Indian J Pediatr DOI 10.1007/s12098-014-1387-6
CLINICAL BRIEF
Spectrum of Pediatric Brain Tumors: A Report of 341 Cases from a Tertiary Cancer Center in India Umesh Das & L. Appaji & B. S. Aruna Kumari & Nagesh T. Sirsath & M. Padma & S. Kavitha & T. Avinash & K. C. Lakshmaiah
Received: 27 November 2013 / Accepted: 20 February 2014 # Dr. K C Chaudhuri Foundation 2014
Abstract Brain tumors are the second most common cancers after hematological malignancies accounting for approximate 21 % of all childhood malignancies in children between ages of 0 and 14 y. The present study was undertaken to determine the spectrum of the brain tumors diagnosed in a tertiary cancer center in South India. A retrospective analysis of the data of pediatric brain tumors diagnosed between 2003 and 2009 was done and data was classified according to the age, gender and histology types. Out of 2,844 pediatric patients, 341 (11.99 %) were diagnosed as having brain tumors. Most of the patients were in the age group of >5–14 y. Male to female ratio was 1.58:1. The most common pediatric brain tumor was medulloblastoma followed by astrocytoma and ependymoma. Glioblastoma multiforme was the most common subtype of astrocytoma. Other common tumors were glioma, oligodendroglioma, periphereral neuroectodermal tumor and germ cell tumor. As compared to western data, incidence of brain tumors in children was found to be less in the present study.
Keywords Brain tumor . Medulloblastoma . Astrocytoma
U. Das : N. T. Sirsath : K. C. Lakshmaiah Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bangalore, Karnataka, India L. Appaji : B. S. A. Kumari : M. Padma : S. Kavitha : T. Avinash Department of Pediatric Oncology, Kidwai Memorial Institute of Oncology, Bangalore, Karnataka, India U. Das (*) Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Room No. 5, OPD Block, Bangalore 560029, Karnataka, India e-mail: [email protected]
Introduction Brain tumors are the most common solid tumors of childhood and the second most common malignancies after hematological malignancies accounting for approximate 21 % of all childhood malignancies in children between 0 and 14 y of age [1]. Apart from being a major cause of cancer related deaths, there is significant morbidity both from the disease and from the treatment with varying degrees of physical, cognitive, neurological, endocrinological, and other deficits in survivors resulting in significant handicap and diminished quality of life in brain tumor patients [2]. However, recently use of advanced surgical and neuroimaging techniques; use of conformal radiation therapy and stereotactic radiotherapy; involvement of clinical psychologists, speech, physical and occupational therapists has helped to improve survival and quality of life of these patients [3]. Although there have been several reports regarding the incidence and prevalence of pediatric brain tumors in the western and far eastern literature [4, 5], such studies have been distinctly lacking in the Indian subcontinent. Till date, there is only one published multi-institutional study regarding the epidemiological profile of pediatric brain tumors in India incorporating seven tertiary hospitals located in various geographical regions across India [6]. The present retrospective study was carried out to analyze the epidemiology of pediatric brain tumors treated at a tertiary cancer center located in south India. With the concurrence of Institutional Ethics and Review Board the authors have retrospectively analyzed patients (age 0–14 y) registered between 2003 and 2009. The authors included all suspected/diagnosed malignancy cases that were referred to authors’ centre from other health care centers of south India. All undiagnosed cases were evaluated thoroughly and outside diagnosed cases were re-confirmed again either by histopathological slides/block review or repeat testing. The hospital database which was made as per ICD- O 10 was
Indian J Pediatr 38
40 33
35
33
31
30
27
26
25
21
20
15
14
15
Table 1 Spectrum of brain tumor histology
20
23
21
male female
12
10 5 0 2003
2004
2005
2006
2007
2008
2009
Fig. 1 Year wise brain tumor incidence
utilized for information regarding final diagnosis of all brain tumors registered for treatment at authors’ institute. Case files of individual patient were analyzed for information regarding age, gender of the patients and histological types of tumor.
Results A total of 341 patients were diagnosed with brain tumors out of 2,844 patients registered with pediatric oncology department at authors’ institute between 2003 and 2009, accounting for 11.99 % of all cases. There were 209 (11.67 %) males and 132 (12.52 %) females (Fig. 1). Male, female ratio was 1.58:1. The patients were divided into three age groups; 0–2 y, >2–5 y and >5–14 y. Maximum number of patients were in age group of >5–14 y (73.6 %) followed by 2–5 y (20.52 %) and 0–2 y (5.85 %) with a male preponderance in all age groups (Fig. 2). Medulloblastoma was the commonest brain tumor accounting for 36 % of cases in present study. Other six most common brain tumors in decreasing frequencies were astrocytoma (22.87 %), ependymoma (9.67 %), glioma (9.38 %), oligodendroglioma (3.51 %), peripheral neuroectodermal tumor (2.63 %) and germ cell tumor (2 %) (Table 1). Other less common tumors were choroid plexus tumor, craniopharyngioma, atypical teratoid rhabdoid tumor, rhabdomyosarcoma and neuroblastoma. In the present study, the patients with age group 0–2 y were less and accounting only 147
160 140 120
No. (%)
Medulloblastoma Astrocytoma Low grade astrocytoma Anaplastic astrocytoma GBM Ependymoma Low grade Anaplastic Glioma Brain stem Optic nerve Other site Oligodendroglioma Low grade High grade PNET
126 (36.95) 78 (22.87) 29 8 41 33 (9.67) 16 17 32 (9.38) 24 1 7 12 (3.51) 6 6 9 (2.63): Temporal: 2, frontal: 4, brain not specified: 1, parietal: 2 7 (2) 6 (1.75) 5 (1.47) 3 (0.87): Temporal: 1, brain non specified: 1, cerebellum: 1 2 (0.85): Parietal lobe: 1, brain stem: 1 2 (0.85) 2 (0.85) 3 (0.87) 16 (4.69)
GCT Choroid plexus tumor Craniopharyngioma ATRT RMS Ganglioneuroblastoma Pinealoblastoma Neuroblastoma Malignant tumor, unknown primary Others Total
5 (1.47) 341
GBM Glioblastoma multiforme; PNET Primitive neuroectodermal tumor; GCT Germ cell tumor; ATRT Atypical teratoid rhabdoid tumor; RMS Rhabdomyosarcoma
5 % of total cases. The three most common tumors in this group were medulloblastoma, ependymoma and astrocytoma. Interestingly in the age group of >2–5 y also the most common tumors were same as the age group 0–2 y. However, patients with age group of >5–14 y the three most common tumor were medulloblastoma, astrocytoma and glioma (Table 2).
104
100 male 80
female
51
60 40 20
Tumor categories 27
11
20 8
0 0-2
>2-5
Fig. 2 Age and sex distributions
>5-14
Table 2 Age wise three most common brain tumors Age group
Three most common tumors (in decreasing order)
0–2 y >2–5 y
Medulloblastoma, ependymoma, astrocytoma Medulloblastoma, ependyemoma, astrocytoma
>5–14 y
Medulloblastoma, astrocytoma, glioma. Other tumors in decreasing frequencies were ependymoma, oligodendroglioma and germ cell tumor
Indian J Pediatr
Discussion In India, the incidence of pediatric brain tumor varies from 0 to 21.1 % according to Indian council of medical research (ICMR), national cancer registry data. In north east India incidence rate is 0 % and in Mumbai it is 21.1 % [7, 8]. In the present study, brain tumors accounted for 11.99 % of all pediatric malignancies and as noted in other centers in India, the present study also showed that the incidence was less when compared with western literature. There were 209 (11.67 %) males and 132 (12.52 %) females in the present study revealing a striking male preponderance with a M: F ratio of 1.58:1. This male preponderance of pediatric CNS tumors was also observed in other centres in India. In the present study, six most common pediatric brain tumors in descending frequencies were medulloblastoma, astrocytoma, ependymoma, glioma, oligodendroglioma and PNET. Medulloblastoma was the most common pediatric CNS tumor reported by Packer et al. [9] and Burger et al. [10] favoring the present result . However, in a large meta-analysis by Rickert and Paulus [5], various European and Asian studies reported that astrocytoma was the most common brain tumor in pediatric age group followed by medulloblastoma. The variation in incidence rate from western literature can be due to the fact that the present data is based on hospital based registry which is a major limitation of the present study. This study may not be a reflection of actual incidence of pediatric brain tumors as many cases would have been treated elsewhere and not referred to authors’ institute. But it can give a rough idea about the spectrum of diseases. Population based statistical data on pediatric brain tumor will help in assessing the magnitude of this cancer problem in our country. Guarantor Dr. L. Appaji will act as guarantor for this paper. Conflict of Interest None.
Role of Funding Source None.
References 1. U.S. Cancer Statistics Working Group. United States Cancer Statistics: 1999–2009 incidence and mortality web-based report. Atlanta: U.S. Department of Health and Human Services, Centers for Disease Control and Prevention and National Cancer Institute; 2013. Available at: www.cdc.gov/uscs. 2. Strother DR, Pollack IF, Fisher PG, Hunter JV, Woo SY, Pomeroy SL, et al. Tumors of the central nervous system. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. 4th ed. Philadelphia: Lippincott Williams and Wilkins; 2002. pp. 751– 824. 3. Khatua S, Sadighi ZS, Pearlman ML, Bochare S, Vats TS. Brain tumors in children–current therapies and newer directions. Indian J Pediatr. 2012;79:922–7. 4. Rosemberg S, Fujiwara D. Epidemiology of pediatric tumors of the nervous system according to the WHO 2000 classification: A report of 1,195 cases from a single institution. Childs Nerv Syst. 2005;21:940–4. 5. Rickert CH, Paulus W. Epidemiology of central nervous system tumors in childhood and adolescence based on the new WHO classification. Childs Nerv Syst. 2001;17:503–11. 6. Jain A, Sharma MC, Suri V, Kale SS, Mahapatra AK, Tatke M, et al. Spectrum of pediatric brain tumors in India: A multi-institutional study. Neurol India. 2011;59:208–11. 7. Consolidated Report of Population Based Cancer Registries 2001-2004. National cancer registry programme, Indian Council of Medical Research, Bangalore, India, Dec 2006. Available from: http://www. icmr.nic.in/ncrp/report_pop_2001-04/cancer_p_based.htm. 8. First report of the population based cancer registries under North Eastern Regional cancer registry 2003–2004. National cancer registry programme, Indian Council of Medical Research, Bangalore, India, Sep 2006. Available from: http://www.icmr.nic.in/ncrp/first_report_ 2003-04/first_report.htm. 9. Packer RJ, Cogen P, Vezina G, Rorke LB. Medulloblastoma: Clinical and biologic aspects. Neuro-Oncol. 1999;1:232–50. 10. Burger PC, Scheithaur BW. Embryonal tumors. In: Tumors of the Central Nervous System, AFIP Atlas of Tumor Pathology. Series 4, fascicle 7. Washington DC: American Registry of Pathology; 2007. pp. 251–95.
CLINICAL BRIEF
Spectrum of Pediatric Brain Tumors: A Report of 341 Cases from a Tertiary Cancer Center in India Umesh Das & L. Appaji & B. S. Aruna Kumari & Nagesh T. Sirsath & M. Padma & S. Kavitha & T. Avinash & K. C. Lakshmaiah
Received: 27 November 2013 / Accepted: 20 February 2014 # Dr. K C Chaudhuri Foundation 2014
Abstract Brain tumors are the second most common cancers after hematological malignancies accounting for approximate 21 % of all childhood malignancies in children between ages of 0 and 14 y. The present study was undertaken to determine the spectrum of the brain tumors diagnosed in a tertiary cancer center in South India. A retrospective analysis of the data of pediatric brain tumors diagnosed between 2003 and 2009 was done and data was classified according to the age, gender and histology types. Out of 2,844 pediatric patients, 341 (11.99 %) were diagnosed as having brain tumors. Most of the patients were in the age group of >5–14 y. Male to female ratio was 1.58:1. The most common pediatric brain tumor was medulloblastoma followed by astrocytoma and ependymoma. Glioblastoma multiforme was the most common subtype of astrocytoma. Other common tumors were glioma, oligodendroglioma, periphereral neuroectodermal tumor and germ cell tumor. As compared to western data, incidence of brain tumors in children was found to be less in the present study.
Keywords Brain tumor . Medulloblastoma . Astrocytoma
U. Das : N. T. Sirsath : K. C. Lakshmaiah Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bangalore, Karnataka, India L. Appaji : B. S. A. Kumari : M. Padma : S. Kavitha : T. Avinash Department of Pediatric Oncology, Kidwai Memorial Institute of Oncology, Bangalore, Karnataka, India U. Das (*) Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Room No. 5, OPD Block, Bangalore 560029, Karnataka, India e-mail: [email protected]
Introduction Brain tumors are the most common solid tumors of childhood and the second most common malignancies after hematological malignancies accounting for approximate 21 % of all childhood malignancies in children between 0 and 14 y of age [1]. Apart from being a major cause of cancer related deaths, there is significant morbidity both from the disease and from the treatment with varying degrees of physical, cognitive, neurological, endocrinological, and other deficits in survivors resulting in significant handicap and diminished quality of life in brain tumor patients [2]. However, recently use of advanced surgical and neuroimaging techniques; use of conformal radiation therapy and stereotactic radiotherapy; involvement of clinical psychologists, speech, physical and occupational therapists has helped to improve survival and quality of life of these patients [3]. Although there have been several reports regarding the incidence and prevalence of pediatric brain tumors in the western and far eastern literature [4, 5], such studies have been distinctly lacking in the Indian subcontinent. Till date, there is only one published multi-institutional study regarding the epidemiological profile of pediatric brain tumors in India incorporating seven tertiary hospitals located in various geographical regions across India [6]. The present retrospective study was carried out to analyze the epidemiology of pediatric brain tumors treated at a tertiary cancer center located in south India. With the concurrence of Institutional Ethics and Review Board the authors have retrospectively analyzed patients (age 0–14 y) registered between 2003 and 2009. The authors included all suspected/diagnosed malignancy cases that were referred to authors’ centre from other health care centers of south India. All undiagnosed cases were evaluated thoroughly and outside diagnosed cases were re-confirmed again either by histopathological slides/block review or repeat testing. The hospital database which was made as per ICD- O 10 was
Indian J Pediatr 38
40 33
35
33
31
30
27
26
25
21
20
15
14
15
Table 1 Spectrum of brain tumor histology
20
23
21
male female
12
10 5 0 2003
2004
2005
2006
2007
2008
2009
Fig. 1 Year wise brain tumor incidence
utilized for information regarding final diagnosis of all brain tumors registered for treatment at authors’ institute. Case files of individual patient were analyzed for information regarding age, gender of the patients and histological types of tumor.
Results A total of 341 patients were diagnosed with brain tumors out of 2,844 patients registered with pediatric oncology department at authors’ institute between 2003 and 2009, accounting for 11.99 % of all cases. There were 209 (11.67 %) males and 132 (12.52 %) females (Fig. 1). Male, female ratio was 1.58:1. The patients were divided into three age groups; 0–2 y, >2–5 y and >5–14 y. Maximum number of patients were in age group of >5–14 y (73.6 %) followed by 2–5 y (20.52 %) and 0–2 y (5.85 %) with a male preponderance in all age groups (Fig. 2). Medulloblastoma was the commonest brain tumor accounting for 36 % of cases in present study. Other six most common brain tumors in decreasing frequencies were astrocytoma (22.87 %), ependymoma (9.67 %), glioma (9.38 %), oligodendroglioma (3.51 %), peripheral neuroectodermal tumor (2.63 %) and germ cell tumor (2 %) (Table 1). Other less common tumors were choroid plexus tumor, craniopharyngioma, atypical teratoid rhabdoid tumor, rhabdomyosarcoma and neuroblastoma. In the present study, the patients with age group 0–2 y were less and accounting only 147
160 140 120
No. (%)
Medulloblastoma Astrocytoma Low grade astrocytoma Anaplastic astrocytoma GBM Ependymoma Low grade Anaplastic Glioma Brain stem Optic nerve Other site Oligodendroglioma Low grade High grade PNET
126 (36.95) 78 (22.87) 29 8 41 33 (9.67) 16 17 32 (9.38) 24 1 7 12 (3.51) 6 6 9 (2.63): Temporal: 2, frontal: 4, brain not specified: 1, parietal: 2 7 (2) 6 (1.75) 5 (1.47) 3 (0.87): Temporal: 1, brain non specified: 1, cerebellum: 1 2 (0.85): Parietal lobe: 1, brain stem: 1 2 (0.85) 2 (0.85) 3 (0.87) 16 (4.69)
GCT Choroid plexus tumor Craniopharyngioma ATRT RMS Ganglioneuroblastoma Pinealoblastoma Neuroblastoma Malignant tumor, unknown primary Others Total
5 (1.47) 341
GBM Glioblastoma multiforme; PNET Primitive neuroectodermal tumor; GCT Germ cell tumor; ATRT Atypical teratoid rhabdoid tumor; RMS Rhabdomyosarcoma
5 % of total cases. The three most common tumors in this group were medulloblastoma, ependymoma and astrocytoma. Interestingly in the age group of >2–5 y also the most common tumors were same as the age group 0–2 y. However, patients with age group of >5–14 y the three most common tumor were medulloblastoma, astrocytoma and glioma (Table 2).
104
100 male 80
female
51
60 40 20
Tumor categories 27
11
20 8
0 0-2
>2-5
Fig. 2 Age and sex distributions
>5-14
Table 2 Age wise three most common brain tumors Age group
Three most common tumors (in decreasing order)
0–2 y >2–5 y
Medulloblastoma, ependymoma, astrocytoma Medulloblastoma, ependyemoma, astrocytoma
>5–14 y
Medulloblastoma, astrocytoma, glioma. Other tumors in decreasing frequencies were ependymoma, oligodendroglioma and germ cell tumor
Indian J Pediatr
Discussion In India, the incidence of pediatric brain tumor varies from 0 to 21.1 % according to Indian council of medical research (ICMR), national cancer registry data. In north east India incidence rate is 0 % and in Mumbai it is 21.1 % [7, 8]. In the present study, brain tumors accounted for 11.99 % of all pediatric malignancies and as noted in other centers in India, the present study also showed that the incidence was less when compared with western literature. There were 209 (11.67 %) males and 132 (12.52 %) females in the present study revealing a striking male preponderance with a M: F ratio of 1.58:1. This male preponderance of pediatric CNS tumors was also observed in other centres in India. In the present study, six most common pediatric brain tumors in descending frequencies were medulloblastoma, astrocytoma, ependymoma, glioma, oligodendroglioma and PNET. Medulloblastoma was the most common pediatric CNS tumor reported by Packer et al. [9] and Burger et al. [10] favoring the present result . However, in a large meta-analysis by Rickert and Paulus [5], various European and Asian studies reported that astrocytoma was the most common brain tumor in pediatric age group followed by medulloblastoma. The variation in incidence rate from western literature can be due to the fact that the present data is based on hospital based registry which is a major limitation of the present study. This study may not be a reflection of actual incidence of pediatric brain tumors as many cases would have been treated elsewhere and not referred to authors’ institute. But it can give a rough idea about the spectrum of diseases. Population based statistical data on pediatric brain tumor will help in assessing the magnitude of this cancer problem in our country. Guarantor Dr. L. Appaji will act as guarantor for this paper. Conflict of Interest None.
Role of Funding Source None.
References 1. U.S. Cancer Statistics Working Group. United States Cancer Statistics: 1999–2009 incidence and mortality web-based report. Atlanta: U.S. Department of Health and Human Services, Centers for Disease Control and Prevention and National Cancer Institute; 2013. Available at: www.cdc.gov/uscs. 2. Strother DR, Pollack IF, Fisher PG, Hunter JV, Woo SY, Pomeroy SL, et al. Tumors of the central nervous system. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. 4th ed. Philadelphia: Lippincott Williams and Wilkins; 2002. pp. 751– 824. 3. Khatua S, Sadighi ZS, Pearlman ML, Bochare S, Vats TS. Brain tumors in children–current therapies and newer directions. Indian J Pediatr. 2012;79:922–7. 4. Rosemberg S, Fujiwara D. Epidemiology of pediatric tumors of the nervous system according to the WHO 2000 classification: A report of 1,195 cases from a single institution. Childs Nerv Syst. 2005;21:940–4. 5. Rickert CH, Paulus W. Epidemiology of central nervous system tumors in childhood and adolescence based on the new WHO classification. Childs Nerv Syst. 2001;17:503–11. 6. Jain A, Sharma MC, Suri V, Kale SS, Mahapatra AK, Tatke M, et al. Spectrum of pediatric brain tumors in India: A multi-institutional study. Neurol India. 2011;59:208–11. 7. Consolidated Report of Population Based Cancer Registries 2001-2004. National cancer registry programme, Indian Council of Medical Research, Bangalore, India, Dec 2006. Available from: http://www. icmr.nic.in/ncrp/report_pop_2001-04/cancer_p_based.htm. 8. First report of the population based cancer registries under North Eastern Regional cancer registry 2003–2004. National cancer registry programme, Indian Council of Medical Research, Bangalore, India, Sep 2006. Available from: http://www.icmr.nic.in/ncrp/first_report_ 2003-04/first_report.htm. 9. Packer RJ, Cogen P, Vezina G, Rorke LB. Medulloblastoma: Clinical and biologic aspects. Neuro-Oncol. 1999;1:232–50. 10. Burger PC, Scheithaur BW. Embryonal tumors. In: Tumors of the Central Nervous System, AFIP Atlas of Tumor Pathology. Series 4, fascicle 7. Washington DC: American Registry of Pathology; 2007. pp. 251–95.
