- Email: [email protected]
Supratentorial pure cortical ependymoma
Case Reports / Journal of Clinical Neuroscience 19 (2012) 1453–1455 7. Barth A, Pizzolato GP, Berney J. Intramedullary meningeal melanocytoma. Neurochirurgie 1993;39:188–94. 8. Chen CJ, Hsu YI, Ho YS, et al. Intracranial meningeal melanocytoma: CT and MRI. Neuroradiology 1997;39:811–4. 9. Glick R, Baker C, Husain S, et al. Primary melanocytomas of the spinal cord: a report of seven cases. Clin Neuropathol 1997;16:127–32. 10. Iiada M, Llena JF, Suarez MA, et al. Two cases of spinal meningeal melanocytoma. Brain Tumor Pathol 2002;19:41–5. 11. Delhaye M, Menei P, Rousselet MC, et al. A case of intramedullary primary melanocytic tumor: meningeal melanocytoma or malignant melanoma? Neurochirurgie 2001;47:133–6.
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12. Turhan T, Oner K, Yurtseven T, et al. Spinal meningeal melanocytoma. Report of two cases and review of the literature. J Neurosurg 2004;100:287–90. 13. Horn EM, Nakaji P, Coons SW, et al. Surgical treatment of intramedullary spinal cord melanocytomas. J Neurosurg Spine 2008;9:48–54. 14. Chacko G, Rajshekar V. Thoracic intramedullary melanocytoma with long term follow-up. J Neurosurg Spine 2008;9:589–92. 15. Perrini P, Caniglia M, Pieroni M, et al. Malignant transformation of intramedullary melanocytoma: case report. Neurosurgery 2010;67:E867–869.
doi:http://dx.doi.org10.1016/j.jocn.2012.02.009
Supratentorial pure cortical ependymoma Satoshi Nakamizo a, Takashi Sasayama a,⇑, Takeshi Kondoh a, Satoshi Inoue a, Ryoji Shiomi a, Hirotomo Tanaka a, Masamitsu Nishihara a, Katu Mizukawa a, Keiichiro Uehara b, Yu Usami b, Eiji Kohmura a a b
Department of Neurosurgery, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe 650-0017, Japan Department of Pathology, Kobe University Graduate School of Medicine, Kusunoki-cho, Chuo-ku, Kobe, Japan
a r t i c l e
i n f o
Article history: Received 14 June 2011 Accepted 10 September 2011
Keywords: Cortical ependymoma Seizure Supratentorial
a b s t r a c t Ependymoma usually occurs in the lateral or the fourth ventricle. Supratentorial extraventricular ependymoma is relatively rare. However, extraventricular ependymoma located at the cerebral cortex is extremely rare. We treated a 20-year-old woman who presented with generalized seizures. Cranial CT scan revealed a calcified mass in the left precentral gyrus. MRI confirmed an extraventricular, 12-mmdiameter intracortical mass. After gadolinium injection, tumor enhancement was mild and heterogeneous. The tumor was totally resected without neurological deterioration. Histological features were consistent with ependymoma, forming perivascular pseudorosettes without anaplastic figures. Immunohistochemistry showed positive staining for glial fibrillary acidic protein, S-100, and epithelial membrane antigen. A diagnosis of ependymoma of World Health Organization grade II was made. The patient has not had a seizure since the operation. There has been no clinical or radiologic evidence of recurrence during a 16-month postoperative follow-up. Ó 2012 Elsevier Ltd. All rights reserved.
1. Introduction Ependymomas occur most commonly in the fourth ventricle in children and in the fourth ventricle and spinal cord of adults.1 Ependymomas can also be supratentorial in location and associated with the third or lateral ventricle, or they may be centered within the subcortical white matter without a direct connection to the ventricular system.2 However, ependymomas that arise within the cerebral cortex are very rare and are referred to as ‘‘pure cortical ependymomas’’.3–7 2. Case Report We treated a 20-year-old woman who presented with a generalized seizure. There was no significant family or medical history. A CT scan revealed a cortical hypodense lesion, approximately 12 mm in diameter, in the precentral gyrus, with calcification (Fig. 1A). On MRI, the lesion appeared somewhat ill-defined and isointense at the periphery, and hypointense centrally on T1weighted MRI (Fig. 1B), and homogeneously slightly hyperintense at the periphery and centrally hypointense on T2-weighted MRI (Fig. 1C). After the injection of gadolinium, enhancement was mild and heterogeneous (Fig. 1D–F). The tumor had no anatomical connection with the wall of the lateral ventricle. Preoperative neurological examination results were normal. A left frontoparietal ⇑ Corresponding author. Tel.: +81 78 382 5966; fax: +81 78 382 5979. E-mail address: [email protected] (T. Sasayama).
craniotomy was performed, and the tumor was totally removed. The tumor was located immediately subpially and was well demarcated from the surrounding parenchyma. Histologically, the tumor cells were arranged around vessels, and there were radially oriented cell processes directed to the vessels producing the perivascular pseudorosettes classically seen in ependymoma (Fig. 2A,B). True ependymal rosettes were not seen. Mitotic activity was quite low (mitotic index: 1 per 10 high-power fields) (Supplementary Fig. 1). Necrosis and vascular endothelial proliferation were absent. In some areas, there were oligodendroglioma-like cells that had round nuclei and clear cytoplasm (Fig. 2C). The lesion contained calcifications (Fig. 2C). Immunohistochemical study revealed that the tumor cells were positive for glial fibrillary acidic protein (GFAP) (Fig. 2D), S-100 protein, Olig-2, and vimentin. The epithelial membrane antigen (EMA) immunostain showed perinuclear dots in scattered cells (Fig. 2E). The MIB-1 labeling index was 4% to 5%, on average (Fig. 2F), but in some areas it was as high as 10%. In addition, tumor cells express Jugged1 and Ephb3 strongly (Supplementary Fig. 2), both of which are known to be expressed in supratentorial ependymomas. Fluorescence in situ hybridization (FISH) analysis was performed, which did not reveal gain or loss of chromosome 1q (Supplementary Fig. 2). A diagnosis of ependymoma (World Health Organization [WHO] grade II) was made. The patient has not had a seizure since surgery. In addition, there has been no clinical or radiologic evidence of recurrence during a 16-month postoperative period.
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Case Reports / Journal of Clinical Neuroscience 19 (2012) 1453–1455
Fig. 1. (A) Axial CT scan showing a calcified mass in the left precentral gyrus. (B–D, axial; E, coronal; F, sagittal) MRI showing the tumor at the cerebral cortex. The tumor was (B) isointense at the periphery and centrally hypointense on T1-weighted MRI and (C) slightly hyperintense at the periphery and centrally hypointense on T2-weighted MRI. (D–F) After the injection of gadolinium, enhancement was mild and heterogeneous.
Fig. 2. (A–C) Photomicrographs showing (A, 100) uniform ependymal cells and perivascular pseudorosettes (no true ependymal rosettes were seen), (B, 400) showing tumor cells and radially oriented cell processes directed to the vessels, and (C, 200) showing calcifications and clear-cell histology in some areas (hematoxylin and eosin stain). (D–F) Immunohistochemistry showing (D, 200) tumor cells staining positive for glial fibrillary acidic protein and (E, 100) epithelial membrane antigen). (F, 100) The MIB-1 labeling index was 4% to 5% on average.
3. Discussion Supratentorial cortical ependymomas are rare. To our knowledge, only 17 patients with supratentorial cortical ependymomas,
including the present patient, have been reported3–7 (Supplementary Table 1). The mean age of the reported patients was 29 years, which is older than the average age of patients with general supratentorial ependymomas. Seizures and focal neurological deficits are common
Case Reports / Journal of Clinical Neuroscience 19 (2012) 1453–1455
in supratentorial cortical ependymomas. The tumor was located in the frontal lobe in 10 patients, the parietal lobe in five, the temporal lobe in one, and the occipital lobe in one. In eight of the 17 patients, cyst formation could be seen. Furthermore, five patients had anaplastic ependymomas (WHO grade III). Given the presence of radiating perivascular processes and the ill-defined location, cortical ependymoma resemble astroblastoma. Astroblastomas are usually supratentorial and superficial, as are cortical ependymomas. Histologically, however, the perivascular cytoplasmic processes of astroblastoma are more robust and broad-based compared with ependymomas.8 In addition, the immunohistochemical (IHC) stain for EMA is either negative or only focal in astroblasomas. Recently, a new entity, designated ‘‘monomorphous angiocentric glioma’’, was described as a neoplasm with similar features to cortical ependymomas.9 Angiocentric gliomas are superficial tumors occurring in children and young adults. They are epileptogenic and generally characterized by indolent behavior. Radiologically, they tend to be ill defined and non-enhancing. Histologically, the constituent cells are monomorphous and angiocentric, with the perivascular pseudorosettes. In some previous reports, they were reported to demonstrate characteristic ‘‘dot-like’’ EMA staining. Therefore, the presence of common features between cortical ependymoma and angiocentric glioma is extensively debated in the neuropathological literature.10 Lehman defined criteria to distinguish cortical ependymoma and angiocentric glioma.8 In our patient there were no non-radial, circumferential or longitudinal angiocentric growth patterns and bipolar spindle cells, which are typically seen in angiocentric glioma. Thus, cortical ependymoma was favored. The pathogenesis of cortical ependymoma is unclear. Recently, it has been shown that ependymomas from different parts of the central nervous system are molecularly distinct diseases. Taylor et al. found that supratentorial ependymomas express elevated levels of the Ephb–Ephrin and Notch cell signaling system compared with infratentorial or spinal ependymomas.11 In the present patient, Jugged1 and Ephb3 were strongly expressed in tumor cells. Therefore, it is possible that the tumorigenesis of cortical ependymoma is genetically similar to supratentorial intraventricular or extraventricular ependymomas. It is generally straightforward to perform gross total resection of cortical ependymoma because of the superficial location and doi:http://dx.doi.org10.1016/j.jocn.2011.09.039
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distinct margin. Most authors agree that prognosis is significantly correlated with the extent of resection.12 The need for postoperative local radiotherapy to control ependymomas is a controversial issue. Postoperative radiotherapy should be considered for highgrade lesions and for tumors located in critical areas whose removal is incomplete.12 In the present patient, postoperative radiotherapy was not performed because there were no anaplastic features and the tumor was totally removed. However, because the MIB-1 index was relatively high, careful follow-up by MRI is required.
Appendix A. Supplementary material Supplementary data associated with this article can be found, in the online version, at doi:10.1016/j.jocn.2011.09.039.
References 1. McLendon RE, D Wiestler O, Kros JM. WHO Classification of Tumours of the Central Nervous System. 4th ed. Lyon: IARC Press; 2007. 2. Vinchon M, Soto-Ares G, Riffaud L. Supratentorial ependymoma in children. Pediatr Neurosurg 2001;34:77–87. 3. Roncaroli F, Consales A, Fioravanti A. Supratentorial cortical ependymoma: report of three cases. Neurosurgery 2005;57:E192. 4. Miyazawa T, Hirose T, Nakanishi K. Supratentorial ectopic cortical ependymoma occurring with intratumoral hemorrhage. Brain Tumor Pathol 2007;24:35–40. 5. Lehman NL. Patterns of brain infiltration and secondary structure formation in supratentorial ependymal tumors. J Neuropathol Exp Neurol 2008;67:900–10. 6. Yadav YR, Neha, Chandrakar SK. Pure cortical supratentorial extraventricular ependymoma. Neurol India 2009;57:213–5. 7. Van Gompel JJ, Koeller KK, Meyer FB, et al. Cortical ependymoma: an unusual epileptogenic lesion. J Neurosurg 2011;114:1187–94. 8. Lehman NL. Central nervous system tumors with ependymal features: a broadened spectrum of primarily ependymal differentiation? J Neuropathol Exp Neurol 2008;67:177–88. 9. Wang M, Tihan T, Rojiani AM. Monomorphous angiocentric glioma: a distinctive epileptogenic neoplasm with features of infiltrating astrocytoma and ependymoma. J Neuropathol Exp Neurol 2005;64:875–81. 10. Lum DJ, Halliday W, Watson M. Cortical ependymoma or monomorphous angiocentric glioma? Neuropathology 2008;28:81–6. 11. Taylor MD, Poppleton H, Fuller C. Radial glia cells are candidate stem cells of ependymoma. Cancer Cell 2005;8:323–35. 12. Palma L, Celli P, Mariottini A. The importance of surgery in supratentorial ependymomas. Long-term survival in a series of 23 cases. Childs Nerv Syst 2000;16:170–5.
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12. Turhan T, Oner K, Yurtseven T, et al. Spinal meningeal melanocytoma. Report of two cases and review of the literature. J Neurosurg 2004;100:287–90. 13. Horn EM, Nakaji P, Coons SW, et al. Surgical treatment of intramedullary spinal cord melanocytomas. J Neurosurg Spine 2008;9:48–54. 14. Chacko G, Rajshekar V. Thoracic intramedullary melanocytoma with long term follow-up. J Neurosurg Spine 2008;9:589–92. 15. Perrini P, Caniglia M, Pieroni M, et al. Malignant transformation of intramedullary melanocytoma: case report. Neurosurgery 2010;67:E867–869.
doi:http://dx.doi.org10.1016/j.jocn.2012.02.009
Supratentorial pure cortical ependymoma Satoshi Nakamizo a, Takashi Sasayama a,⇑, Takeshi Kondoh a, Satoshi Inoue a, Ryoji Shiomi a, Hirotomo Tanaka a, Masamitsu Nishihara a, Katu Mizukawa a, Keiichiro Uehara b, Yu Usami b, Eiji Kohmura a a b
Department of Neurosurgery, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe 650-0017, Japan Department of Pathology, Kobe University Graduate School of Medicine, Kusunoki-cho, Chuo-ku, Kobe, Japan
a r t i c l e
i n f o
Article history: Received 14 June 2011 Accepted 10 September 2011
Keywords: Cortical ependymoma Seizure Supratentorial
a b s t r a c t Ependymoma usually occurs in the lateral or the fourth ventricle. Supratentorial extraventricular ependymoma is relatively rare. However, extraventricular ependymoma located at the cerebral cortex is extremely rare. We treated a 20-year-old woman who presented with generalized seizures. Cranial CT scan revealed a calcified mass in the left precentral gyrus. MRI confirmed an extraventricular, 12-mmdiameter intracortical mass. After gadolinium injection, tumor enhancement was mild and heterogeneous. The tumor was totally resected without neurological deterioration. Histological features were consistent with ependymoma, forming perivascular pseudorosettes without anaplastic figures. Immunohistochemistry showed positive staining for glial fibrillary acidic protein, S-100, and epithelial membrane antigen. A diagnosis of ependymoma of World Health Organization grade II was made. The patient has not had a seizure since the operation. There has been no clinical or radiologic evidence of recurrence during a 16-month postoperative follow-up. Ó 2012 Elsevier Ltd. All rights reserved.
1. Introduction Ependymomas occur most commonly in the fourth ventricle in children and in the fourth ventricle and spinal cord of adults.1 Ependymomas can also be supratentorial in location and associated with the third or lateral ventricle, or they may be centered within the subcortical white matter without a direct connection to the ventricular system.2 However, ependymomas that arise within the cerebral cortex are very rare and are referred to as ‘‘pure cortical ependymomas’’.3–7 2. Case Report We treated a 20-year-old woman who presented with a generalized seizure. There was no significant family or medical history. A CT scan revealed a cortical hypodense lesion, approximately 12 mm in diameter, in the precentral gyrus, with calcification (Fig. 1A). On MRI, the lesion appeared somewhat ill-defined and isointense at the periphery, and hypointense centrally on T1weighted MRI (Fig. 1B), and homogeneously slightly hyperintense at the periphery and centrally hypointense on T2-weighted MRI (Fig. 1C). After the injection of gadolinium, enhancement was mild and heterogeneous (Fig. 1D–F). The tumor had no anatomical connection with the wall of the lateral ventricle. Preoperative neurological examination results were normal. A left frontoparietal ⇑ Corresponding author. Tel.: +81 78 382 5966; fax: +81 78 382 5979. E-mail address: [email protected] (T. Sasayama).
craniotomy was performed, and the tumor was totally removed. The tumor was located immediately subpially and was well demarcated from the surrounding parenchyma. Histologically, the tumor cells were arranged around vessels, and there were radially oriented cell processes directed to the vessels producing the perivascular pseudorosettes classically seen in ependymoma (Fig. 2A,B). True ependymal rosettes were not seen. Mitotic activity was quite low (mitotic index: 1 per 10 high-power fields) (Supplementary Fig. 1). Necrosis and vascular endothelial proliferation were absent. In some areas, there were oligodendroglioma-like cells that had round nuclei and clear cytoplasm (Fig. 2C). The lesion contained calcifications (Fig. 2C). Immunohistochemical study revealed that the tumor cells were positive for glial fibrillary acidic protein (GFAP) (Fig. 2D), S-100 protein, Olig-2, and vimentin. The epithelial membrane antigen (EMA) immunostain showed perinuclear dots in scattered cells (Fig. 2E). The MIB-1 labeling index was 4% to 5%, on average (Fig. 2F), but in some areas it was as high as 10%. In addition, tumor cells express Jugged1 and Ephb3 strongly (Supplementary Fig. 2), both of which are known to be expressed in supratentorial ependymomas. Fluorescence in situ hybridization (FISH) analysis was performed, which did not reveal gain or loss of chromosome 1q (Supplementary Fig. 2). A diagnosis of ependymoma (World Health Organization [WHO] grade II) was made. The patient has not had a seizure since surgery. In addition, there has been no clinical or radiologic evidence of recurrence during a 16-month postoperative period.
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Case Reports / Journal of Clinical Neuroscience 19 (2012) 1453–1455
Fig. 1. (A) Axial CT scan showing a calcified mass in the left precentral gyrus. (B–D, axial; E, coronal; F, sagittal) MRI showing the tumor at the cerebral cortex. The tumor was (B) isointense at the periphery and centrally hypointense on T1-weighted MRI and (C) slightly hyperintense at the periphery and centrally hypointense on T2-weighted MRI. (D–F) After the injection of gadolinium, enhancement was mild and heterogeneous.
Fig. 2. (A–C) Photomicrographs showing (A, 100) uniform ependymal cells and perivascular pseudorosettes (no true ependymal rosettes were seen), (B, 400) showing tumor cells and radially oriented cell processes directed to the vessels, and (C, 200) showing calcifications and clear-cell histology in some areas (hematoxylin and eosin stain). (D–F) Immunohistochemistry showing (D, 200) tumor cells staining positive for glial fibrillary acidic protein and (E, 100) epithelial membrane antigen). (F, 100) The MIB-1 labeling index was 4% to 5% on average.
3. Discussion Supratentorial cortical ependymomas are rare. To our knowledge, only 17 patients with supratentorial cortical ependymomas,
including the present patient, have been reported3–7 (Supplementary Table 1). The mean age of the reported patients was 29 years, which is older than the average age of patients with general supratentorial ependymomas. Seizures and focal neurological deficits are common
Case Reports / Journal of Clinical Neuroscience 19 (2012) 1453–1455
in supratentorial cortical ependymomas. The tumor was located in the frontal lobe in 10 patients, the parietal lobe in five, the temporal lobe in one, and the occipital lobe in one. In eight of the 17 patients, cyst formation could be seen. Furthermore, five patients had anaplastic ependymomas (WHO grade III). Given the presence of radiating perivascular processes and the ill-defined location, cortical ependymoma resemble astroblastoma. Astroblastomas are usually supratentorial and superficial, as are cortical ependymomas. Histologically, however, the perivascular cytoplasmic processes of astroblastoma are more robust and broad-based compared with ependymomas.8 In addition, the immunohistochemical (IHC) stain for EMA is either negative or only focal in astroblasomas. Recently, a new entity, designated ‘‘monomorphous angiocentric glioma’’, was described as a neoplasm with similar features to cortical ependymomas.9 Angiocentric gliomas are superficial tumors occurring in children and young adults. They are epileptogenic and generally characterized by indolent behavior. Radiologically, they tend to be ill defined and non-enhancing. Histologically, the constituent cells are monomorphous and angiocentric, with the perivascular pseudorosettes. In some previous reports, they were reported to demonstrate characteristic ‘‘dot-like’’ EMA staining. Therefore, the presence of common features between cortical ependymoma and angiocentric glioma is extensively debated in the neuropathological literature.10 Lehman defined criteria to distinguish cortical ependymoma and angiocentric glioma.8 In our patient there were no non-radial, circumferential or longitudinal angiocentric growth patterns and bipolar spindle cells, which are typically seen in angiocentric glioma. Thus, cortical ependymoma was favored. The pathogenesis of cortical ependymoma is unclear. Recently, it has been shown that ependymomas from different parts of the central nervous system are molecularly distinct diseases. Taylor et al. found that supratentorial ependymomas express elevated levels of the Ephb–Ephrin and Notch cell signaling system compared with infratentorial or spinal ependymomas.11 In the present patient, Jugged1 and Ephb3 were strongly expressed in tumor cells. Therefore, it is possible that the tumorigenesis of cortical ependymoma is genetically similar to supratentorial intraventricular or extraventricular ependymomas. It is generally straightforward to perform gross total resection of cortical ependymoma because of the superficial location and doi:http://dx.doi.org10.1016/j.jocn.2011.09.039
1455
distinct margin. Most authors agree that prognosis is significantly correlated with the extent of resection.12 The need for postoperative local radiotherapy to control ependymomas is a controversial issue. Postoperative radiotherapy should be considered for highgrade lesions and for tumors located in critical areas whose removal is incomplete.12 In the present patient, postoperative radiotherapy was not performed because there were no anaplastic features and the tumor was totally removed. However, because the MIB-1 index was relatively high, careful follow-up by MRI is required.
Appendix A. Supplementary material Supplementary data associated with this article can be found, in the online version, at doi:10.1016/j.jocn.2011.09.039.
References 1. McLendon RE, D Wiestler O, Kros JM. WHO Classification of Tumours of the Central Nervous System. 4th ed. Lyon: IARC Press; 2007. 2. Vinchon M, Soto-Ares G, Riffaud L. Supratentorial ependymoma in children. Pediatr Neurosurg 2001;34:77–87. 3. Roncaroli F, Consales A, Fioravanti A. Supratentorial cortical ependymoma: report of three cases. Neurosurgery 2005;57:E192. 4. Miyazawa T, Hirose T, Nakanishi K. Supratentorial ectopic cortical ependymoma occurring with intratumoral hemorrhage. Brain Tumor Pathol 2007;24:35–40. 5. Lehman NL. Patterns of brain infiltration and secondary structure formation in supratentorial ependymal tumors. J Neuropathol Exp Neurol 2008;67:900–10. 6. Yadav YR, Neha, Chandrakar SK. Pure cortical supratentorial extraventricular ependymoma. Neurol India 2009;57:213–5. 7. Van Gompel JJ, Koeller KK, Meyer FB, et al. Cortical ependymoma: an unusual epileptogenic lesion. J Neurosurg 2011;114:1187–94. 8. Lehman NL. Central nervous system tumors with ependymal features: a broadened spectrum of primarily ependymal differentiation? J Neuropathol Exp Neurol 2008;67:177–88. 9. Wang M, Tihan T, Rojiani AM. Monomorphous angiocentric glioma: a distinctive epileptogenic neoplasm with features of infiltrating astrocytoma and ependymoma. J Neuropathol Exp Neurol 2005;64:875–81. 10. Lum DJ, Halliday W, Watson M. Cortical ependymoma or monomorphous angiocentric glioma? Neuropathology 2008;28:81–6. 11. Taylor MD, Poppleton H, Fuller C. Radial glia cells are candidate stem cells of ependymoma. Cancer Cell 2005;8:323–35. 12. Palma L, Celli P, Mariottini A. The importance of surgery in supratentorial ependymomas. Long-term survival in a series of 23 cases. Childs Nerv Syst 2000;16:170–5.