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|LETTERS TO EDITOR
|Year : 2018 | Volume
| Issue : 3 | Page : 854-857
Primary spinal cord glioblastoma metastasizing to the cerebellum: A missed entity
Akarsh Jayachandran1, Gandham E Jonathan1, Bimal Patel2, Krishna Prabhu1
1 Department of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu, India
2 Department of Neuropathology, Christian Medical College, Vellore, Tamil Nadu, India
|Date of Web Publication||15-May-2018|
Dr. Krishna Prabhu
Department of Neurosurgery, Christian Medical College, Vellore, Tamil Nadu
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Jayachandran A, Jonathan GE, Patel B, Prabhu K. Primary spinal cord glioblastoma metastasizing to the cerebellum: A missed entity. Neurol India 2018;66:854-7
A 31-year old male patient presented with back pain and progressive tightness in his lower limbs for the last 6 months, associated with urinary urgency and incontinence for 1 week prior to admission. On examination, he had a severe spastic paraparesis with graded sensory loss to touch and pain sensations below the T8 dermatome. He was in Nurick grade 5 stage at presentation. Magnetic resonance imaging (MRI) of the spine showed a well-defined, ovoid, mildly enhancing intramedullary lesion at the T10 level measuring approximately 2.0 × 0.9 cm with perilesional edema [Figure 1]. He underwent a T9, 10 laminectomy and radical excision of the intramedullary mass with motor evoked potential (MEP) monitoring. Peroperatively, we found that the cord was swollen and the tumor had a poor plane of cleavage with the surrounding cord. The tumor was radically excised. The histopathology was consistent with the diagnosis of a small cell glioblastoma. On microscopy, there were fragments of a hypercellular tumor composed of sheets and closely packed cords of small-to-medium-sized cells with mildly pleomorphic nuclei containing vesicular-to-dispersed chromatin, inconspicuous nucleoli, and scanty eosinophilic cytoplasm. There was brisk mitotic and apoptotic activity in the cells [Figure 2]. On immunohistochemistry, the tumor cells showed weak-to-patchy strong cytoplasmic and dot-like positivity for glial fibrillary acidic protein (GFAP), patchy positivity for S100 protein, and weak positivity for synaptophysin. The tumor cells were immunonegative for isocitrate dehydrogenase (IDH)-1 mutation with retained expression of ATRX.
|Figure 1: MRI thoracic spine T2-weighted axial images (a), T1-weighted contrast axial (b), and sagittal (c) images showing an oval 2 cm well-defined, intramedullary tumor with intense contrast enhancement, at the T10 level|
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|Figure 2: (a-i): Hematoxylin and eosin stained sections (a, ×100, b, ×400) show a hypercellular tumor composed of sheets and closely packed cords of small-to-medium-sized cells with mildly pleomorphic nuclei containing vesicular-to-dispersed chromatin, inconspicuous nucleoli, and scanty eosinophilic cytoplasm. There is brisk mitotic and apoptotic activity. Tumor cells show positivity for GFAP (c), weak positivity for synaptophysin (d), and are negative for EMA (e), pancytokeratin (f), and CD20 (g). Tumor cells are immunonegative for IDH-1 (i) and show retained expression of ATRX (h)|
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Postoperatively, he had significant improvement in his spasticity and bladder complaints. He received focal radiation therapy (RT) to the thoracic spinal cord along with temozolomide-based concurrent and adjuvant chemotherapy. During RT planning, the screening MRI brain revealed a non-enhancing 1.2 cm lesion in the right cerebellum, which did not show any features of a high-grade glioma [Figure 3]a, [Figure 3]b, [Figure 3]c. As the imaging was not suggestive of any malignancy, we decided to keep the patient on close follow-up. However, 7 months later, he presented with features of raised intracranial pressure and right cerebellar signs. A repeat MRI brain showed an increase in the right cerebellar hemispheric lesion suggestive of a metastasis or a high-grade glioma [Figure 3]d, [Figure 3]e, [Figure 3]f. Spinal screening at this time showed no residual tumor or tumor recurrence at the previous operative site. He subsequently underwent total excision of the right cerebellar lesion. Following surgery, he had a significant improvement in his headache and his gait also improved. Histopathology was consistent with small cell glioblastoma [Figure 4]. He received whole brain radiation therapy and temozolomide-based chemotherapy. He was disease free at a 10-month follow-up visit.
|Figure 3: (a-c) Radiotherapy planning MRI brain with gadolinium showed nonenhancing right cerebellar lesion without features of a high-grade neoplasm. (d-f) MRI brain and spine with contrast showing significant increase in the size of right cerebellar lesion with features of a high-grade glioma; spine screening showed no evidence of residual tumor at the previous operative site|
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|Figure 4: (a-f): Hematoxylin and eosin stained sections (a, ×100, b, ×400) show cerebellar tissue infiltrated by a hypercellular tumor composed of cells, as described in the above figure. Tumor cells show positivity for GFAP (c) and weak positivity for synaptophysin (d). Scattered tumor cells are positive for p53 (e). The MIB-1 labelling index is high (f)|
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Spinal intramedullary tumors account for approximately 5–10% of all spinal cord tumors. Primary spinal cord glioblastoma multiforme (sGBM) is a rare entity and comprises only 1.5% of primary intramedullary spinal cord tumors and 1–5% of all glioblastomas. Most sGBMs are drop metastases from cerebral glioblastomas. sGBM are more common in young patients and have a predilection for the cervical spine. Tumors in the cervical spine have a poor prognosis compared to those of the thoracic spine due to the morbidity caused by high cervical cord compression. sGBM rarely spreads to the brain. Tumors arising from the thoracic cord have a greater risk of metastasizing to the brain than those from the cervical cord. They tend to have a rapid progression of symptoms from the onset leading to death. These tumors commonly disseminate by the leptomeningeal route via cerebrospinal fluid (CSF) in the subarachnoid space and reach the arachnoid granulations and the surrounding cerebral cortex. These tumor cells can also block the basal cisterns, increase the protein content, and cause hydrocephalus., The most common sites for metastasis are the cerebellum, thalamus, septum pellucidum, and medulla.
The mainstay of therapy for this tumor is surgical resection followed by high-dose radiation; however, its infiltrative nature makes radical resection difficult. This is followed up with adjuvant temozolomide-based chemotherapy. There is also evidence to recommend whole spine and brain irradiation even in the absence of intracranial dissemination due to the nature of the disease, which can spread through the leptomeninges and CSF.,,,, A few recently published cases of primary spinal cord glioblastoma and their clinical outcomes are summarized in [Table 1].
|Table 1: Review of literature of recently published cases of primary spinal cord glioblastomas and their outcomes|
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Despite aggressive multimodality treatment, prognosis of sGBMs with intracranial metastases remains poor. The average mean survival is 10.4 months (range, 1–36 months). We should have irradiated the right cerebellar lesion along with whole spine radiation in the first sitting. This could have prevented the progression of the disease, necessitating a surgery at later date.
The incidence of brain metastasis from a sGBM is very rare. Careful evaluation using MRI of the brain along with entire spinal axis screening helps in detecting the presence of early metastasis. If metastatic lesions are detected early, they should be treated with radiation and adjuvant chemotherapy.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]