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ORIGINAL ARTICLE
Year : 2014  |  Volume : 9  |  Issue : 3  |  Page : 216-220
 

Outcome of supratentorial intraaxial extra ventricular primary pediatric brain tumors: A prospective study


1 Department of Neurosurgery, Krishna Institute of Medical Sciences, Secunderabad, USA
2 Department of Neurosurgery, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India
3 Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India

Date of Web Publication23-Dec-2014

Correspondence Address:
Mohana Rao Patibandla
Department of Neurosurgery, University of Colorado Denver, 13123, E 16th Ave, Aurora, CO 80045
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1817-1745.147571

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   Abstract 

Introduction: Tumors of the central nervous system (CNS) are the second most frequent malignancy of childhood and the most common solid tumor in this age group. CNS tumors represent approximately 17% of all malignancies in the pediatric age range, including adolescents. Glial neoplasms in children account for up to 60% of supratentorial intraaxial tumors. Their histological distribution and prognostic features differ from that of adults. Aims and Objectives: To study clinical and pathological characteristics, and to analyze the outcome using the Engel's classification for seizures, Karnofsky's score during the available follow-up period of minimum 1 year following the surgical and adjuvant therapy of supratentorial intraaxial extraventricular primary pediatric (SIEPP) brain tumors in children equal or less than 18 years. Materials and Methods: The study design is a prospective study done in NIMS from October 2008 to January 2012. All the patients less than 18 years of age operated for SIEPP brain tumors proven histopathologically were included in the study. All the patients with recurrent or residual primary tumors or secondaries were excluded from the study. Post operative CT or magnetic resonance imaging (MRI) is done following surgery. Results and Analysis: There were 2, 8 and 20 patients in the age range of 0-2 years, >2-10 years and 10-18 years, respectively. There were 21 male patients and 9 female patients. Out of 30 patients, 16 had lesion in the temporal lobe, 6 in frontal lobe, 4 in thalamus, 3 in parietal lobe and 1 in occipital lobe. Out of 30 patients, 11 patients had malignant lesions and nineteen patients had benign lesions. Gross total excision could be achieved in 19 patients and subtotal in 11 patients. Seven patients had mortality and four of the remaining 23 patients had increased deficits postoperatively. Remaining 19 patients either improved or remained same. Conclusions: SIEPP brain tumors have male preponderance, occur in 95% of patients in the age range of 7-18 years and have temporal lobe as the most common site of origin. The seizure presentation has good outcome compared to raised intracranial pressure features. The seizure control is quite good irrespective of subtotal or gross total excision in temporal lobe low grade tumors. All PNETs have survival of less than 1 year even with adjuvant chemo and radiotherapy.


Keywords: Brain tumors, extraventricular, intraaxial, outcome, pediatric, supratentorial


How to cite this article:
Patibandla MR, Bhattacharjee S, Uppin MS, Purohit AK. Outcome of supratentorial intraaxial extra ventricular primary pediatric brain tumors: A prospective study. J Pediatr Neurosci 2014;9:216-20

How to cite this URL:
Patibandla MR, Bhattacharjee S, Uppin MS, Purohit AK. Outcome of supratentorial intraaxial extra ventricular primary pediatric brain tumors: A prospective study. J Pediatr Neurosci [serial online] 2014 [cited 2019 Jul 19];9:216-20. Available from: http://www.pediatricneurosciences.com/text.asp?2014/9/3/216/147571



   Introduction Top


Tumors of the central nervous system (CNS) are, as a group, the second most frequent malignancy of childhood and the most common solid tumor in this age group. CNS tumors represent approximately 17% of all malignancies in the pediatric age range, including adolescents. [1]

Glial neoplasms in children account for up to 60% of supratentorial hemispheric tumors. They encompass a diverse range of pathology, including pilocytic and fibrillary astrocytomas, ependymomas, oligodendrogliomas, and gangliogliomas, as well as less common lesions unique to the pediatric age group: pleomorphic xanthoastrocytoma (PXA), dysembryoplastic neuroepithelial tumor (DNET), and desmoplastic infantile ganglioglioma (DIG). Their histologic distribution and prognostic features differ from that of adults, because the majority of these tumors in children are low grade (up to 80% of supratentorial tumors), and a strong association exists between extent of surgical resection and patient outcome. [2]

Many supratentorial gliomas are also found deep, near midline structures as opposed to a more common lobar location in adults. Radiologically, pediatric low-grade gliomas also differ in that they often enhance with contrast, and this enhancement does not suggest that these neoplasms are malignant. [2]

In the supratentorial compartment, low-grade gliomas in children can arise not only from the hemisphere but also from optic nerve, chiasm, and visual pathways, as well as deep seated regions like hypothalamus, thalamus, and basal ganglia.


   Aims and Objectives Top


To study clinical and pathological characteristics of supratentorial intraaxial extraventricular primary pediatric (SIEPP) brain tumors in children equal or less than 18 years. To analyze factors that affect outcome following management of these tumors. To determine the outcome using the Engel's classification for seizures, Karnofsky's score during the available follow-up period of minimum 1 year following surgery and adjuvant treatment of these tumors.


   Materials and Methods Top


The study design is a prospective study done in Nizam's Institute of Medical Sciences from October 2008 to January 2012. All the patients with age less than 18 years operated for SIEPP brain tumors proven histopathologically were included in the study. All the patients with recurrent or residual primary tumors or secondaries were excluded from the study.

The patients were evaluated with complete neurological examination and radiologically with CT and MRI studies. In the surgery, if greater than 90% of the lesion was excised, it was considered as gross total excision and others were considered subtotal excision. Chemotherapy and radiotherapy were given specific to the lesion. In the follow up patients were assessed at the end of the 1 st month, 3 rd month, 6 th month, and 1 year and then each year following surgery. Postoperative CT or MRI was done following surgery.


   Results and Analysis Top


There were 2, 8 and 20 patients in the age range of 0-2 years, >2-10 years and 10-18 years, respectively. There were 21 male patients and 9 female patients. Five patients presented with hemiparesis. Twelve of them presented with seizures. Four of them had only headache and 14 patients had raised ICP. Out of 30 patients, 16 had lesion in the temporal lobe, 6 had in the frontal lobe, 4 in thalamus, 3 in the parietal lobe and 1 in the occipital lobe. Out of 30 patients, 11 patients had malignant lesions, among them glioblastoma multiforme (GBM), primitive neuroectodermal tumor (PNET), anaplastic astrocytoma (AA) and anaplastic ependymoma (AE) were present in 4, 4, 2 and 1, respectively. Nineteen patients had benign lesions, among them 5, 1, 1, 1, 2, 2, 4, and 3 were pilocytic astrocytoma (PA), pilomyxoid astrocytoma (PMA), fibrillary astrocytoma (FA) oligodendroglioma (OLIGO), pleomorphic xanthoastrocytoma (PXA), ependymoma (EPEN), dysembryoplastic neuroepithelial tumor (DNET), and ganglioglioma, respectively. Gross total excision could be achieved in 19 patients and subtotal in 11 patients [Figure 1], [Table 1]. Seven patients had mortality and among them three had PNET, three had GBM and one had pleomorphic xanthoastrocytoma. Four of the remaining 23 patients had increased deficits postoperatively. Remaining 19 patients either improved or remained the same.
Figure 1: Extent of excision vs pathology

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Table 1: Extent of excision vs pathology

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   Discussion Top


Total number of patients with gliomas in this study was 22. Of the 22 patients, 15 were astrocytomas, 3 ependymomas, 1 oligodendroglioma, 3 gangliogliomas. Seventeen of the 22 glioma patients were males and females were 5 only. Except one all (n = 22) patients (95%) were in the age range of 7-18 years. This could be multifactorial like the lesion incidence increases with age. Also, the referral pattern to our institute might have affected the age range of the patients because usually the infant patient population is referred to the pediatric hospitals close to our area e.g. Govt. Pediatrics Hospital etc., Seventeen of the 22 glioma patients were males and only 5 were females. Contrary to the existing western literature, [1] social factors in the Indian population may have contributed to this finding in our study. Contrary to the literature where the frontal lobe is the most common site in our study, the temporal lobe is the most common site of the gliomas origin as seen in 50% of the 22 glioma patients. [1]

Of the five pilocytic astrocytomas, three of them were present in the temporal lobe; the other two were in the thalamic region. The only patient with PMA originated in the thalamus. The two patients with PXA were located in the temporoparietal region. All the three patients of ganglioglioma originated in the temporal lobe. Temporal lobe is the most common site of the glioma origin as seen in the 11 of the 22 glioma patients (50%) in our study.

In the published medical literature, astrocytomas account for approximately 50% of hemispheric tumors in children, occurring at all ages, with a peak incidence between ages 8 and 12. There is no gender predilection. [1]

In this study, the number of patients with astrocytomas was 15 constituting 50% of the total study group. There were 12 male and 3 female patients having astrocytoma. The incidence of astrocytoma increases with age as noted in this study. 1, 3 and 11 patients were with age ranging from 0-2, 2-10 and 11-18 years, respectively. Fourteen of the 15 patients were within 8-18 years age range. There is no preference to side of the cerebrum. As per the location of the lesion, the temporal lobe is the most common location of the lesion constituting 7 of the 15 lesions. The other locations are frontal lobe (n = 4) and thalamus (n = 4).

As per Qaddoumi et al., (n = 3419), high grade gliomas represent 17% of pediatric gliomas and their frequency depends upon site of the lesion and age of the patient. Most of the high grade tumors occur in the cerebrum and brainstem and in the age group more than 5 years. [1] In this study, out of the 22 gliomas, high grade lesions were 7 (32%), and low grade lesions were 15 (68%). In our study, six of the seven high grade glioma patients were in the age range of 8-17 years. This incidence correlates with other studies. [1] The percentage of high grade gliomas and age representation in the present study correlates with other studies.

The extent of surgery and grade of the lesion have the major impact in survival of pediatric high grade gliomas, which is proved in the largest series of Qaddoumi et al. and the Children's Cancer Group (CCG) - 43, and CCG - 945 patients. [1] The better survival of the high grade glioma patients with gross total resection is also confirmed in our study as shown in the literature. In the present study, of the seven high grade lesions, gross total excision was done in two patients and subtotal excision was done in rest of the five patients. All the patients were administered chemotherapy and six of the patients with age more than or equal to 8 years received radiotherapy. Three of the five patients in whom subtotal excision was done died within 1 year follow up. Mortality in this subset of patients (3/7) is 43%. The other two patients with subtotal excision treated with radiotherapy (RT) and chemotherapy (CT) had recurrence in 1 year. No recurrence of the lesion was noticed in the two patients who underwent gross total excision and postoperative RT at the end of 1 year follow up.

Of the 15 patients with low grade gliomas (62%), gross total excision was achieved in six patients and subtotal excision was done in the remaining nine patients. One patient died at 1 year of the follow-up period (PXA). Mortality in this subset of patients (1/15) is 7%. One patient got recurrence 10 months after the subtotal excision of the PMA in the left thalamus. Remaining 13 patients with low-grade gliomas, whether total or subtotal excision was performed, are doing well at the end of the follow-up period of maximum 3 years. Further follow up of these patients will determine the status of the lesion and outcome of the surgery.

Outcome of low grade gliomas is better than that of high-grade gliomas in this study as already proved in many other studies. The mortality was 7% compared to 43% to latter. The recurrence rate is also less in the low-grade glioma patients. As per Qaddoumi et al.,(n = 3419), low grade gliomas represent 55.5% of pediatric gliomas and grade I lesions showed better survival than grade II lesions in all age groups except among infants. [1] Reports of the prognostic value of grade I versus grade II LGG are inconsistent. Some studies have found no difference in outcome between grades I and II, [2],[3],[4] possibly because of the small number of subjects. A larger study (n = 278) showed that tumor grade (after pathology review) significantly influenced survival estimates, which were 92% for PA, 86% for glioma not otherwise specified, and 48% for diffuse astrocytoma (P < 0.0001). [5],[6],[7],[8]

However, the optimal surgical treatment for children suffering from epilepsy associated with temporal-lobe tumors remains controversial. [9],[10],[11],[12],[13],[14] Some authors consider complete tumor resection alone as an adequate treatment, [11],[12],[13],[14] whereas others recommend the additional removal of epileptogenic surrounding brain tissue to provide a good seizure outcome. [15],[16],[17] While some investigators [17] reported significant seizure control following radical tumor removal. From a surgical point of view, another controversial issue is the resection of mesial temporal structures in addition to the tumor removal. Whether the additional vital brain tissue resection improves the outcome significantly or not continues to be a matter of debate. In our study, all the temporal low-grade gliomas with seizures as presenting symptom were completely seizure free at an average follow-up of 2.4 years. Seizure-free outcomes were good irrespective of subtotal or gross total excision in temporal lobe tumors, correlating with other studies. [18]

Regarding the site of origin of DNET, initial reports suggested the location of the tumor to be in temporal lobe in 60% of the cases. [19] However, others reported 80-90% temporal lobe location. [20] All of the four patients of the present study had the location in the temporal lobe. The male and female ratio was 1:1. Age range of this subset of patients was 9 to 17 years. In the literature, surgery is the only required therapy and multiple surgeries are a better alternative than CT or RT. All the patients in the present study were operated by gross total excision. All of them were doing well at the end of one year with good control of seizures (Engel 1).

In the present study, the gender ratio was 1:1. Age range was 1-14 years. Two patients were within 0-2 years range. Other two were 9 years and 14 years of age. Subtotal excision could be achieved in all the patients. Postoperatively, the two patients within 0-2 years range were given CT. The other two patients were given both CT and RT. All four patients died within one year follow up. These study findings are correlating with the existing studies in the literature. [21]

Future suggestions to improve study

More number of patients to be involved in each subset of patients including those below 7 years of age. Volumetric analysis depicting objective evidence of extent of tumor excision is more scientific than the personal opinion of operating surgeon and may be included in future studies. Low grade gliomas require long-term follow up for better insight into the natural and intervened progression/regression. Pediatric CNS tumor registry has to be formulated for better analysis of natural history.


   Conclusions Top


Supratentorial intraaxial extraventricular primary pediatric (SIEPP) brain tumors have male preponderance, occur in 95% of patients in the age range of 7-18 years and have temporal lobe as the most common site of origin. The seizure presentation has good outcome compared to raised intracranial pressure features. The seizure control is quite good irrespective of subtotal or gross total excision in temporal lobe low grade tumors. All PNETs with subtotal excision have survival of less than 1 year even with adjuvant chemo and radiotherapy.

 
   References Top

1.
Qaddoumi I, Sultan I, Gajjar A. Outcome and prognostic features in pediatric gliomas: A review of 6212 cases from the surveillance, epidemiology, and end results database. Cancer 2009;115:5761-70.  Back to cited text no. 1
    
2.
Rivera-Luna R, Zapata-Tarrés M, Medina-Sansón A, López-Aguilar E, Niembro-Zúñiga A, Amador Zarco J, et al. Long-term survival in children under 3 years of age with low-grade astrocytoma. Childs Nerv Syst 2007;23:543-7.  Back to cited text no. 2
    
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Prados MD, Edwards MS, Rabbitt J, Lamborn K, Davis RL, Levin VA. Treatment of pediatric low-grade gliomas with a nitrosourea-based multiagent chemotherapy regimen. J Neurooncol 1997;32:235-41.  Back to cited text no. 3
    
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Packer RJ, Ater J, Allen J, Phillips P, Geyer R, Nicholson HS, et al. Carboplatin and vincristine chemotherapy for children with newly diagnosed progressive low-grade gliomas. J Neurosurg 1997;86:747-54.  Back to cited text no. 4
    
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Fisher PG, Tihan T, Goldthwaite PT, Wharam MD, Carson BS, Weingart JD, et al. Outcome analysis of childhood low-grade astrocytomas. Pediatr Blood Cancer 2008;51:245-50.  Back to cited text no. 5
    
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Sievert AJ, Fisher MJ. Pediatric low-grade gliomas. J Child Neurol 2009;24:1397-408.  Back to cited text no. 6
    
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Gajjar A, Sanford RA, Heideman R, Jenkins JJ, Walter A, Li Y, et al. Low-grade astrocytoma: A decade of experience at St. Jude Children′s Research Hospital. J Clin Oncol 1997;15:2792-9.  Back to cited text no. 7
    
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Fisher BJ, Leighton CC, Vujovic O, Macdonald DR, Stitt L. Results of a policy of surveillance alone after surgical management of pediatric low grade gliomas. Int J Radiat Oncol Biol Phys 2001;51:704-10.  Back to cited text no. 8
    
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Kim SK, Wang KC, Hwang YS, Kim KJ, Cho BK. Intractable epilepsy associated with brain tumors in children: Surgical modality and outcome. Childs Nerv Syst 2001;17:445-52.  Back to cited text no. 9
    
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Blümcke I, Wiestler OD. Gangliogliomas: An intriguing tumor entity associated with focal epilepsies. J Neuropathol Exp Neurol 2002;61:575-84.  Back to cited text no. 10
    
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Clusman H, Kral T, Gleissner U, Sassen R, Urbach H, Blümcke I, et al. Analysis of different types of resection for pediatric patients with temporal lobe epilepsy. Neurosurgery 2004;54:847-60.  Back to cited text no. 11
    
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Hammond RR, Duggal N, Woulfe JM, Girvin JP. Malignant transformation of a dysembryoplastic neuroepithelial tumor: Case report. J Neurosurg 2000;92:722-5.  Back to cited text no. 12
    
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Johnson JH Jr, Hariharan S, Berman J, Sutton LN, Rorke LB, Molloy P, et al. Clinical outcome of pediatric gangliogliomas: Ninety-nine cases over 20 years. Pediatr Neurosurg 1997;27:203-7.  Back to cited text no. 13
    
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Montes JL, Rosenblatt B, Farmer JP O′Gorman AM, Andermann F, Walters GV, et al. Lesionectomy of MRI detected lesions in children with epilepsy. Pediatr Neurosurg 1995;22:167-73.  Back to cited text no. 14
    
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Aronica E, Leenstra S, van Veelen CW, van Rijen PC, Hulsebos TJ, Tersmette AC, et al. 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.  Back to cited text no. 15
    
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Sutten LN, Packer RJ, Rorke LB, Bruce DA, Schut L. Cerebral gangliogliomas during childhood. Neurosurgery 1983;13:124-8.  Back to cited text no. 16
    
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Morris HH, Matkovic Z, Estes ML, Prayson RA, Comair YG, Turnbull J, et al. Ganglioglioma and intractable epilepsy: Clinical and neurophysiologic features and predictors of outcome after surgery. Epilepsia 1998;39:307-13.  Back to cited text no. 17
    
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Raymond AA, Halpin SF, Alsanjari N, Cook MJ, Kitchen ND, Fish DR, et al. Dysembryosplastic neuroepithelial tumor. Features in 16 patients. Brain 1994;117:461-75.  Back to cited text no. 19
    
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