- Department of Neurosurgery, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires, Argentina
- Department of Anatomical Pathology, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires, Argentina
- Department of Neurology, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires, Argentina
Correspondence Address:
Damián Bendersky
Department of Neurosurgery, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires, Argentina
DOI:10.4103/2152-7806.98511
Copyright: © 2012 Bendersky D. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.How to cite this article: Damián Bendersky, Nicolás Tedesco, Christiansen S, María del Carmen García, Ciraolo C. Epilepsy due to a temporal ganglioglioma and its subsequent malignant transformation into a primitive neuroectodermal tumor. Surg Neurol Int 14-Jul-2012;3:79
How to cite this URL: Damián Bendersky, Nicolás Tedesco, Christiansen S, María del Carmen García, Ciraolo C. Epilepsy due to a temporal ganglioglioma and its subsequent malignant transformation into a primitive neuroectodermal tumor. Surg Neurol Int 14-Jul-2012;3:79. Available from: http://sni.wpengine.com/surgicalint_articles/epilepsy-due-to-a-temporal-ganglioglioma-and-its-subsequent-malignant-transformation-into-a-primitive-neuroectodermal-tumor/
Abstract
Background:Gangliogliomas (GGs) are rare brain tumors, and their malignant changes are still less frequent. In these cases, almost always the malignant component is the glial one. However, there are few cases in which the neuronal component exhibits malignant transformation.
Case Description:We described a case of a 14-year-old male patient who started with seizures and 5 years later, its frequency was almost daily despite being treated with several antiepileptic drugs. Magnetic resonance imaging showed a nonenhancing lesion located at the right inferior temporal gyri. He underwent surgery, and the tumor was completely removed. Histological diagnosis was GG. He had no seizures anymore, but 3 years later, the tumor recurred as a giant heterogeneously enhancing space-occupying mass within the right temporal lobe. A second surgical resection was performed. Histological diagnosis was a primitive neuroectodermal tumor (PNET). One month and a half later, the tumor has recurred again. He and his family decided not to undergo another operation, so he was referred to the radiotherapy department.
Conclusion:As in this patient, GGs may recur as a primitive neuroectodermal tumor, likely because both types of neoplasm form part of the same heterogeneous group of brain tumors which contains neuronal components, but on the other side of the malignancy spectrum.
Keywords: Ganglioglioma, glioneural tumors, malignant transformation, supratentorial primitive neuroectodermal tumor, tumoral epilepsy
INTRODUCTION
Gangliogliomas (GGs) are rare brain tumors, accounting for approximately 0.4–2% of all intracranial neoplasms, but in fact it is more common in children.[
CASE REPORT
A 14-year-old male patient started with automotor seizures without loss of consciousness, which included oroalimentary and distal portions of the extremity automatisms, preceded by an abdominal aura. Initially he had persistent seizures, approximately three attacks per month. He was treated with antiepileptic drugs, which reduced their frequency to one attack per month. When he was 19 years old, seizures frequency was almost daily despite being treated with several antiepileptic drugs such as topiramate, carbamazepine, valproate, and clobazam. Also, automotor seizures evolved to secondary generalized tonic–clonic ones, three times during that year. He had two seizures during the video-electroencephalography monitoring, which showed an epileptiform activity compatible with acute temporal waves. Magnetic resonance imaging (MRI) showed a nonenhancing lesion located at the right inferior temporal gyri, which was hypointense and hyperintense in T1- and T2-weighted images, respectively. There were only few linear enhancing images near the lesion after contrast administration, which were thought as vessels. Furthermore, MRI revealed cortical retraction in the right temporal lobe, in which both subarachnoid spaces within the sulcus and the sylvian cistern were wider than left side ones [
Figure 1
(a) Coronal T2-weighted image showing a hyperintense lesion located at the right inferior temporal gyri. Right subarachnoid spaces within the sulcus and the sylvian cistern are wider than left side ones. (b and c) Sagittal and axial T1-weighted scans following gadolinium administration. (d) Postoperative axial T1-weighted sequence showing complete resection
Figure 2
(a) Some neuronal dysplastic elements (arrows) between glial neoplastic cells in a H and E, ×40. (b) Microcalcifications in a H and E, ×10. (c and d) Neuronal component was immunoreactive for neurofilament protein (c) and NeuN (d). (e) Glial component was positive for GFAP. (f) The Ki-67 (MIB-1) proliferation index was 1%
Thereafter, the patient continued with antiepileptic drugs, and he had no seizures anymore. Three years later, he started with headache, nausea, and vomiting. New MRI demonstrated a giant heterogeneously enhancing space-occupying mass within the right temporal lobe. It had cystic areas mainly in its upper parts. There was brainstem compression, and the midline shift was evident. The middle cerebral artery was displaced; it was just above and beyond the superomedial aspect of the lesion [Figure
Figure 3
(a) Axial T2-weighted image showing a giant mass within the right temporal lobe which presents cystic areas and causes brainstem compression. (b) Coronal T2-weighted sequence revealing the middle cerebral artery displacement. (c, d) Heterogeneous contrast enhancement after gadolinium administration on sagittal (c) and coronal (d) T1-weighted image. (e) Postoperative coronal T1-weighted scan. (f) Coronal T1-weighted image demonstrating a recurrent tumor 1 month and a half after the second surgical resection
Figure 4
(a) High grade tumor with abrupt borders formed by monomorphic cells; H and E, ×10. (b) Areas of necrosis within the tumor in a H and E, ×4. (c) Neuroblastic rosette formation, H and E, ×10. (d) Positive immunoreactivity for synaptophysin. (e) Focal positive immunoreactivity for GFAP at the right side of the histological section. (f) The Ki-67 (MIB-1) proliferation index was 50%, magnification 4×
Immediate postoperative recovery was uneventful. One month and a half after the last operation, he was admitted again because of headaches and left hemiparesis. MRI showed a giant right temporal tumor again [
DISCUSSION
GGs are mixed tumors, formed by both neuronal and glial elements, which are usually classified as WHO grade I lesions, although there are some cases of atypical (WHO grade II) and anaplastic (WHO grade III) GGs described in the literature. Thus, GGs form part of the heterogeneous group of brain tumors which contains neuronal components such as dysembryoplastic neuroepithelial tumors, medulloblastomas, supratentorial PNETs, and medulloepitheliomas. If there are neuronal cells alone, it is named gangliocytoma. The glial component is ordinarily astrocytic, but rarely, it may have oligodendroglial differentiation.[
GGs are a bit more frequent in males than females. It is more common in children; however, it may develop at any age. Therefore, mean age at diagnosis varies widely in the literature from 8.5 to 26.4 years.[
The recommended treatment for GGs is surgical excision. Epilepsy caused by GG may be cured after tumor resection. Postoperative seizure control is associated with gross total resection of the mass, shorter duration of epilepsy, younger age at surgery, and the absence of generalized seizures.[
Supratentorial PNET is a very rare and aggressive embryonal tumor, which corresponds to WHO grade IV and is composed of neuroepithelial cells which are able to differentiate along neuronal, astrocytic, muscular, or melanocytic lines. Usually, the proliferation index is high, as in our patient, who, moreover, had a very early recurrence even after complete resection.[
Although being almost always benign lesions, some GGs may develop malignant transformation.[
GGs are thought to originate from glioneural precursor cells, like neural stem cells.[
CONCLUSION
Despite being infrequent, it must be taken in mind that GGs may become aggressive tumors with malignant transformation in either or both neuronal and glial components, probably due to the existence of a common glioneural precursor cell. As in this patient, it may recur as a PNET, likely because both types of neoplasm form part of the same heterogeneous group of brain tumors which contains neuronal components, but on the other side of the malignancy spectrum.
References
1. Aronica E, Leenstra S, van Veelen CW, van Rijen PC, Hulsebos TJ, Tersmette AC. Glioneuronal tumors and medically intractable epilepsy: A clinical study with long-term follow-up of seizure outcome after surgery. Epilepsy Res. 2001. 43: 179-91
2. Bevilacqua G, Sarnelli R. Ganglioglioma of the spinal cord. A case with a long survival. Acta Neuropathol. 1979. 48: 239-42
3. Biernat W, Zakrzewski K, Polis L, Liberski PP. Glioneuronal-mesenchymal tumour with malignant transformation. Folia Neuropathol. 2007. 45: 140-3
4. Chintagumpala M, Hassall T, Palmer S, Ashley D, Wallace D, Kasow K. A pilot study of risk-adapted radiotherapy and chemotherapy in patients with supratentorial PNET. Neuro Oncol. 2009. 11: 33-40
5. Dastur DK. Cerebral ganglioglio-neuroblastoma: An unusual brain tumour of the neuron series. J Neurol Neurosurg Psychiatry. 1982. 45: 139-42
6. David KM, de Sanctis S, Lewis PD, Noury AM, Edwards JM. Neuroblastomatous recurrence of ganglioglioma. Case report. J Neurosurg. 2000. 93: 698-700
7. Fassunke J, Majores M, Tresch A, Niehusmann P, Grote A, Schoch S. Array analysis of epilepsy-associated gangliogliomas reveals expression patterns related to aberrant development of neuronal precursors. Brain. 2008. 131: 3034-50
8. Gelabert-González M, Amo JM, Arcos Algaba A, Serramito García R, Castro Bouzas D, Díaz Cabana L. Intracranial gangliogliomas. A review of a series of 20 patients. Neurologia. 2011. 26: 405-15
9. Gelebert-Gonzalez M, Serramito-Garcia R. The role of postoperative radiotherapy for the treatment of gangliogliomas. Cancer. 2010. 116: 3071-
10. Jay V, Squire J, Becker LE, Humphreys R. Malignant transformation in a ganglioglioma with anaplastic neuronal and astrocytic components. Report of a case with flow cytometric and cytogenetic analysis. Cancer. 1994. 73: 2862-8
11. Kang DH, Lee CH, Hwang SH, Park IS, Han JW, Jung JM. Anaplastic ganglioglioma in a middle-aged woman: A case report with a review of the literature. J Korean Med Sci. 2007. 22: S139-44
12. Kawataki T, Sato E, Sato T, Kinouchi H. Anaplastic ganglioglioma with malignant features in both neuronal and glial components--case report. Neurol Med Chir (Tokyo). 2010. 50: 228-31
13. Kim DG, Lee DY, Paek SH, Chi JG, Choe G, Jung HW. Supratentorial primitive neuroectodermal tumors in adults. J Neurooncol. 2002. 60: 43-52
14. Lafay-Cousin L, Strother D. Current treatment approaches for infants with malignant central nervous system tumors. Oncologist. 2009. 14: 433-44
15. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK.editorsWHO classification of tumours of the central nervous system. Lyon: IARC; 2007. p.
16. Luyken C, Blümcke I, Fimmers R, Urbach H, Wiestler OD, Schramm J. Supratentorial gangliogliomas: Histopathologic grading and tumor recurrence in 184 patients with a median follow-up of 8 years. Cancer. 2004. 101: 146-55
17. Majores M, von Lehe M, Fassunke J, Schramm J, Becker AJ, Simon M. Tumor recurrence and malignant progression of gangliogliomas. Cancer. 2008. 113: 3355-63
18. McLendon RE, Provenzale J. Glioneuronal tumors of the central nervous system. Brain Tumor Pathol. 2002. 19: 51-8
19. Mittelbronn M, Schittenhelm J, Lemke D, Ritz R, Nägele T, Weller M. Low grade ganglioglioma rapidly progressing to a WHO grade IV tumor showing malignant transformation in both astroglial and neuronal cell components. Neuropathology. 2007. 27: 463-7
20. Mittler MA, Walters BC, Fried AH, Sotomayor EA, Stopa EG. Malignant glial tumor arising from the site of a previous hamartoma/ganglioglioma: Coincidence or malignant transformation?. Pediatr Neurosurg. 1999. 30: 132-4
21. Pandita A, Balasubramaniam A, Perrin R, Shannon P, Guha A. Malignant and benign ganglioglioma: A pathological and molecular study. Neuro Oncol. 2007. 9: 124-34
22. Rades D, Zwick L, Leppert J, Bonsanto MM, Tronnier V, Dunst J. The role of postoperative radiotherapy for the treatment of gangliogliomas. Cancer. 2010. 116: 432-42
23. Reis F, Vieira GH, Rogerio F, Guerreiro MM, Queiroz Lde S, Zanardi Vde A. Supratentorial primitive neuroectodermal tumor (PNET): An uncommon location. Arq Neuropsiquiatr. 2011. 69: 997-
24. Shingu T, Kagawa T, Kimura Y, Takada D, Moritake K, Hoshii Y. Supratentorial primitive neuroectodermal tumor in an aged patient--case report--. Neurol Med Chir (Tokyo). 2005. 45: 530-5
25. Tarnaris A, O’Brien C, Redfern RM. Ganglioglioma with anaplastic recurrence of the neuronal element following radiotherapy. Clin Neurol Neurosurg. 2006. 108: 761-7
26. Zhu JJ, Leon SP, Folkerth RD, Guo SZ, Wu JK, Black PM. Evidence for clonal origin of neoplastic neuronal and glial cells in gangliogliomas. Am J Pathol. 1997. 151: 565-71