Journal of Clinical Neuroscience xxx (2014) xxx–xxx
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Case Report
Dorsally exophytic cervicomedullary glioblastoma Abhijit G. Warade, Basant K. Misra ⇑ Department of Neurosurgery & Gamma Knife Radiosurgery, P.D. Hinduja Hospital & Medical Research Centre, Veer Savarkar Marg, Mahim, Mumbai 400016, Maharashtra, India
a r t i c l e
i n f o
Article history: Received 25 December 2013 Accepted 1 January 2014 Available online xxxx Keywords: Cervical Exophytic Glioblastoma multiforme Intramedullary Surgery
a b s t r a c t Spinal glioblastoma multiforme is a rare entity comprising 1.5% of all spinal cord tumors. We report a 57year-old man presenting with a 1.5 month history of left sided radiculopathy, lower cranial nerve weakness and difficulty in walking. MRI of the brain and spine showed an exophytic intramedullary lesion extending from the cervicomedullary junction to the lower margin of C4. To our knowledge, we report the first patient with dorsally exophytic cervicomedullary and cervical intramedullary glioblastoma multiforme. Ó 2014 Elsevier Ltd. All rights reserved.
1. Introduction Spinal glioblastoma multiforme (GBM) is a rare entity comprising 1.5% of all spinal cord tumors and 1% to 5% of all GBM cases [1]. It is mostly seen during the second and third decades of life and shows a predilection for the thoracic region [1,2]. There are a few case reports of intramedullary GBM of the cervical spinal cord (C3–C5), with the tumor extending from the lower medulla to upper thoracic cord [1]. To our knowledge, our patient’s primary dorsally exophytic cervicomedullary intramedullary GBM is the first to be reported in the literature. 2. Case report A 57-year-old man presented with a 1.5 month history of left upper limb radiculopathy, hoarseness of voice, increased urinary frequency and difficulty in walking. On examination he had weakness of muscles supplied by the left spinal accessory nerve, wasting of the left upper limb, weakness of the left shoulder (abduction grade 3/5), exaggerated deep tendon reflexes and left ankle clonus. MRI showed an intramedullary lesion extending from the cervicomedullary junction to the lower margin of C4 (Fig. 1). Preoperative somatosensory evoked potentials were delayed bilaterally. He underwent midline suboccipital craniotomy, C1–C5 laminoplasty and radical decompression of the lesion (Fig. 2). The patient developed transient worsening of lower cranial nerve paresis, worsening weakness of the left upper limb and posterior column ⇑ Corresponding author. Tel.: +91 22 2444 7214; fax: +91 22 2445 515. E-mail address: [email protected] (B.K. Misra).
dysfunction. He was discharged on the tenth postoperative day with improved power and posterior column function. The histopathology was reported as GBM, World Health Organization grade IV, with an MIB-1 index of 8%. He was referred to the medical and radiation oncologists for further management. He received whole brain radiation therapy and spinal irradiation with adjuvant chemotherapy. He died within 3 months of surgery.
3. Discussion Astrocytoma is the most common intramedullary spinal cord tumor [3]. The ratio of low-grade to high-grade astrocytoma in the spinal cord is 3 to 1 [3–5]. Histopathologically, spinal and cerebral GBM are identical and both show strong immunohistochemical staining for p53 [1,6]. The relative rarity of the tumor in the spine can be explained by the difference in the masses of the two organs, as the absolute number of cells (including glial cells) in the spinal cord may be one-tenth of the brain [1,7]. The high rate of leptomeningeal spread has been attributed to the relatively thin parenchyma in the spinal cord, and hence, the short distance to the subarachnoid space [1,7]. Surgery should be radical, enabling procurement of adequate tissue for diagnosis, cytoreductive tumor debulking and relief of pain [8,9]. The recommended spinal radiation dose is 35 to 50 Gy and the dose of temozolomide is 75 mg/m2. In spite of all the available treatments, the outcome in spinal intramedullary GBM is very poor, with estimated survival being 6 to 16 months. This raises the question about the advisability of radical excision, that carries the risk of permanent neurological sequelae and impairment of the quality of the brief remaining life of the patient [1,8–13]. Intracra-
http://dx.doi.org/10.1016/j.jocn.2014.01.014 0967-5868/Ó 2014 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Warade AG, Misra BK. Dorsally exophytic cervicomedullary glioblastoma. J Clin Neurosci (2014), http://dx.doi.org/ 10.1016/j.jocn.2014.01.014
2
Case Report / Journal of Clinical Neuroscience xxx (2014) xxx–xxx
rates of mean survival [1,12,14,15]. The exophytic nature of the disease in our patient probably resulted in widespread metastasis and resulted in very short survival. Cervical GBM has a poorer outcome because of early involvement of the phrenic nerve nucleus (C4–C6) and vasomotor centers and the propensity for early brain metastasis [1]. GBM of the spinal cord is rare and has the same dismal prognosis as brain GBM. We report, to our knowledge, the first patient with exophytic cervicomedullary GBM. An exophytic spinal GBM probably predisposes patients to a worse outcome and very short survival. The benefits of radical excision of such a tumor with the inherent risk of neurological worsening may be debatable. Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. Fig. 1. Post-gadolinium T1-weighted (left) sagittal and (right) axial MRI showing a heterogeneously enhancing lesion surfacing on the left side extending from the cervicomedullary junction to lower margin of C4.
Fig. 2. Intraoperative photograph of the tumor showing the dorsally exophytic component of the tumor on the left side, exposed after dural incision. (This figure is available in colour at http://www.sciencedirect.com/.)
nial dissemination through cerebrospinal fluid channels is a common feature making the prognosis even worse [14]. Whole brain radiation therapy is recommended in addition to focal spinal radiation because of its high rate of spread to the brain and improved
References [1] Singh PK, Singh VK, Tomar J, et al. Spinal glioblastoma multiforme: unusual cause of post-traumatic tetraparesis. J Spinal Cord Med 2009;32:583–6. [2] Caroli E, Salvati M, Ferranate L. Spinal glioblastoma with brain relapse in a child: clinical considerations. Spinal Cord 2005;43:565–7. [3] Moraisi N, Mascarenhas L, Soares-Fernandes JP, et al. Primary spinal glioblastoma: a case report and review of the literature. Oncol Lett 2013;5: 992–6. [4] Medhkour A, Chan M. Extremely rare glioblastoma multiforme of the conus Medullaris with holocord and brain stem metastases, leading to cranial nerve defcit and respiratory failure: a case report and review of the literature. Surg Neurol 2005;63:576–82 [discussion 582-3]. [5] Stein BM. Surgery of intramedullary spinal cord tumors. Clin Neurosurg 1979;26:529–42. [6] Ramamurthi B, Rao SB. Tumours of the spinal cord and the cauda equine. In: Ramamurthi B, Tandon PN, editors. Textbook of neurosurgery. New Delhi: BI Churchill Living-Stone; 2012. p. 1181. [7] Yeung YF, Wong GK, Zhu XL, et al. Radiation induced spinal glioblastoma multiforme. Acta Oncol 2006;45:87–90. [8] Lober R, Sharma S, Bell B, et al. Pediatric primary intramedullary spinal cord glioblastoma. Rare Tumors 2010;2. e48. [9] Cohen AR, Wisoff JH, Allen JC, et al. Malignant astrocytomas of the spinal cord. J Neurosurg 1989;70:50–4. [10] Andrews AA, Enriques L, Renaudin J, et al. Spinal intramedullary glioblastoma with intracranial seeding. Arch Neurol 1978;35:244–5. [11] Chigasaki H. Surgical treatment of malignant spinal tumors. Shinkei Shinpo 1978;20:90–9. [12] Takara E, Ide M, Yamamoto M, et al. A case of the intracranial and spinal dissemination of primary spinal glioma. No Shinkei Geka 1985;13:301–5. [13] Minehan KJ, Brown PD, Scheithauer BW. Prognosis and treatment of spinal cord astrocytoma. Int J Radiat Oncol Biol Phys 2009;73:727–33. [14] Asano N, Kitamura K, Seo Y, et al. Spinal cord glioblastoma multiforme with intracranial dissemination case report. Neurol Med Chir (Tokyo) 1990;30: 489–94. [15] Shirato H, Kamada T, Hida K. The role of radiotherapy in the management of spinal cord glioma. Int J Radiat Oncol Biol Phys 1995;33:323–8.
Please cite this article in press as: Warade AG, Misra BK. Dorsally exophytic cervicomedullary glioblastoma. J Clin Neurosci (2014), http://dx.doi.org/ 10.1016/j.jocn.2014.01.014
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Case Report
Dorsally exophytic cervicomedullary glioblastoma Abhijit G. Warade, Basant K. Misra ⇑ Department of Neurosurgery & Gamma Knife Radiosurgery, P.D. Hinduja Hospital & Medical Research Centre, Veer Savarkar Marg, Mahim, Mumbai 400016, Maharashtra, India
a r t i c l e
i n f o
Article history: Received 25 December 2013 Accepted 1 January 2014 Available online xxxx Keywords: Cervical Exophytic Glioblastoma multiforme Intramedullary Surgery
a b s t r a c t Spinal glioblastoma multiforme is a rare entity comprising 1.5% of all spinal cord tumors. We report a 57year-old man presenting with a 1.5 month history of left sided radiculopathy, lower cranial nerve weakness and difficulty in walking. MRI of the brain and spine showed an exophytic intramedullary lesion extending from the cervicomedullary junction to the lower margin of C4. To our knowledge, we report the first patient with dorsally exophytic cervicomedullary and cervical intramedullary glioblastoma multiforme. Ó 2014 Elsevier Ltd. All rights reserved.
1. Introduction Spinal glioblastoma multiforme (GBM) is a rare entity comprising 1.5% of all spinal cord tumors and 1% to 5% of all GBM cases [1]. It is mostly seen during the second and third decades of life and shows a predilection for the thoracic region [1,2]. There are a few case reports of intramedullary GBM of the cervical spinal cord (C3–C5), with the tumor extending from the lower medulla to upper thoracic cord [1]. To our knowledge, our patient’s primary dorsally exophytic cervicomedullary intramedullary GBM is the first to be reported in the literature. 2. Case report A 57-year-old man presented with a 1.5 month history of left upper limb radiculopathy, hoarseness of voice, increased urinary frequency and difficulty in walking. On examination he had weakness of muscles supplied by the left spinal accessory nerve, wasting of the left upper limb, weakness of the left shoulder (abduction grade 3/5), exaggerated deep tendon reflexes and left ankle clonus. MRI showed an intramedullary lesion extending from the cervicomedullary junction to the lower margin of C4 (Fig. 1). Preoperative somatosensory evoked potentials were delayed bilaterally. He underwent midline suboccipital craniotomy, C1–C5 laminoplasty and radical decompression of the lesion (Fig. 2). The patient developed transient worsening of lower cranial nerve paresis, worsening weakness of the left upper limb and posterior column ⇑ Corresponding author. Tel.: +91 22 2444 7214; fax: +91 22 2445 515. E-mail address: [email protected] (B.K. Misra).
dysfunction. He was discharged on the tenth postoperative day with improved power and posterior column function. The histopathology was reported as GBM, World Health Organization grade IV, with an MIB-1 index of 8%. He was referred to the medical and radiation oncologists for further management. He received whole brain radiation therapy and spinal irradiation with adjuvant chemotherapy. He died within 3 months of surgery.
3. Discussion Astrocytoma is the most common intramedullary spinal cord tumor [3]. The ratio of low-grade to high-grade astrocytoma in the spinal cord is 3 to 1 [3–5]. Histopathologically, spinal and cerebral GBM are identical and both show strong immunohistochemical staining for p53 [1,6]. The relative rarity of the tumor in the spine can be explained by the difference in the masses of the two organs, as the absolute number of cells (including glial cells) in the spinal cord may be one-tenth of the brain [1,7]. The high rate of leptomeningeal spread has been attributed to the relatively thin parenchyma in the spinal cord, and hence, the short distance to the subarachnoid space [1,7]. Surgery should be radical, enabling procurement of adequate tissue for diagnosis, cytoreductive tumor debulking and relief of pain [8,9]. The recommended spinal radiation dose is 35 to 50 Gy and the dose of temozolomide is 75 mg/m2. In spite of all the available treatments, the outcome in spinal intramedullary GBM is very poor, with estimated survival being 6 to 16 months. This raises the question about the advisability of radical excision, that carries the risk of permanent neurological sequelae and impairment of the quality of the brief remaining life of the patient [1,8–13]. Intracra-
http://dx.doi.org/10.1016/j.jocn.2014.01.014 0967-5868/Ó 2014 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Warade AG, Misra BK. Dorsally exophytic cervicomedullary glioblastoma. J Clin Neurosci (2014), http://dx.doi.org/ 10.1016/j.jocn.2014.01.014
2
Case Report / Journal of Clinical Neuroscience xxx (2014) xxx–xxx
rates of mean survival [1,12,14,15]. The exophytic nature of the disease in our patient probably resulted in widespread metastasis and resulted in very short survival. Cervical GBM has a poorer outcome because of early involvement of the phrenic nerve nucleus (C4–C6) and vasomotor centers and the propensity for early brain metastasis [1]. GBM of the spinal cord is rare and has the same dismal prognosis as brain GBM. We report, to our knowledge, the first patient with exophytic cervicomedullary GBM. An exophytic spinal GBM probably predisposes patients to a worse outcome and very short survival. The benefits of radical excision of such a tumor with the inherent risk of neurological worsening may be debatable. Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. Fig. 1. Post-gadolinium T1-weighted (left) sagittal and (right) axial MRI showing a heterogeneously enhancing lesion surfacing on the left side extending from the cervicomedullary junction to lower margin of C4.
Fig. 2. Intraoperative photograph of the tumor showing the dorsally exophytic component of the tumor on the left side, exposed after dural incision. (This figure is available in colour at http://www.sciencedirect.com/.)
nial dissemination through cerebrospinal fluid channels is a common feature making the prognosis even worse [14]. Whole brain radiation therapy is recommended in addition to focal spinal radiation because of its high rate of spread to the brain and improved
References [1] Singh PK, Singh VK, Tomar J, et al. Spinal glioblastoma multiforme: unusual cause of post-traumatic tetraparesis. J Spinal Cord Med 2009;32:583–6. [2] Caroli E, Salvati M, Ferranate L. Spinal glioblastoma with brain relapse in a child: clinical considerations. Spinal Cord 2005;43:565–7. [3] Moraisi N, Mascarenhas L, Soares-Fernandes JP, et al. Primary spinal glioblastoma: a case report and review of the literature. Oncol Lett 2013;5: 992–6. [4] Medhkour A, Chan M. Extremely rare glioblastoma multiforme of the conus Medullaris with holocord and brain stem metastases, leading to cranial nerve defcit and respiratory failure: a case report and review of the literature. Surg Neurol 2005;63:576–82 [discussion 582-3]. [5] Stein BM. Surgery of intramedullary spinal cord tumors. Clin Neurosurg 1979;26:529–42. [6] Ramamurthi B, Rao SB. Tumours of the spinal cord and the cauda equine. In: Ramamurthi B, Tandon PN, editors. Textbook of neurosurgery. New Delhi: BI Churchill Living-Stone; 2012. p. 1181. [7] Yeung YF, Wong GK, Zhu XL, et al. Radiation induced spinal glioblastoma multiforme. Acta Oncol 2006;45:87–90. [8] Lober R, Sharma S, Bell B, et al. Pediatric primary intramedullary spinal cord glioblastoma. Rare Tumors 2010;2. e48. [9] Cohen AR, Wisoff JH, Allen JC, et al. Malignant astrocytomas of the spinal cord. J Neurosurg 1989;70:50–4. [10] Andrews AA, Enriques L, Renaudin J, et al. Spinal intramedullary glioblastoma with intracranial seeding. Arch Neurol 1978;35:244–5. [11] Chigasaki H. Surgical treatment of malignant spinal tumors. Shinkei Shinpo 1978;20:90–9. [12] Takara E, Ide M, Yamamoto M, et al. A case of the intracranial and spinal dissemination of primary spinal glioma. No Shinkei Geka 1985;13:301–5. [13] Minehan KJ, Brown PD, Scheithauer BW. Prognosis and treatment of spinal cord astrocytoma. Int J Radiat Oncol Biol Phys 2009;73:727–33. [14] Asano N, Kitamura K, Seo Y, et al. Spinal cord glioblastoma multiforme with intracranial dissemination case report. Neurol Med Chir (Tokyo) 1990;30: 489–94. [15] Shirato H, Kamada T, Hida K. The role of radiotherapy in the management of spinal cord glioma. Int J Radiat Oncol Biol Phys 1995;33:323–8.
Please cite this article in press as: Warade AG, Misra BK. Dorsally exophytic cervicomedullary glioblastoma. J Clin Neurosci (2014), http://dx.doi.org/ 10.1016/j.jocn.2014.01.014
