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Original Article

Spectrum of pediatric brain tumors in


India: A multi-institutional study
Ayushi Jain, Mehar C. Sharma, Vaishali Suri, Shashank S. Kale1, A. K. Mahapatra1, Medha Tatke2, Geeta Chacko3, Ashish
Pathak4, Vani Santosh5, Preeta Nair6, Nuzhat Husain7, Chitra Sarkar

Departments of Pathology and 1Neurosurgery, All India Institute of Medical Sciences (AIIMS), New Delhi, 2Department of
Pathology, GB Pant Hospital, New Delhi, 3Christian Medical College (CMC), Vellore, 5National Institute of Mental Health and
Neuro Sciences (NIMHANS), Bangalore, 6Tata Memorial Hospital (TMH), Mumbai, 7Chhatrapati Shahuji Maharaj Medical
University (CSMMU), Lucknow, 4Department of Neurosurgery, Post Graduate Institute of Medical Education and Research
(PGIMER), Chandigarh, India

Abstract
Background: Till date there is no published multi-institutional data regarding the
epidemiological profile of pediatric brain tumors in India. Aim: The present retrospective
study analyses the histological spectrum of pediatric age group brain tumors in seven
tertiary care hospitals in India. Material and Methods: Data regarding frequencies of various
primary brain tumors (diagnosed according to the World Health Organization (WHO)
classification), in 3936 pediatric patients (<18 yrs of age), was collected from seven
tertiary care hospitals in India.Results: The most common primary pediatric brain tumors
were astrocytic tumors (34.7%), followed by medulloblastoma and supratentorial primitive
Address for correspondence: neuro-ectodermal tumors (22.4%), craniopharyngiomas (10.2%) and ependymal tumors
Dr. Chitra Sarkar, (9.8%). The most common astrocytic tumor was pilocytic astrocytoma. In comparison to
Department of Pathology, All India adults, oligodendrogliomas and lymphomas were rare in children. Conclusions: Our study
Institute of Medical Sciences (AIIMS), is the first such report on the histological spectrum of brain tumors in children in India.
New Delhi - 110 029, India. Except for a slightly higher frequency of craniopharyngiomas, the histological profile of
E-mail: sarkar.chitra@gmail.com
pediatric brain tumors in India is similar to that reported in the Western literature.
Received : 24-09-2010
Review completed : 03-11-2010 Key words: Astrocytoma, craniopharyngioma, epidemiology, medulloblastoma,
Accepted : 27-12-2010 pediatric brain tumor, World Health Organization classification

Introduction tumors differ significantly from adult brain tumors in


reference to their sites of origin, clinical presentation,
Tumors of the nervous system are the second most tendency to disseminate early, histological features
common childhood tumor after leukemia,[1] constituting and their biological behavior. Whereas in adults the
approximately 35% of all childhood malignancies and predominant CNS tumor types are metastases, glial
remain the leading cause of cancer-related deaths in neoplasms and meningiomas, in children, besides
children.[2] Childhood central nervous system (CNS) gliomas, other major tumor types including primitive
embryonal neoplasms are also common. In recent
Access this article online times, an enhanced understanding of these biological
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differences between adult and childhood CNS neoplasms
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www.neurologyindia.com has led to investigations in distinct molecular and genetic
pathways and therapeutic approaches for each tumor
PMID:
***
type. However, for the necessary research required in the
field of pediatric brain tumors, a thorough knowledge
DOI: of the worldwide incidence and distribution of the
10.4103/0028-3886.79142
various neoplasms is essential. There are several reports

208 Neurology India | Mar-Apr 2011 | Vol 59 | Issue 2


Jain, et al.: Brain tumors in Indian children

regarding the epidemiology of CNS tumors in children Hospital, New Delhi; Christian Medical College (CMC),
in the Western literature.[1,3] However, due to scarcity Vellore; Post Graduate Institute of Medical Education
of reliable data collection and monetary resources, and Research (PGIMER), Chandigarh; National Institute
information on the demographic profiles of these tumors of Mental Health and Neurosciences (NIMHANS),
is scant in the developing world, where due to a large Bangalore; Tata Memorial Hospital (TMH), Mumbai;
population load the burden of such tumors is high. Chhatrapati Shahuji Maharaj Medical University
Specifically, till date, there is no published database (CSMMU), Lucknow, and All India Institute of Medical
on the profile of pediatric brain tumors in India, which Sciences (AIIMS), New Delhi. The time periods for which
is indeed a difficult and marathon task. The present data was available differed among the institutes. Primary
study therefore attempts to profile the hospital-based tumors of the brain were included, while metastatic
prevalence of pediatric CNS tumors (according to the tumors and vascular malformations were excluded. The
WHO classification[4]) at seven large centers in India cases were diagnosed and characterized where necessary
having excellent neurosurgical and neuropathology using immunohistochemistry and categorized according
facilities. The data has been compared with international to the most recent WHO classification prevalent at the
statistics from population- and hospital-based series. time of diagnosis.[4] With these criteria, a total of 3936
pediatric brain tumors were collected and analyzed to
assess breakup of different histological types and grades
Material and Methods
and compared with available international data in tumor
registries and hospital-based studies.
Data on CNS tumors in the pediatric age group (<18 years
of age) was collected from the neuropathology records of
seven tertiary hospitals in India, which included GB Pant Results

Table 1: Prevalence of pediatric tumors reported from various Pediatric CNS tumors accounted on an average
centers in India 14.8% of total intracranial tumors (ranging from 10-
Institution Time Total no. of Pediatric No. of 21%, Table 1). The percentage breakup of various
period pediatric tumors as % pediatric histological subtypes from various hospitals is provided
tumors of total ICT cases/year in Table 2, and percentage breakup of histological
(approx) subtypes of the most common gliomas (astrocytomas,
AIIMS 2002-2007 819 17 160 oligodendrogliomas and ependymomas) in Table 3.
NIMHANS 2003-2007 648 11 130
TMH 2006-2007 288 21 144
On comparing the histological subtypes of pediatric
CSMMU 2003- 2007 137 10 38 CNS tumors, it was seen that astrocytomas averaged
PGIMER, 2003- 2007 369 15 90 34.7% (range 22.3-46.7%), and were the commonest
Chandigarh tumors in almost all the hospitals. Embryonal tumors
CMC Vellore 1990-2007 1297 10 75
GB Pant 2003-2007 378 20.1 75
including medulloblastomas, supratentorial primitive
neuroectodermal tumors (PNETs), Atypical teratoid
AIIMS - All India Institute of Medical Sciences, New Delhi; NIMHANS -
National Institute of Mental Health and Neuro Sciences, Bangalore; rhabdoid tumors (ATRTs), etc, comprised the second
TMH - Tata Memorial Hospital, Mumbai; CSMMU - Chhatrapati Shahuji largest group after astrocytomas (average 22.4%).
Maharaj Medical University, Lucknow; PGIMER - Post Graduate Institute
of Medical Education and Research, Chandigarh; CMC - Christian Medical Craniopharyngiomas (10.2%) were the third most
College, Vellore; GB Pant - Govind Ballabh Pant Hospital, New Delhi common tumors followed by ependymal tumors (9.8%),

Table 2: Percentage breakup of various histological subtypes of pediatric CNS tumors


Tumor AIIMS NIMHANS GB Pant TMH CSMMU CMC Vellore PGIMER Average
Astrocytoma 33.7 44.1 22.3 28.6 30.6 46.7 37 34.7
MB and PNETs 16.8 19.7 32 29 27.7 10.3 21.6 22.4
Craniopharyngioma 12.7 7.7 13.5 4.5 13.1 8.5 11.5 10.2
Ependymal 8.5 8.5 12.2 19.1 9.4 4.8 6.3 9.8
Nerve sheath 7 4.3 1.3 2.4 2.2 4.6 NA 3.6
Meningeal 5.6 4.3 0.3 3.4 2.2 3.5 NA 3.2
Neuronal and mixed neuronal glial 4.1 2.8 5.2 2.1 0 NA NA 2.4
Germ cell tumors 2.2 2.2 3.3 1.7 2.2 NA NA 2
Choroid plexus tumors 1.5 2.6 1.6 1.7 1.5 NA 3.5 1.8
Pineal tumors 0.7 1.4 1.3 1 3 NA NA 1.3
Oligodendroglioma 0.7 0.9 2.9 1.4 1.5 0 0 1.1
Lymphoma 1 0.5 0.3 0 0 1.1 NA 0.5
MB and PNET - Medulloblastomas and supratentorial PNETs, includes other rare embryonal tumors; AIIMS - All India Institute of Medical Sciences, New Delhi;
NIMHANS - National Institute of Mental Health and Neuro Sciences, Bangalore; TMH - Tata Memorial Hospital, Mumbai; CSMMU - Chhatrapati Shahuji Maharaj
Medical University, Lucknow; PGIMER - Post Graduate Institute of Medical Education and Research, Chandigarh; CMC - Christian Medical College, Vellore; GB
Pant - Govind Ballabh Pant Hospital, New Delhi; NA - Data not available

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Jain, et al.: Brain tumors in Indian children

neuronal and mixed-neuronal glial tumors (4%), nerve Discussion


sheath tumors (3.6%) and meningeal tumors (3.2%).
In developing countries like India, due to lack of complete
Among the astrocytomas [Table 3], WHO Grade I tumors registration of newly diagnosed cases with local cancer
(pilocytic astrocytomas and subependymal giant cell registries, the exact tumor burden of such diseases
astroytoma (SEGA) were the commonest (average 23%). goes unnoticed and is underestimated. Hospital-based
Grade IV astrocytomas (glioblastoma multiforme (GBM) prevalence data therefore forms the basis for estimating
comprised on an average 4.46%. In ependymal tumors, the disease load. This data is essential for ascertaining the
anaplastic ependymoma (Grade III) was relatively more required healthcare infrastructure in the management of
common and comprised one-thirds to half of ependymal these diseases, and for assessing geographical differences
tumors. Oligodendrogliomas were very rare in the in their molecular and genetic profiles. With increased
pediatric age group (1.1%). CNS lymphomas also formed availability of diagnostic facilities and better healthcare,
a rare diagnosis in children. the incidence of CNS tumors seems to be on the rise in
developing countries. Due to the high incidence and
Table 4 compares the frequency of various CNS tumors mortality of childhood CNS tumors, they form the most
reported in different countries, including developed demanding group of tumors for neuro-oncologists. In
countries such as Canada, Sweden, Japan and Germany contrast to the Western literature, there is to date no
as well as developing ones like Brazil, China and report exclusively profiling pediatric CNS tumors in
Morocco.[1,5-11] The frequency of tumors in India fell India. We assessed the hospital-based prevalence of
between the ranges formed by the international data. such tumors by collecting data (categorized according

Table 3: Percentage breakup of various subtypes of pediatric CNS gliomas- astrocytomas, oligodendrogliomas and
ependymomas
Tumor AIIMS NIMHANS GB Pant TMH CSMMU CMC Vellore PGIMER Average
Astrocytoma Grade I (SEGA + Pilocytic) 23 29 19.4 17.4 14.6 34.9 NA 23.1
Astrocytoma Grade II (PXA + diffuse) 2.7 1.1 1.1 7.7 12.4 5.3 NA 5.1
Astrocytoma Grade III 2.4 4.8 0 1.4 0 2.9 NA 2.1
Astrocytoma Grade IV 5.6 9.2 1.8 2.1 3.6 3.6 NA 4.4
Total astrocytic tumors 33.7 44.1 22.3 28.6 30.6 46.7 37 34.7
Oligodendroglioma Grade II 0.3 0.2 1.3 1.4 1.5 0 0 0.7
Anaplastic Oligodendroglioma Grade III 0.2 0.5 1.6 0 0 0 0 0.4
Oligoastrocytoma 0.2 0.2 0 0 0 0 0 <0.1
Grade II
Anaplastic Oligoastrocytoma Grade III 0 0 0 0 0 0 0 0
Total oligodendroglial tumors 0.7 0.9 2.9 1.4 1.5 0 0 1.1
Myxopapillary ependymoma Grade I 0.2 0.5 0 0 0 NA NA 0.2
Ependymoma Grade II 3.7 3.7 11.4 14.6 8.7 NA NA 8.5
Anaplastic ependymoma Grade III 4.6 4.3 0.8 4.5 0.7 NA NA 3
Total ependymal tumors 8.5 8.5 12.2 19.1 9.4 4.8 6.3 9.8
SEGA - Subependymal giant cell astrocytoma; PXA - Pleomorphic xanthoastrocytoma; AIIMS - All India Institute of Medical Sciences, New Delhi; NIMHANS -
National Institute of Mental Health and Neuro Sciences, Bangalore; TMH - Tata Memorial Hospital, Mumbai; CSMMU - Chhatrapati Shahuji Maharaj Medical
University, Lucknow; PGIMER - Post Graduate Institute of Medical Education and Research, Chandigarh; CMC - Christian Medical College, Vellore; GB Pant -
Govind Ballabh Pant Hospital, New Delhi; CNS - Central nervous system; NA - Data not available

Table 4: Frequency of various types of pediatric CNS tumors reported in different countries (in percentage)
Tumor Brazil[1] Korea[5] Germany[6] Canada[7] Beijing[8] Sweden[9] Morocco[10] Japan[11] India
(present
study)
Astrocytomas 32.5 27.8 41.7 39.4 30.5 51 37.1 35.7 34.7
Oligodendrogliomas 0.9 2.6 1.1 1.7 6.2 0 1.7 0 1.1
Ependymomas 7.4 8.1 10.4 7 5.6 8 12 4.8 9.8
Choroid plexus tumors 3 2.2 NA 2.3 1.8 1.9 NA 0 1.8
Neuronal and mixed 7.6 6.2 3.2 <2 3.1 0 1.3 0 2.4
neuronal glial
MB and PNETs 13.9 19.8 25.7 15.4 14.6 17 28.9 10 22.4
Meningeal 3 2.6 1.2 <2 3.1 1.6 2.2 1.9 3.2
Nerve sheath NA 0.4 NA 3.1 2.8 1.1 NA 0 3.6
Germ cell 3.6 8.1 NA 3.1 7.9 1.5 0.9 14.3 2
Craniopharyngioma 11 9.2 4.4 6.8 18.4 4.6 6.6 10.5 10.2
Pineal tumors NA NA 1.3 0.5 0.6 2.7 0.7 0 1.3
MB and PNET - Medulloblastomas and supratentorial PNET, includes other rare embryonal tumors; CNS - Central nervous system; NA - Data not available

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Jain, et al.: Brain tumors in Indian children

to the most recent WHO classification[4]) from seven pediatric brain tumor research in India, particularly
centers with first-rate neurosurgical facilities in different when analyzing differences in their molecular and
geographical locations in India, in an attempt to represent genetic pathways, which could aid in the development
the profile of the entire country. of targeted, individualized therapies and planning
treatment protocols and strategies.
In our study, the most common brain tumors in the
pediatric age group in descending order are astrocytoma, To conclude, there is a rising global trend in the incidence
medulloblastoma, craniopharyngioma followed closely of pediatric CNS tumors. Based on large hospital series
by ependymoma. However, in a large meta-analysis by of pediatric patients, the present survey revealed the
Rickert and Paulius,[3] it was seen that internationally, histopathological diversity of childhood neurological
ependymomas are the third most common tumors neoplasms, and provides the first profile of the spectrum
followed by craniopharyngioma occupying the fourth of CNS tumors in children in India.
place. This trend can be seen in Table 4 in the data from
Canada,[7] Germany,[6] Sweden,[9] and Morocco[10] while References
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patient population. Also, the tumor frequencies differed
between different hospitals, as all are tertiary level How to cite this article: Jain A, Sharma MC, Suri V, Kale SS,
referral hospitals. The incidence of various CNS tumors Mahapatra AK, Tatke M, Chacko G, Pathak A, Santosh V, Nair P,
in the current study falls well within the range seen Husain N, Sarkar C. Spectrum of pediatric brain tumors in India: A
multi-institutional study. Neurol India 2011;59:208-11.
in the international studies for every tumor category.
This can have important connotations in the field of Source of Support: Nil, Conflict of Interest: None declared.

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