Case Report
 

By Dr. Issam Lalya , Dr. Sara Bellefqih , Dr. Jihane Khalil , Dr. Mohamed Allaoui , Prof. Basma Elkhannoussi , Prof. Khalid Hassouni , Prof. Tayeb Kebdani , Prof. Brahim Khalil Elgueddari , Prof. Noureddine Benjaafar
Corresponding Author Dr. Issam Lalya
National Institute of Oncology , Rabat , - Morocco
Submitting Author Dr. Issam Lalya
Other Authors Dr. Sara Bellefqih
Department of radiation oncology, National Institute of Oncology , Rabat , - Morocco

Dr. Jihane Khalil
Department of Radiation Oncology, National Institute of Oncology , Rabat , - Morocco

Dr. Mohamed Allaoui
Department of Pathology, National Institute of Oncology , Rabat , - Morocco

Prof. Basma Elkhannoussi
Department of Pathology, National Institute of Oncology , Rabat , - Morocco

Prof. Khalid Hassouni
Department of Radiation Oncology, National Institute of Oncology , Rabat , - Morocco

Prof. Tayeb Kebdani
Department of Radiation Oncology, National Institute of Oncology , Rabat , - Morocco

Prof. Brahim Khalil Elgueddari
Department of Radiation Oncology, Cheikh Zaid Hospital , Rabat , - Morocco

Prof. Noureddine Benjaafar
Department of Radiation Oncology, National Institute of Oncology , Rabat , - Morocco

ONCOLOGY

Gangliogliomas, Cerebellar, Surgery, Radiation Therapy, Chemotherapy .

Lalya I, Bellefqih S, Khalil J, Allaoui M, Elkhannoussi B, Hassouni K, et al. Radiation Therapy of Cerebellar Gangliogliomas: Case Report and Review of the Literature. WebmedCentral ONCOLOGY 2012;3(6):WMC003536
doi: 10.9754/journal.wmc.2012.003536

This is an open-access article distributed under the terms of the Creative Commons Attribution License(CC-BY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
No
Submitted on: 28 Jun 2012 11:47:41 PM GMT
Published on: 30 Jun 2012 04:00:54 PM GMT

Abstract


Gangliogliomas are rare neoplasms, histologically defined by the presence of both atypical ganglion cells and neoplastic glial cells. Usually located in the supratentorial space, they occur frequently during childhood and adolescence. Clinical and radiological features of GGs are not specific and are very similar to those of cystic cerebellar astrocytoma. Surgery is the treatment of choice of GGs and the role of radiotherapy and chemotherapy remains unclear. We report the case of an 8-year-old child who presented with inoperable cerebellar GG and treated exclusively by chemotherapy followed by radiotherapy.

Introduction


Gangliogliomas (GGs) are uncommon slow-growing tumors of the central nervous system histologically defined by the presence of both atypical ganglion cells and neoplastic glial cells (1). These tumors are usually located in the supratentorial space, primarily the temporal lobe, but they can arise in virtually any part of the brain (2-6). GGs occur frequently during childhood and adolescence (7,8). Clinical and radiological features of GGs are not specific and are very similar to those of cystic cerebellar astrocytomas (3,9). Surgery is the treatment of choice of GGs and the role of radiotherapy and chemotherapy remains unclear. (4,5,8) In the literature, only a few cases of GGs arising from the cerebellum have been reported. Because of their localisation complete resection of infratentorial GG is often not possible. In this work we report an inoperable cerebellar GG in an 8-year-old child treated exclusively by chemotherapy followed by radiotherapy.

Case Report


An 8-year-old girl presented at our institution with headache and right hemiparesis of 4-months duration.  On admission, neurological examination revealed right hemiparesis associated with gait disturbance. Computed tomography (CT) and Magnetic resonance imaging  (MR) scans showed a cystic and solid lesion in the right lateral cerebellar hemisphere extending to the bulbar region and the bulbo-medullary junction (Figures 1,2,3). A stereotactic biopsy was performed, which confirmed the diagnosis of benign gangliogloma (WHO grade I). Because of the localisation of the tumor, surgery was not possible, so the patient received a first-line chemotherapy with carboplatin and vincristine. After six cycles, a new MRI shows a stability of the lesion whereas the patient develops a left hemiparesis. Radiotherapy was given to the patient who received a dose of 45 Gy in 25 fractions delivered over 5 weeks. The entire regimen of radiation was well tolerated by the patient.

Discussion


GG is a relatively uncommon tumour of the central nervous system that was first described in 1926 as a distinct clinicopathologic entity containing both mature neuronal and glial neoplastic elements (10). They represent between 0.4% and 7.6% of all paediatric central nervous system (CNS) neoplasms and 1.3% of those in adults (4-6, 11). This tumor is usually seen in children and young adults during the first three decades of life, but they can occur at any age; extremes of a 3-day-old neonate and 80-year-old adults have been documented (7-8, 12-14). Ganglioglioma are more commonly located in the supratentorial area, mainly in the temporal lobe, but it can occur anywhere in the CNS. Less frequent locations within the cerebral hemisphere are (in order of decreasing frequency): the frontal, parietal, and occipital lobes (3-6, 8,15,16). Gangliogliomas occur rarely in the cerebellum with 34 cases reported previously (17-23). Clinical symptoms depend of the location of the tumors: for supratentorial GGs, seizure occurs with an incidence rate of 72-100% (3,4, 24,25). Posterior fossa GGs are more presented with focal neurological deficits, cranial nerve palsy, hydrocephalus, increased intracranial pressure, speech or gait disturbance, and myoclonus (1, 17, 18, 21, 26, 27). There are no specific radiological findings that could be discriminate GGs from other cerebellar lesions that are more common in pediatric patients such as cerebellar astrocytomas, ependymomas, desmoplastic medulloblastomas, and hemangioblastomas. These tumors may also demonstrate cyst formation as well as a solid portion, CT-scan shows an isodense or hypodense tumor, with calcification present in 6% to 30% (26). Mass effect and oedema are minimal (3,28,29). MR findings also are not characteristic. GGs appears as a well-defined lesion with variable mass effect. They usually have low signal on T1-weighted images but some are isointense. On T2-weighted images, solid lesions show increased signal. The variable signal in cystic components depends on whether the contents are proteinaceous, haemorrhagic, or contain cerebrospinal fluid (3,28,29). Correct diagnosis of cerebellar GGs requires adequate sampling of the tumor and elaborate histological examinations. This is determined by identification of atypical-appearing neuronal or ganglion cells in the tumor with the glial component of the tumor that may demonstrate variable degrees of hyper cellularity and nuclear pleomorphism (4,30,31).Synaptophysin, neurofilament protein glial and fibrillary acidic protein may also help to the diagnosis (32). GGs can be categorized further as either low-grade or high-grade tumors. Low-grade GGs are slow-growing tumors that generally have a favourable prognosis unless occurring in a non-resectable location. If the tumor has anaplastic features or necrosis of the glial components or an elevated MIB-1 labelling index ≥10%, it is considered a high-grade glioma and tends to behave in a more aggressive fashion (33). Gross total resection is the recommended treatment of choice for GGs and complete tumor resection is associated with good prognosis (4,5). Long-term survival is achieved if gross total resection is possible, ranging between 7 and 17 years (24,26,34,35). The benefit of adjuvant radiotherapy has not been proven. In fact, many physicians are hesitant to administer radiotherapy because of the potential morbidity in long-term survivors (34,35). Furthermore, it have been suggested that postoperative radiation may predispose GGs to malignant degeneration (36,37). As GGs are quite uncommon, no prospective studies have been performed or can be expected in the near future to assess the role of radiotherapy. In the review of Rades et al that includes 402 cases of GGs, local control (but not overall survival) was improved significantly with the addition of RT after subtotal resection in both the low grade and high-grade subgroups.  After gross total resection, RT conferred no improvement in outcome for patients with either low-grade or high-grade tumors (8). Because of their location complete resection of infratentorial GG is often not possible without producing severe deficits or even death. In our case, the resection was not possible so the patient was treated with chemotherapy, followed by radiotherapy after progression. Cerebellar GGs are uncommon tumors that occur frequently during childhood and adolescence. CT scan and MR imaging could suggest the diagnosis but it’s rarely made prior to surgery. With complete tumor resection, cerebellar GGs could be cured and no additional therapy is required. For unresecable GGs, or after subtotal resection, radiotherapy and/or chemotherapy should be considered. 

 

Conclusion


Cerebellar GGs are uncommon tumors that occur frequently during childhood and adolescence. CT scan and MR imaging could suggest the diagnosis but it’s rarely made prior to surgery. With complete tumor resection, cerebellar GGs could be cured and no additional therapy is required. For unresecable GGs, or after subtotal resection, radiotherapy and/or chemotherapy should be considered. 

 

Abbreviations


GGs: Gangliogiomas 

MRI: Magnetic resonance imagine

CT scan: computed tomography scanner 

References


1. Johannsson JH, Rekate HL, Roessmann U. Gangliogliomas : pathological and clinical correlation. J Neurosurg. 1981;54:58–63.
2. M. Castillo, P.C. Davis, Y. Takei et al. Intracranial ganglioglioma: MR, CT, and clinical findings in 18 patients .AJR Am J Roentgenol, 154 (1990), pp. 607–612
3. Zhang D, Henning TD, Zou LG, Hu LB, Wen L, Feng XY, Dai SH, Wang WX, Sun QR, Zhang ZG. Intracranial ganglioglioma: clinicopathological and MRI findings in 16 patients. Clin Radiol. 2008 Jan;63(1):80-91. Epub 2007 Nov 5.
4. Zentner J, Wolf H K, Ostertun B, et al. Gangliogliomas: clinical, radiological, and histopathological findings in 51 patients. J Neurol Neurosurg Psychiatry. 1994;57:1497–1502.
5. Koeller K K, Henry J M. Superficial gliomas: radiologic-pathologic correlation. Armed Forces Institute of Pathology. Radiographics. 2001;21:1533–1556. [PubMed]
6. Ildan F, Tuna M, Gocer I A, Erman T, Cetinalp E. Intracerebral ganglioglioma: clinical and radiological study of eleven surgically treated cases with follow-up. Neurosurg Rev. 2001;24:114–118.
7. Russell DS, Rubinstein LJ. Pathology of tumors of the central nervous system. Baltimore, Md: Williams & Wilkins, 1989.
8. Rades D, Zwick L, Leppert J, Bonsanto MM, Tronnier V, Dunst J, Schild SE. The role of postoperative radiotherapy for the treatment of gangliogliomas. Cancer. 2010 Jan 15;116(2):432-42.
9. Matsumoto K, Tamiya T, Ono Y, Furuta T, Asari S, Ohmoto T. Cerebral gangliogliomas: clinical characteristics, CT and MRI. Acta Neurochir (Wien). 1999;141(2):135-41.
10. Perkins OC: Ganglioglioma. Arch Pathol Lab Med 2:11–17, 1926
11. Selch M, Goy B, Lee S, El-Sadin S, Kincaid P, Park SH. Gangliogliomas : experience with 34 patients and review of the literature. Am J Clin Oncol. 1998;21:557–564.
12. Gelabert-González M, Amo JM, Arcos Algaba A, Serramito García R, Castro Bouzas D, Díaz Cabana L, Prieto González A, Aran Echabe E, Bandín Diéguez FJ, Villa Fernández J, García Allut A. Intracranial gangliogliomas. A review of a series of 20 patients. Neurologia. 2011 Sep;26(7):405-15.
 13. Haddad SF, Moore SA, Menezes AH, VanGilder JC : Ganglioglioma : 13 years of experience. Neurosurgery 31 : 171-178, 1992
14. Hanquinet S, Christophe C, Rummens E, et al. Ultrasound,computed tomography and magnetic resonance of a neonatal ganglioglioma of the brain. Pediatr Radiol 1986; 16:501–503.
15. M. Castillo, P.C. Davis, Y. Takei et al. Intracranial ganglioglioma: MR, CT, and clinical findings in 18 patients .AJR Am J Roentgenol, 154 (1990), pp. 607–612
16. Johnson J H, Jr, Hariharan S, Berman J, et al. Clinical outcome of pediatric gangliogliomas: ninety-nine cases over 20 years. Pediatr Neurosurg. 1997;27:203–207
17. Safavi-Abbasi S, Di Rocco F, Chantra K, Feigl GC, El-Shawarby A, Samii A, Samii M. Posterior Cranial Fossa Gangliogliomas. Skull Base. 2007 July; 17(4): 253–264.
18.  Park SH, Kim E, Son EI.  Cerebellar ganglioglioma. J Korean Neurosurg Soc. 2008 Mar;43(3):165-8.
19. Fedoul B,  Souirti Z. Cerebellar ganglioglioma.  The Pan African Medical Journal. 2012;12:12
20. Mekni A, Chelly I, Haouet S, Zitouna M, Kchir N. Malignant cerebellar ganglioglioma. J Clin Neurosci. 2008 May;15(5):582-5. Epub 2008 Mar 3.
21. Harding M, Brophy B, Geake T. Malignant cerebellar ganglioglioma. J Clin Neurosci. 2008 May;15(5):58
22. Hanai S, Okazaki K, Fujikawa Y, Nakagawa E, Sugai K, Sasaki M, Otsuki T. Hemifacial seizures due to ganglioglioma of cerebellum. Brain Dev. 2010 Jun;32(6):499-501.
23. Tokunaga H, Sunami K, Wagai N, Murai H, Nagai Y, Tanizawa T, Nakatani Y, Iwadate Y. Ganglioglioma originating in the cerebellum with a large cyst--a case report and review of the literature. Clin Neuropathol. 2008 Nov-Dec;27(6):369-72. Review.
24. Haddad SF, Moore SA, Menezes AH, VanGilder JC : Ganglioglioma : 13 years of experience. Neurosurgery 31 : 171-178, 1992
25. Im SH, Chung CK, Cho BK, Wang KC, Yu IK, Sone IC, et al. Intracranial gangioglioma : preoperative characteristics and oncologic outcome after surgery. J Neurooncol 59 : 173-183, 2002
26. Hakim R, Loeffler JS, Anthony DC, Black PM. Gangliogliomas in adults. Cancer. 1997;79:127–131.
27. Im SH, Chung CK, Cho BK, Wang KC, Yu IK, Song IC, et al. Intracranial ganglioglioma : preoperative characteristics and oncologic outcome after surgery. J Neurooncol. 2002;59:173–183. [PubMed]
28. Blatt GL, Ahuja A, Miller LL, Ostrow PT, Soloniuk DS. Cerebellomedullary ganglioglioma : CT and MR findings. AJNR Am J Neuroradiol. 1995;16:790–792.
29. Matsumoto K, Tamiya T, Ono Y, Furuta T, Asari S, Ohmoto T. Cerebral gangliogliomas: clinical characteristics, CT and MRI. Acta Neurochir (Wien). 1999;141(2):135-41.
30. Courville C B. Gangliogliomas: tumor of the central nervous system—review of the literature and report of 2 cases. Arch Neurol Psychiatry. 1930; 24 :39–91.
31. Courville CB, Anderson F M. Neuro-gliogenic tumor of the central nervous system: report of two additional cases of ganglioglioma of the brain. Bull Los Angeles Neurol Soc. 1941; 6 :154–176.
32. Miller DC, Lang FF, Epstein FJ. Central nervous system gangliogliomas. I. Pathology. J Neurosurg 1993; 73:859-866
33. Brat DJ, Parisi JE, Kleinschmidt-DeMasters BK, et al. Surgical neuropathology update. A review of changes intro-duced by the WHO Classification of Tumors of theCentral Nervous System, 4th edition.
Arch Pathol Lab Med . 2008;132:993-1007.
34. Silver JM, Rawlings CE 3rd, Rossitch E Jr, Zeidman SM, Friedman AH. Ganglioglioma: a clinical study with long-term follow-up. Surg Neurol. 1991 Apr;35(4):261-6.
35. Johnson JH Jr, Hariharan S, Berman J, Sutton LN, Rorke LB, Molloy P, Phillips PC. Clinical outcome of pediatric gangliogliomas: ninety-nine cases over 20 years. Pediatr Neurosurg. 1997 Oct;27(4):203-7.
36. Rumana CS, Valadka AB. Radiation therapy and malignant degeneration of benign supratentorial gangliogliomas. Neurosurgery. 1998; 42: 1038-1043.
37. Kalyan-Raman UP, Olivero WC. Ganglioglioma: a correlative clinicopathological and radiological study of ten surgically treated cases with follow-up. Neurosurgery. 1987 Mar;20(3):428-33.

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