puthenpura V, et al.
Spinal cord glioblastoma: 25 years of experience from a single institution. J Clin Neurosci 2016;27:138-41.
Goodarzi A, Thaci B, Toussi A, Karnati T, Kim K, Fragoso R. Glioblastoma multiforme of the conus medullaris – Management strategies and complications. World Neurosurg 2019. doi: 10.1016/j.wneu.2018.12.164.
Yang K, Man W, Jing L, Sun Z, Liang P, Wang J, et al.
Clinical features and outcomes of primary spinal cord glioblastoma: A single-center experience and literature review. World Neurosurg 2020;143:e157-65.
Scarrow AM, Rajendran P, Welch WC. Glioblastoma multiforme of the conus medullaris. Clin Neurol Neurosurg 2000;102:166-7.
Medhkour A, Chan M. Extremely rare glioblastoma multiforme of the conus medullaris with holocord and brain stem metastases, leading to cranial nerve deficit and respiratory failure: A case report and review of the literature. Surg Neurol 2005;63:576-82.
Sanborn MR, Pramick M, Brooks J, Welch WC. Glioblastoma multiforme in the adult conus medullaris. J Clin Neurosci 2011;18:842-3.
Konar SK, Maiti TK, Bir SC, Kalakoti P, Bollam P, Nanda A. Predictive factors determining the overall outcome of primary spinal glioblastoma multiforme: An integrative survival analysis. World Neurosurg 2016;86:341-8.e1-3.
Adams H, Avendaño J, Raza SM, Gokaslan ZL, Jallo GI, Quiñones-Hinojosa A. Prognostic factors and survival in primary malignant astrocytomas of the spinal cord: A population-based analysis from 1973 to 2007. Spine (Phila Pa 1976) 2012;37:E727-35.
Eden KC. Dissemination of a glioma of the spinal cord in the leptomeninges. Brain 1938;61:298-310.
O'Connell JE. The subarachnoid dissemination of spinal tumours. J Neurol Neurosurg Psychiatry 1946;9:55-62.
Tashiro K, Tachibana S, Tsura M. Clinicopathological studies of spinal cord neoplasm with disseminating intracranial metastasis possibly producing akinetics mutism. No To Shinkei 1976;28:1311-8.
Cohen AR, Wisoff JH, Allen JC, Epstein F. Malignant astrocytomas of the spinal cord. J Neurosurg 1989;70:50-4.
Kawanishi M, Kuroiwa T, Nagasawa S, Ohta T, Oketa M, Onomura T. A case of spinal glioblastoma with intracranial dissemination. No Shinkei Geka 1993;21:1109-12.
Shirato H, Kamada T, Hida K, Koyanagi I, Iwasaki Y, Miyasaka K, et al.
The role of radiotherapy in the management of spinal cord glioma. Int J Radiat Oncol Biol Phys 1995;33:323-8.
Strik HM, Effenberger O, Schäfer O, Risch U, Wickboldt J, Meyermann R. A case of spinal glioblastoma multiforme: Immunohistochemical study and review of the literature. J Neurooncol 2000;50:239-43.
Santi M, Mena H, Wong K, Koeller K, Olsen C, Rushing EJ. Spinal cord malignant astrocytomas. Clinicopathologic features in 36 cases. Cancer 2003;98:554-61.
Banczerowski P, Simó M, Sipos L, Slowik F, Benoist G, Veres R. Primary intramedullary glioblastoma multiforme of the spinal cord: Report of eight cases. Ideggyogy Sz 2003;56:28-32.
Stecco A, Quirico C, Giampietro A, Sessa G, Boldorini R, Carriero A. Glioblastoma multiforme of the conus medullaris in a child: Description of a case and literature review. AJNR Am J Neuroradiol 2005;26:2157-60.
Elsamaloty H, Zenooz NA, Mossa-Basha M. Glioblastoma multiforme (GBM) of the conus medullaris with brain and brain stem metastases. Eur J Radiol Extra 2006;58:59-62.
Bonde V, Balasubramaniam S, Goel A. Glioblastoma multiforme of the conus medullaris with holocordal spread. J Clin Neurosci 2008;15:601-3.
Choi WC, Lee JH, Lee SH. Spinal cord glioblastoma multiforme of conus medullaris masquerading as high lumbar disk herniation. Surg Neurol 2009;71:234-7.
Sun J, Wang Z, Li Z, Liu B. Microsurgical treatment and functional outcomes of multi-segment intramedullary spinal cord tumors. J Clin Neurosci 2009;16:666-71.
Mayer RR, Warmouth GM, Troxell M, Adesina AM, Kass JS. Glioblastoma multiforme of the conus medullaris in a 28-year-old female: A case report and review of the literature. Clin Neurol Neurosurg 2012;114:275-7.
Mori K, Imai S, Shimizu J, Taga T, Ishida M, Matsusue Y. Spinal glioblastoma multiforme of the conus medullaris with holocordal and intracranial spread in a child: A case report and review of the literature. Spine J 2012;12:e1-6.
Gee TS, Ghani AR, Idris B, Awang MS. Case report: A rare case of pediatric conus medularis glioblastoma multiforme. Med J Malaysia 2012;67:438-41.
Yan C, Kong X, Yin H, Wang Y, He H, Zhang H, et al.
Glioblastoma multiforme in conus medullaris with intracranial metastasis after postoperative adjuvant therapy. Medicine (Baltimore) 2017;96:e6500.
Lim S, Lee SJ, Rhim SC. Primary spinal cord astrocytoma presenting as intracranial hypertension: A case report. Korean J Spine 2012;9:272-4.
Birzu C, Tran S, Bielle F, Touat M, Mokhtari K, Younan N, et al.
Leptomeningeal spread in glioblastoma: Diagnostic and therapeutic challenges. Oncologist 2020;25:e1763-76.
Mandel JJ, Yust-Katz S, Cachia D, Wu J, Liu D, de Groot JF, et al.
Leptomeningeal dissemination in glioblastoma; an inspection of risk factors, treatment, and outcomes at a single institution. J Neurooncol 2014;120:597-605.
Shen CX, Wu JF, Zhao W, Cai ZW, Cai RZ, Chen CM. Primary spinal glioblastoma multiforme: A case report and review of the literature. Medicine (Baltimore) 2017;96:e6634.
Yi S, Choi S, Shin DA, Kim DS, Choi J, Ha Y, et al.
Impact of H3.3 K27M mutation on prognosis and survival of grade IV spinal cord glioma on the basis of new 2016 World Health Organization classification of the central nervous system. Neurosurgery 2019;84:1072-81.
Noh JH, Lee MH, Kim WS, Lim DH, Kim ST, Kong DS, et al.
Optimal treatment of leptomeningeal spread in glioblastoma: Analysis of risk factors and outcome. Acta Neurochir (Wien) 2015;157:569-76.
Schwartz C, Romagna A, Machegger L, Weiss L, Huemer F, Fastner G, et al.
Extensive leptomeningeal intracranial and spinal metastases in a patient with a supratentorial glioblastoma multiforme, IDH-wildtype. World Neurosurg 2018;120:442-7.
Nunn A, Polyzoidis S, Piechowski-Jozwiak B, Brazil L, Ashkan K. Primary glioblastoma multiforme of the conus medullaris with leptomeningeal metastasis. J Neurol Sci 2017;381:315-7.
Andersen BM, Miranda C, Hatzoglou V, DeAngelis LM, Miller AM. Leptomeningeal metastases in glioma: The Memorial Sloan Kettering Cancer Center experience. Neurology 2019;92:e2483-91.
[Figure 1], [Figure 2]