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Original Article Outcome of Supratentorial Intraaxial Extra Ventricular Primary Pediatric Brain Tumors

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Original Article Outcome of Supratentorial Intraaxial Extra Ventricular Primary Pediatric Brain Tumors Original Article Outcome of supratentorial intraaxial extra ventricular primary pediatric brain tumors: A prospective study Mohana Rao Patibandla, Suchanda Bhattacharjee1, Megha S. Uppin2, Aniruddh Kumar Purohit1 Department of Neurosurgery, Krishna Institute of Medical Sciences Secunderabad, Departments of 1Neurosurgery and 2Pathology, Nizam’s Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India Address for correspondence: Dr. Mohana Rao Patibandla, Department of Neurosurgery, University of Colorado Denver, 13123, E 16th Ave, Aurora, CO 80045, USA. Email: [email protected] 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. Key words: Brain tumors, extraventricular, intraaxial, outcome, pediatric, supratentorial Introduction Access this article online Tumors of the central nervous system (CNS) are, as a group, Quick Response Code: the second most frequent malignancy of childhood and the Website: most common solid tumor in this age group. CNS tumors www.pediatricneurosciences.com represent approximately 17% of all malignancies in the pediatric age range, including adolescents.[1] DOI: 10.4103/1817-1745.147571 Glial neoplasms in children account for up to 60% of supratentorial hemispheric tumors. They encompass a 216 / Journal of Pediatric Neurosciences / Volume 9 / Sep-Dec / 2014 Patibandla, et al.: Supratentorial intra axial pediatric brain tumors diverse range of pathology, including pilocytic and fibrillary Results and Analysis astrocytomas, ependymomas, oligodendrogliomas, and gangliogliomas, as well as less common lesions unique to the There were 2, 8 and 20 patients in the age range of 0‑2 years, pediatric age group: pleomorphic xanthoastrocytoma (PXA), >2‑10 years and 10‑18 years, respectively. There were 21 male dysembryoplastic neuroepithelial tumor (DNET), and patients and 9 female patients. Five patients presented with desmoplastic infantile ganglioglioma (DIG). Their histologic hemiparesis. Twelve of them presented with seizures. Four of distribution and prognostic features differ from that of adults, them had only headache and 14 patients had raised ICP. Out because the majority of these tumors in children are low of 30 patients, 16 had lesion in the temporal lobe, 6 had in the grade (up to 80% of supratentorial tumors), and a strong frontal lobe, 4 in thalamus, 3 in the parietal lobe and 1 in the association exists between extent of surgical resection and occipital lobe. Out of 30 patients, 11 patients had malignant patient outcome.[2] lesions, among them glioblastoma multiforme (GBM), primitive neuroectodermal tumor (PNET), anaplastic Many supratentorial gliomas are also found deep, near midline astrocytoma (AA) and anaplastic ependymoma (AE) were structures as opposed to a more common lobar location present in 4, 4, 2 and 1, respectively. Nineteen patients had in adults. Radiologically, pediatric low‑grade gliomas also benign lesions, among them 5, 1, 1, 1, 2, 2, 4, and 3 were pilocytic differ in that they often enhance with contrast, and this astrocytoma (PA), pilomyxoid astrocytoma (PMA), fibrillary enhancement does not suggest that these neoplasms are astrocytoma (FA) oligodendroglioma (OLIGO), pleomorphic malignant.[2] xanthoastrocytoma (PXA), ependymoma (EPEN), dysembryoplastic neuroepithelial tumor (DNET), and In the supratentorial compartment, low‑grade gliomas in ganglioglioma, respectively. Gross total excision could be children can arise not only from the hemisphere but also achieved in 19 patients and subtotal in 11 patients [Figure 1, from optic nerve, chiasm, and visual pathways, as well as Table 1]. Seven patients had mortality and among them deep seated regions like hypothalamus, thalamus, and basal three had PNET, three had GBM and one had pleomorphic ganglia. xanthoastrocytoma. Four of the remaining 23 patients had increased deficits postoperatively. Remaining 19 patients either improved or remained the same. Aims and Objectives To study clinical and pathological characteristics of Discussion supratentorial intraaxial extraventricular primary pediatric (SIEPP) brain tumors in children equal or less than Total number of patients with gliomas in this study was 22. 18 years. To analyze factors that affect outcome following Of the 22 patients, 15 were astrocytomas, 3 ependymomas, management of these tumors. To determine the outcome using 1 oligodendroglioma, 3 gangliogliomas. Seventeen of the the Engel’s classification for seizures, Karnofsky’s score during 22 glioma patients were males and females were 5 only. the available follow‑up period of minimum 1 year following Except one all (n = 22) patients (95%) were in the age range surgery and adjuvant treatment of these tumors. 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 Materials and Methods because usually the infant patient population is referred to the pediatric hospitals close to our area e.g. Govt. Pediatrics The study design is a prospective study done in Nizam’s Hospital etc., Seventeen of the 22 glioma patients were males Institute of Medical Sciences from October 2008 to January and only 5 were females. Contrary to the existing western 2012. All the patients with age less than 18 years operated literature,[1] social factors in the Indian population may have 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 WRWDO excised, it was considered as gross total excision and VXEWRWDO O $ F 0 $ )$ others were considered subtotal excision. Chemotherapy $$ 3; *% 31(7 30 and radiotherapy were given specific to the lesion. In '1(7 the follow up patients were assessed at the end of the JDJQOLR st rd th $(RFFLSLWD 1 month, 3 month, 6 month, and 1 year and then each 3$WKDODPL 3$WHPSRUDO year following surgery. Postoperative CT or MRI was done HSHQG\PRPD« following surgery. Figure 1: Extent of excision vs pathology 2014 / Sep-Dec / Volume 9 / Journal of Pediatric Neurosciences / 217 Patibandla, et al.: Supratentorial intra axial pediatric brain tumors Table 1: Extent of excision vs pathology depends upon site of the lesion and age of the patient. Most of the high grade tumors occur in the cerebrum and Pathology Sub total Total Outcome brainstem and in the age group more than 5 years.[1] In DNET 0 4 Engel I KPS-100 this study, out of the 22 gliomas, high grade lesions were PNET 4 0 3 died and 1 lost for 7 (32%), and low grade lesions were 15 (68%). In our study, follow up six of the seven high grade glioma patients were in the age GBM 3 1 3 died, 1 no recurrence range of 8‑17 years. This incidence correlates with other KPS-100 studies.[1] The percentage of high grade gliomas and age PA thalamic 2 0 No recurrence KPS-80 representation in the present study correlates with other PA temporal 1 2 No recurrence studies. KPS-100 PXA 2 1 1 died, 2 no recurrence The extent of surgery and grade of the lesion have the
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