Clinical Neurology and Neurosurgery 123 (2014) 61–63
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Case report
Cervical intramedullary metastasis from cerebral glioblastoma multiforme: Case report Thomas Robert a, * , Rosa Martinez-Mañas a , Diego San Millán Ruíz b , Marc Morard a a b
Department of Neurosurgery, Hôpital de Sion, Réseau Santé Valais, Sion, Switzerland Department of Radiology, Hôpital de Sion, Réseau Santé Valais, Sion, Switzerland
A R T I C L E I N F O
Article history: Received 28 March 2014 Received in revised form 6 May 2014 Accepted 12 May 2014 Available online 21 May 2014 Keywords: Glioblastoma multiforme Intramedullary tumor Metastasis Case report
1. Introduction Cerebral glioblastoma multiforme (GBM) accounts for approximately 60% of all primary brain tumors in adults [1,4]. The standard of care for this tumor is surgical resection followed by chemotherapy and radiation therapy [5]. With this optimal treatment, the median survival time extends approximately from 9 to 12 months. In general, death is due to local progression of the tumor [3]. Metastasis of intracranial GBM by cerebro-spinal fluid (CSF) dissemination has been described with increased frequency in recent years [4]. Autopsy series suggest that 25% of patients with cerebral GBM have evidence of CSF dissemination [5]. Symptomatic intramedullary metastases from GBM are rarely reported. We report a case of symptomatic cervical intramedullary metastasis from a temporal GBM treated 23 months before. 2. Case report A 75-year-old man presented with a 3 weeks history of progressive weakness on the left side associated with walk disability. He was treated in our neurosurgical department for a right temporal glioblastoma multiforme 23 months before with complete resection followed by an oral chemotherapy
* Corresponding author. Present address: Department of Interventional Neuroradiology, Rothschild foundation Hospital, 25 rue Manin, 75019 Paris, France. Tel.: +33 781305266. E-mail address:
[email protected] (T. Robert). http://dx.doi.org/10.1016/j.clineuro.2014.05.009 0303-8467/ ã 2014 Elsevier B.V. All rights reserved.
(temozolomide) and external beam radiotherapy (60 Gy). After this treatment, he has been symptom free during 23 months without any recurrence of the right temporal lesion on serial follow-up magnetic resonance imaging. On his admission, the neurological examination showed cervical myelopathy with left sided weakness and general examination was normal. Cerebral MRI (Fig. 1) showed no recurrence of the temporal lesion with no contrast enhancement of the surgical cavity. Spinal MRI (Fig. 2) revealed the presence of an intramedullary lesion (26 8 6 mm) at the C3–C5 level predominant on the left side of the medulla. This lesion was isointense on T1-weighted images (WI) with enhancement after contrast administration. T2WI signal changes consistent with cord edema were also present in the cervical medulla. This cervical tumor was not present on a follow-up MRI realized 4 months before. One week later, surgical excision of the lesion was planned by laminectomy and midline myelotomy under intra-operative motor and sensitive evoked potential. Only a biopsy of the lesion could be realized because the tumor margin from the spinal cord was undefined. Midline myelotomy showed a grayish, elastic, soft tumor at the C3–C5 level. Histologically, intraspinal metastasis of GBM was diagnosed according to pathology analysis. The tumor consisted of a markedly pleomorphic neoplasm and was characterized by necrosis, atypical mitotic changes and endothelial proliferation. No comparison of genetic characteristics could be done between the two lesions. Postoperative course was marked by a rapidly progressive cervical myelopathy. One week after the surgery, the patient was unable to walk and presented a tetraparesis predominantly on the
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Fig. 1. Axial slices of cerebral pre (a) and post (b) gadolinium MR images showing right temporal cavity without sign of tumoral recurrence 18 months after the surgery.
left side. Regarding to the patient status, the histological diagnosis and the rapidity of the neurological degradation, we decided not to introduce chemotherapy or cervical radiation therapy. The patient died 3 months after the beginning of the neurological symptoms. 3. Discussion Glioblastoma multiforme is the most common primary malignant brain tumor with an incidence of 3/100,000 new patients per year [3]. The primary treatment modalities remain surgical excision followed by external beam radiation therapy and chemotherapy with a mean survival of about 12 months from the diagnosis. Despite these most aggressive treatments, only 5% of people survive during 5 years after the diagnosis [2]. The ability of supratentorial glioblastoma multiforme to metastasize along CSF pathways to the spinal cord was first described in 1931 by Cairns and Russell [4]. On autopsy series, CSF dissemination
occurs in 15–25% of cases of supratentorial GBM and up to 60% in case of infratentorial GBM [5]. The rarity of symptomatic metastasis of GBM suggests that the presence of metastasis of GBM occurs relatively late in the course of the disease [3]. Spinal GBM metastases are more common in the leptomeningeal spaces with predominance of upper lumbar and lumbosacral regions [1]. The nerve roots of the cauda equina, the nerve root sleeves and the fundus of the dural sac are also common locations. Only one case of cervical GBM metastasis is described in a metaanalysis published in 1992 [2] which summarized 22 cases of spinal metastases of intracerebral high grade glioma. The intramedullary location is also a rare finding for these lesions. Our case is atypical for the absence of recurrence of a cerebral GBM after a long period but the onset of a GBM metastasis in the cervical spine. We have not found a similar case in the literature, the typical history is the presence of metastasis of GBM when the cerebral tumor growing despite adequate treatment. The prognosis
Fig. 2. (a) Sagittal T1-weighted MR image shows isointense tumor at the level C3–C5. Post-gadolinium sagittal (b) MR images reveal homogeneous enhancement of the lesion.
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for patients who have developed symptomatic spinal GBM metastasis is poor with an average survival time of 3 months after an appropriate therapy has been started. There is no consensus for the treatment of metastasis of cerebral GBM. Surgical resection could be challenging for intramedullary glioblastoma because of the undefined tumor margins. Cytoreductive debulking of a large metastasis may be important to consider chemotherapy or radiation therapy [2]. Intravenous or intrathecal chemotherapy is always used but is more effective for extraneural metastases. Radiotherapy is the most commonly proposed treatment modality with 25–40 Gy delivered in 2.5 Gy by fraction. This treatment is only palliative with a temporary pain decreased with no improvement in neurological deficit [2–4]. An analysis of PubMed publications allows us to observe that the number of publications concerning metastases of cerebral GBM increase since 10–15 years. Mainly two reasons might be responsible for this increase in frequency. First, diagnostic tools have improved since computerized tomography (CT) and MRI are available; but better diagnostic techniques could not explain alone the increasing number of symptomatic metastases of GBM. Therefore, the prolonged survival time due to new chemotherapeutic agents and to new therapeutic protocols might be the second reason.
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4. Conclusion Our case highlights the importance of considering secondary localization of cerebral GBM. Intramedullary location is rare and considerably diminishes the survival time to 3–4 months. Treatment options are today ineffective and could not permit to prolong the life. New diagnostic tools and adjuvant therapy for cerebral high grade gliomas explain the increase of spinal metastases of GBM. References [1] Hamilton MG, Tranmer BI, Hagen NA. Supratentorial glioblastoma with spinal cord intramedullary metastasis. Can J Neurol Sci 1993;20:65–8. [2] Hubner F, Braun V, Richter HP. Case reports of symptomatic metastases in four patients with primary intracranial gliomas. Acta Neurochir (Wien) 2001;143:25–9. [3] Scoccianti S, Detti B, Meattini I, Iannalfi A, Sardaro A, Leonulli BG, et al. Symptomatic leptomeningeal and intramedullary metastases from intracranial glioblastoma multiforme: a case report. Tumori 2008;94:877–81. [4] Shah A, Redhu R, Nadkarni T, Goel A. Supratentorial glioblastoma multiforme with spinal metastases. J Craniovertebr Junction Spine 2010;1:126–9. [5] Vertosick Jr. FT, Selker RG. Brain stem and spinal metastases of supratentorial glioblastoma multiforme: a clinical series. Neurosurgery 1990;27:516–21 discussion 512–521.
