2156
Primary Pediatric Brain Tumors
Statistics of Taipei VGH, Taiwan (1975–2004)
Tai-Tong Wong, M.D.1
Donald M. Ho, M.D.2
Kai-Ping Chang, M.D.3
Sang-Hue Yen, M.D.4
Wan-You Guo, M.D., Ph.D.5
Feng-Chi Chang, M.D.5
Muh-Lii Liang, M.D.1
Hung-Chi Pan, M.D.1
Wen-Yuh Chung, M.D.1
1
Department of Neurosurgery, Neurological Insti-
tute, Taipei Veterans General Hospital and National
Yang Ming University, School of Medicine, Taiwan,
Republic of China.
2
Department of Pathology and Laboratory Medi-
cine, Taipei Veterans General Hospital and National
Yang Ming University, School of Medicine, Taiwan,
Republic of China.
3
Department of Pediatrics, Taipei Veterans Gen-
eral Hospital and National Yang Ming University,
School of Medicine, Taiwan, Republic of China.
4
Cancer Center, Taipei Veterans General Hospital
and National Yang Ming University, School of Med-
icine, Taiwan, Republic of China.
5
Department of Radiology, Taipei Veterans Gen-
eral Hospital and National Yang Ming University,
School of Medicine, Taiwan, Republic of China.
Supported by the Pediatric Neuroscience Research
Fund of Taipei Veterans General Hospital, Taipei,
Taiwan, Republic of China.
Address for reprints: Tai-Tong Wong M.D., Division
of Pediatric Neurosurgery, Neurological Institute,
Taipei Veterans General Hospital, VACRS, No. 210,
Sec 2, Shih-Pai, Taipei, Taiwan, Republic of China,
11217; Fax: (011) 886-2-28757587; E-mail:
ttwong@vghtpe.gov.tw
Received February 9, 2005; revision received April
29, 2005; accepted June 3, 2005.
© 2005 American Cancer Society
DOI 10.1002/cncr.21430
Published online 11 October 2005 in Wiley InterScience (www.interscience.wiley.com).
BACKGROUND. The purpose of the current study was to investigate a hospital series
of 986 cases of primary pediatric brain tumors in Taiwan.
METHODS. The authors reviewed the database of primary pediatric brain tumors in
patients ⬍ 18 years of age collected in Taipei Veterans General Hospital (Taipei
VGH) from 1975 to May 2004. Age and gender distribution, location, and classifi-
cation of brain tumors were analyzed. Intracranial tumors with diagnostic imaging
were included. Nontumoral lesions, cysts, and vascular malformations were ex-
cluded.
RESULTS. The mean age of these 986 patients was 7.8 years, and the male to female
ratio was 1.4:1. Supratentorial (including pineal– quadrigeminal) located tumors
(58.3%) was predominant to infratentorial tumors (41.1%). In these series, 886
patients had either histologic diagnosis (842 patients) or clinical diagnosis (44
patients). The most common 5 categories of tumors were astrocytic tumors
(31.1%), germ cell tumors (14.0%), medulloblastomas (13.3%), craniopharyngio-
mas (8.3%), and ependymal tumors (5.8%). Atypical teratoid/rhabdoid tumors
(AT/RTs), a rare but highly malignant tumor, were 2.1%. The high incidence of
primary intracranial germ cell tumors correlated with reported series from Japan
and Korea. For the remaining 100 patients without diagnostic classifications, the
majority were most likely astrocytic tumors in brain stem.
CONCLUSIONS. The authors analyzed a large hospital series of primary brain tumors
in children. Both histologically verified and unverified tumors were recruited to
avoid selective bias. Although it was not a study of a population-based brain tumor
registry, it could still be representative of primary pediatric brain tumors in Tai-
wan. Cancer 2005;104:2156 – 67. © 2005 American Cancer Society.
KEYWORDS: brain tumor, childhood, demographic data, Taiwan.
I n Taiwan, the earliest reported series of pediatric brain tumor was
126 cases at the National Defense Medical Center (NDMC) in 1977
and 87 cases at the National Taiwan University Hospital (NTUH) in
1980.1,2 Taipei VGH later reported a series of 171 CT scan detected
childhood brain tumors in 1987.3 Since then, large series of pediatric
brain tumors has not been studied. From 1975 to May 2004, we
collected 986 cases of primary brain tumors in children younger than
18 years of age in Taipei VGH. Demographic data (age distribution,
gender ratio, location, and classification of tumors) of these patients
were reviewed and analyzed. We were interested in classifying either
histologically or clinically the tumors that were detected by neuroim-
aging studies.
MATERIALS AND METHODS
In this study, we reviewed the database of 986 cases of primary
pediatric brain tumors of younger than 18 years of age collected in
Taipei VGH from 1975 to May 2004. Primary intracranial tumors that
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Primary Pediatric Brain Tumors/Wong et al. 2157

were diagnosed by CT scan or MRI were included.

Nontumoral lesions, cysts, and vascular malforma-
tions were excluded. Among them, 849 (86.1%) pa-
tients received surgeries for tumors, and 842 (85.4%)
had histologic diagnosis. Of the 144 patients without
histologic diagnosis, 44 had clinical diagnosis. Eighty-
four patients were referred after initial tumor surgeries
in other hospitals, and 46 of them received further
tumor surgeries at Taipei VGH for residue or recurrent
tumors. Twenty-six patients died within 1 month after
tumor surgeries with a surgical mortality rate of 3.2%.
Demographic data (age, gender, location, and classi-
fication of tumors) of these patients were analyzed. FIGURE 1. Chart graphically depicts the age distribution of 986 patients.
The distribution of tumors was defined by imaging
studies and/or operative findings. Tumor locations TABLE 1
Age and Sex Distribution of Pediatric Brain Tumors (N ⴝ 986)
were counted as supratentorial, infratentorial, both
supratentorial and infratentorial, and also their re- Male Female Total
lated neuroanatomic locations. Tumors with histo-
logic diagnosis were classified according to the World Age yrs No. % No. % No. %
Heath Organization 2000 classification as closely as
0–1 36 3.7 25 2.5 61 6.2
possible.4 For tumors that were operated upon in Tai-
0–2 68 6.9 69 7.0 137 13.9
pei VGH, the histologic diagnosis was reviewed and 0–5 173 17.5 133 13.5 306 31.0
reconfirmed by one senior neuropathologist. For tu- 6–10 197 20.0 148 15.0 345 35.0
mors without tissue proof, the diagnoses and classifi- 11–15 177 18.0 107 10.8 284 28.8
cations were made from related specific clinical fea- 16–18 32 3.2 19 1.9 51 5.2
Total 579 58.7 407 41.3 986 100
tures, image findings, and locations of tumors. For
germ cell tumors (GCTs) without histologic verifica- Average age: 7.8 yrs Male to female ratio: 1.4
tion, the diagnosis and classification were achieved
through image findings, locations, tumor markers and
response to radiotherapy and/or chemotherapy. Brain mors (2.2 yrs), supratentorial primitive neuroectoder-
tumors of phacomatoses without surgical resection mal tumors (PNET) (3.5 yrs), AT/RTs (4.2 yrs), pine-
were classified according to specific locations, and oblastomas (4.8 yrs), and ependymal tumors (6.0 yrs)
entities of central nervous system (CNS) tumors oc- occurred in younger age groups. Medulloblastomas
curred in different syndromes. For hypothalamic (7.0 yrs), astrocytic tumors (7.5 yrs), craniopharyngio-
hamartomas, the classification could be made simply mas (7.8 yrs), and oligodendroglioma (7.9 yrs) were
through magnetic resonance imaging (MRI) findings. common tumors in children 7– 8 years old. Neuronal
We were also interested in studying data of several and mixed neuronal glial tumors (8.6 yrs), germ cell
specific entities: GCTs, atypical teratoid/rhabdoid tu- tumors (10.6 yrs), tumor of meninges (10.7 yrs), and
mors (AT/RTs), phacomatoses, and radiation-induced pituitary tumors (14.3 yrs) were usually diagnosed in
second tumors. older children. The age distribution and gender ratio
of different types of tumors are listed in Tables 2–3.
RESULTS
Age Distribution and Gender Ratio Gender ratio (male to female ratio) of different types of
Of these 986 patients, the mean age was 7.8 years tumors
(range from the first day of life to 18 yrs) with peak Of the five most common types of tumors in children,
frequency at 2, 6, 10, and 13 years. The tumors were the gender ratios of astrocytic tumors, ependymal tu-
diagnosed within 1-year-olds in 61 (6.2%) patients. mors, craniopharyngiomas, medulloblastomas, and
There were 579 (58.7%) boys and 407 (41.3%) girls, germ cell tumors were 1.0, 1.4, 1.5, 1.9, and 3.8, re-
with male to female ratio of 1.4:1. Except the age spectively. Except astrocytic tumors, all other four
groups of 2 and 8 years, there was male predominance types of tumors were predominantly in males. This
in all other age groups (Fig.1, Table 1). tendency of male predominance (above the average
ratio of 1.4) was clear in medulloblastoma and germ
Age distribution of different types of tumors cell tumors. For germ cell tumors, only yolk sac tu-
According to the mean age at diagnosis, immature mors or tumors in the suprasellar region were equal in
teratomas in ventricles (2.1 yrs), choroids plexus tu- gender ratio or female predominant (Tables 2–3).
 10970142, 2005, 10, Downloaded from https://acsjournals.onlinelibrary.wiley.com/doi/10.1002/cncr.21430 by Readcube-Labtiva, Wiley Online Library on [16/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
2158 CANCER November 15, 2005 / Volume 104 / Number 10

TABLE 2
Classification and Age and Gender Distributions of Primary Pediatric Brain Tumors in Taipei VGH

Cases of Cases of Gender Percentage

No. of histology clinical ratio histologic Percentage
Types of tumors patients diagnosis diagnosis Mean age (range) (M/F) diagnosis N ⴝ 986

Gliomas 403 401 2 7.0 yrs (3 days–17.8 yrs) 1.1 99.5 40.9
Astrocytic tumors 307 305 2 7.5 yrs (3 days–17.8 yrs) 1.0 99.3 31.1
Pilocytic astrocytoma 131 131 0 7.0 yrs (4.4 mos–16.4 yrs) 1.0 100 13.3
Astrocytoma 74 74 0 7.5 yrs (4.4 mos–15.7 yrs) 1.0 100 7.5
Anaplastic astrocytoma 45 45 0 8.6 yrs (3 days–16.6 yrs) 1.3 100 4.6
Glioblastoma 39 39 0 7.0 yrs (2.8 mos–17.8 yrs) 0.7 100 4.0
Giant cell astrocytoma 13 11 2 8.0 yrs (1–11.6 yrs) 3.3 84.6 1.3
PXAa 4 4 0 7.8 yrs (7.9 mos–13 yrs) 4/0 100 0.4
Primary leptomeningeal gliomatosis 1 1 0 12.5 yrs 0/1 100 0.1
Oligodendroglial tumors 12 12 0 7.9 yrs (1.1 mos–15.1 yrs) 1.4 100 1.2
Ependymal tumors 56 56 0 6.0 yrs (3.8–17.4 mos) 1.3 100 5.7
Ependymoma 25 25 0 5.8 yrs (10.1 mos–16.8 yrs) 1.3 2.5
Anaplastic ependymoma 31 31 0 6.0 yrs (3.8 mos–17.4 yrs) 1.4 3.1
Choroid plexus tumors 19 19 0 2.2 yrs (0.5 mos–12.4 yrs) 1.4 100 1.9
Choroid plexus papilloma 17 17 0 2.4 yrs (0.5 mos–12.4 yrs) 1.1 1.7
Choroid plexus carcinoma 2 2 0 1.2 yrs (9.0 mos–1.6 yrs) 2/0 0.2
Glial tumor of uncertain origin 1 1 0 9.0 yrs 0/1 100 0.1
Astroblastoma —
Mixed gliomas 7 7 0 6.1 yrs (5.6 mos–12.7 yrs) 2.5 100 0.7
Embryonal tumors 165 165 0 6.4 yrs (1 day–18 yrs) 1.7 100 16.7
Medulloblastomas 131 131 0 7.0 yrs (2.5 mos–18 yrs) 1.9 100 13.2
PNET/supratentorial 12 12 0 3.5 yrs (1 day–13 yrs) 0.7 100 1.2
Ependymoblastoma 1 1 0 1.7 yrs 1/0 100 0.1
AT/RTs 21 21 0 4.2 yrs (0.5 mos–10.7 yrs) 1.6 100 2.1
Germ cell tumors 138 105 33 10.6 yrs (3 days–18 yrs) 3.8 75.5 14.0
Germinoma 75 52 23 11.9 yrs (1.3–18 yrs) 4.0 69.3 7.6
Teratoma, mature 8 8 0 6.9 yrs (8.5 mos–15.3 yrs) 8/0 100 0.8
Teratoma, immature 14 14 0 6.8 yrs (7 days–14.4 yrs) 13 100 1.4
YST, pure 10 10 0 10.0 yrs (2.6–14.5 yrs) 1.0 100 1.0
Mixed GCTsb 19 19 0 10.1 yrs (1.6–15.4 yrs) 2.8 100 1.9
G ⫹ T (MT2/IT3) 5 5 0
G ⫹ IT ⫹ YST 4 4 0
G ⫹ AFP/HCG 2 2 0
T(MT2/IT5)⫹YST 7 7 0
MT ⫹ unclassified GCT 1 1 0
Unclassified GCT 2 2 0 7.2 yrs (5.8–8.7 yrs) 2/0 100 0.2
Diagnosed by markers 10 0 10 11 yrs (3 days–16.5 yrs 2.3 0 1.0
Craniopharyngioma 82 81 1 7.8 yrs (7.3 mos–17.7 yrs) 1.5 98.8 8.3
Neuronal and mixed neuronal-glial
tumors 21 21 0 8.6 yrs (1.3–17.8 yrs) 0.8 100 2.1
Ganglioglioma 12 12 0 8.5 yrs (1.4–17.8 yrs) 0.7 100 1.2
Anaplastic ganglioglioma 2 2 0 5.2 yrs (2.6–7.8 yrs) 1 100 0.2
DNT 5 5 0 10.4 yrs (6.6–14.6 yrs) 0.7 100 0.5
Desmoplastic infantile
astrocytoma/ganglioglioma 1 1 0 5.2 yrs 1/0 100 0.1
Central neurocytoma 1 1 0 8.6 yrs 0/1 100 0.1
Hamartoma 9 4 5 4.5 yrs (8.8 mos–12.8 yrs) 0.1 44.4 0.9
Pineal parenchymal tumors 9 9 0 5.2 yrs (1.6–13 yrs) 0.8 100 0.9
Pineoblastoma 8 8 0 4.8 yrs (1.6–13 yrs) 1.0 100 0.8
Pineocytoma 1 1 0 8.7 yrs 0/1 100 0.1
Tumors of meninges 19 19 0 10.9 yrs (11.9 mos–17.4 yrs) 0.7 100 1.9
Meningiomas 13 13 0 10.6 yrs (11.9 mos–17.4 yrs) 0.6 100 1.3
Dural sarcoma 5 5 0 10.6 yrs (1.6–14.6 yrs) 0.7 100 0.5
(continued)
 10970142, 2005, 10, Downloaded from https://acsjournals.onlinelibrary.wiley.com/doi/10.1002/cncr.21430 by Readcube-Labtiva, Wiley Online Library on [16/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
Primary Pediatric Brain Tumors/Wong et al. 2159

TABLE 2
(continued)

Cases of Cases of Gender Percentage

No. of histology clinical ratio histologic Percentage
Types of tumors patients diagnosis diagnosis Mean age (range) (M/F) diagnosis N ⴝ 986

Chondroma 1 1 0 15.3 yrs 1/0 100 0.1

Tumors of peripheral nerve 14 14 0 12.7 yrs (5.4–17.9 yrs) 1.0 50 1.4
Vestibular schwannoma 13 13 0 12.8 yrs (5.4–17.9 yrs) 1.2 46.2 1.3
MPNSTc 1 1 0 11.5 yrs 0/1 100 0.1
Pituitary adenomas 10 10 0 14.3 yrs (12.4–17.7 yrs) 2.3 100 1.0
Primary melanocytic lesions 2 2 0 7.7 yrs (1.5–13.9 yrs) 0/2 100 0.2
Malignant melanoma
Tumors of uncertain histogenesis 2 0 0 10.8 yrs (6.7–14.8 yrs) 1/1 100 0.2
Hemangioblastoma —
Chordoma 5 5 0 7.6 yrs (4.4–13 yrs) 1.5 100 0.5
Tumor without histology clinical
diagnosisd 100 0 0 7.8 yrs (0.9 mos–17.9 yrs) 1.5 0 10.1

a
PXA: pleomorphic xanthoastrocytoma.
b
Mixed GCTs: G ⫹ T(MT/IT) represents mixed GCTs with components of germinoma (G), mature teratoma (MT) or immature teratoma (IT); G ⫹ IT ⫹ YST represents mixed GCTs with components of germinoma
(G), immature teratoma (IT), and yolk sac tumor (YST); G ⫹ AFP/HCG represents mixed GCTs with components germinoma with elevated serum titer of AFP with/without elevated serum titer of HCG; T(MT/IT)⫹YST
represents GCTs with component of teratoma (mature teratoma or immature teratoma) and yolk sac tumor; MT ⫹ unclassified GCT represents mixed GCT with components of mature teratoma and unclassified
GCT.
c
MPNST: malignant Triton tumor arising from trigeminal nerve.
d
Tumors without histologic or clinical diagnosis included 73 tumors in brainstem, 5 tumors in cerebral hemisphere, 5 tumors in thalamus, 1 tumor in hypothalamus, 5 tumors in sellar-suprasellar region, 5 tumors
in pineal region, 2 tumors in basal ganglia, and 4 tumors in cerebellar vermis and 4th ventricle. The majority of these tumors may be astrocytic tumors.

Classification of Tumors mas would be up to 50% of primary brain tumors in

With reference to the World Heath Organization 2000 children (Tables 2–3).
classification,4 we classified and counted relative inci-
dence of different types of brain tumors with either Specific entities of tumors
histologic diagnosis or clinical diagnosis in the whole Germ cell tumors (GCTs). In this series, a total of 138
series. In this series of 986 patients, 842 (85.4%) pa- GCTs were diagnosed at an average age of 10.7 years
tients had histologic diagnoses, and the other 44 pa- with a male to female ratio of 3.8:1. One hundred-five
tients had clinical diagnoses. The common entities of patients had histologic verification, and the remaining
tumors were astrocytic tumors (31.1%), germ cell tu- 33 were diagnosed clinically. Of these 33 histologically
mors (14.0%), medulloblastomas (13.2%), craniophar- unverified GCTs, 23 tumors were defined as germino-
yngiomas (8.3%), and ependymal tumors (5.7%). Rel- mas, and 10 tumors were defined as nongerminoma-
ative rare entities of tumors included neuronal and tous malignant germ cell tumors (NG-MGCTs). The
mixed neuronal glial tumors (2.1%), AT/RTs (2.1%), diagnosis was made according to clinical features,
tumors of meninges (1.9%), tumors of choroids plexus neuroimaging, serum tumor markers (alpha-fetapro-
(1.9%), tumors of cranial nerves (1.4%), oligodendro- tein [AFP], beta human chorionic gonadotropin [beta-
glioma (1.2%), supratentorial PNET (1.2%), pituitary hCG]), and response to radiation therapy and/or che-
adenomas (1.0%), pineal parenchymal tumors (0.9%), motherapy. The diagnosed entities of these tumors
hamartomas (0.9%), chordomas (0.5%), hemangio- included germinomas (54.4%), mature and immature
blastomas (0.3%), primary melanocytic tumors (0.2%), teratomas (15.9%), pure yolk sac tumors (7.3%), mixed
ependymoblastomas (0.1%), and astroblastoma GCTs (13.8%), unclassified GCTs (1.5%), and tumors
(0.1%). For remaining 100 cases with tumors having diagnosed by tumor markers (7.3%). Among 19 cases
neither histologic nor clinical classification, there were of mixed GCTs, 16 cases had component of teratoma,
73 brainstem tumors. The majority of these unclassi- 11 cases had component of germinoma, 11 cases had
fied tumors in brainstem, cerebral hemisphere, optic component of yolk sac tumor, and 1 case had compo-
apparatus, and thalamus were most likely astrocytic nent of unclassified GCT. In two cases with compo-
tumors (Table 2). Classified gliomas constituted 40.8% nent of germinoma, the diagnosis of mixed GCTs re-
of tumors in this series. If most of the tumors without lied on elevated serum titer of AFP. Five of the 19
histologic diagnosis or clinical diagnosis were astro- mixed GCTs were initially teratomas (3 immature ter-
cytic tumors or other gliomas, the proportion of glio- atomas and 2 mature teratomas) in their first tumor
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2160 CANCER November 15, 2005 / Volume 104 / Number 10

TABLE 3
Classification, Locations, Age Distribution, Gender Ratio, and Percentage of Specific Types of Primary Intracranial Germ Cell Tumors (N ⴝ 138/
986)

Cases of Percentage
No. of histologic Sex ratio histologic Percentage
Types of tumors cases diagnosis Mean age (range) (M/F) diagnosis N ⴝ 986

Total 138 105 10.6 yrs (3 days–18 yrs) 3.8/1 76 14.0

Germinoma 75 52 11.9 yrs (1.3–18 yrs) 4 69.3 7.6
Suprasellar 19 15 11.8 yrs (5.6–16.5 yrs) 0.9 78.9 12.9
Pineal 29 19 11.7 yrs (1.3–17.7 yrs) 8.7 65.5 2.9
Suprasellar ⫹ pineal 7 6 14.5 yrs (8.7–18 yrs) 2.5 85.7 0.7
Basal ganglia 18 10 11.1 yrs (7.3–14.4 yrs) 18/0 55.6 1.8
Cerebral lobes 2 2 14.4 yrs (14–14.7 yrs) 2/0 100 0.2
Teratoma, mature 8 8 6.9 yrs (8.5 mos–15.3 yrs) 8/0 100 0.8
Pineal 7 7 7.8 yrs (1.9–15.3 yrs) 7/0 100 0.7
Deep hemisphere 1 1 8.5 mos. 1/0 100 0.1
Teratoma, immature 14 14 6.8 yrs (7 days–14.4 yrs) 13 100 1.4
Suprasellar 1 1 6.0 yrs 0/1 100 0.1
Pineal 4 4 12.0 yrs (7.2–14.3 yrs) 4 100 0.4
Basal ganglia 2 2 13.1 yrs (11.7–14.5 yrs) 2/0 100 0.2
Ventricle 7 7 2.1 yrs (7 days–13.2 yrs) 7/0 100 0.7
YST, pure 10 10 10.0 yrs (2.6–14.5 yrs) 1 100 1.0
Suprasellar 5 5 11.3 yrs (7.7–14.5 yrs) 1/4 100 0.5
Pineal 4 4 10.3 yrs (8.3–12.5 yrs) 3 100 0.4
Cerebellum 1 1 2.6 yrs 1/0 100 0.1
Mixed GCT 19 19 10.1 yrs (1.6–15.4 yrs) 2.8 100 1.9
Suprasellar 6 6 8.9 yrs (1.6–13.1 yrs) 1/1 100 0.6
Pineal 8 8 10.5 yrs (3–15.4 yrs) 3/1 100 0.8
Suprasellar ⫹ pineal 1 1 15.0 yrs 1/0 100 0.1
Basal ganglia 4 4 10.1 yrs (8.8–11.5 yrs) 4/0 100 0.4
Unclassified GCT 2 2 7.2 yrs (5.8–8.7 yrs) 2/0 100 0.2
Suprasellar 1 1 8.7 yrs 1/0 100 0.1
Pineal 1 1 5.8 yrs 1/0 100 0.1
Diagnosed by markers 10 0 11 yrs (3 days–16.5 yrs) 2.3 0 1.0
Suprasellar 4 0 11.7 yrs (7.1–16.5 yrs) 1 0 0.4
Pineal 5 0 9.3 yrs (3 days–13.5 yrs) 4 0 0.5
Suprasellar ⫹ pineal 1 0 16.4 yrs 1/0 0 0.1

resection. The pathologies changed to other types of
GCTs on recurrence.
Common locations of GCTs in these series were
pineal (58, 42%), suprasellar–pineal (9, 6.5%), sellar–
anterior third ventricle– hypothalamic region (36,
26.1%), cerebral hemisphere including 25 basal gan-
glia tumors and 2 cerebral lobe tumors (27, 19.6%),
and ventricle (7, 5.1%). The location, age distribution,
gender ratio, and percentage to the whole series of
different types of GCTs are shown in Tables 3– 8. In
these 138 GCTs, 111 tumors had serum tumor markers
(AFP, beta-hCG) studies at diagnosis. Eighty-two pa-
tients had both histologic diagnosis and serum tumor
marker studies. Correlation with different histologic
types of GCTs and serum titers of tumor markers is
shown in Table 4.
Atypical rhabdoid/teratoid tumors (AT/RTs). Among
165 embryonal tumors, there were 131 medulloblas-
tomas and 21 AT/RTs. AT/RTs constituting 2.1% of
primary brain tumors in this series. The mean age of
diagnosis was 4.2 years (range, 0.5 mos to 10.7 yrs)
with a male to female ratio of 1.6:1. These AT/RTs of
the brain were located predominately in the cere-
bellum (71.4%). Other locations included cerebral
hemisphere, pineal– quadrigeminal region, and lat-
eral ventricle. Cerebellar AR/RT mimics medullo-
blastoma. The ratio of cerebellar medulloblastoma
to cerebellar AT/RTs was 131/15 (8.7:1) (Tables 2, 8).
Phacomatoses. In this series of primary pediatric brain
tumors, 39 (4.0%) children had phacomatoses. The
average age of diagnosis was 9.6 years (range, 1–18
yrs). Male to female ratio was 1.5:1. Ten children had
NFI. Five of them had optic gliomas, with histologic
diagnosis of pilocytic astrocytomas in 2 patients. Four
patients had astrocytic tumors including 1 in pons
(pilocytic astrocytoma), 1 in cerebellar vermis (pilo-
cytic astrocytoma), 1 in thalamus (pilocytic astrocy-
toma), and 1 in hypothalamus (astrocytoma). The re-
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Primary Pediatric Brain Tumors/Wong et al. 2161

TABLE 4
Correlation of Serum Tumor Markers (AFP, HCG) and Different Types of Intracranial GCTs with Histologic Diagnosis

Serum AFP ng/mL Serum HCG mIU/mL

No. of
Histologic types patients ND N E ND N E

Germinoma 40 0 38 2 13.3 20.1 3 30 7 44.6 778

59.1 1519
80.8 6555
128
Teratoma, mature 7 2 5 0 0 7 0
Teratoma, immature 9 0 0 9 21.5 116 2 6 1 27.6
26 537
38 552
92.8 9011
93.9
YST, pure 9 0 0 9 1293 2650 1 5 3 28.8 804
1355 5427 187
1590 6109
1876 7304
2177
Mixed GCT 17 1 3 13 21.5 146 3 7 7 10.5 175
24.9 365 30.8 2802
38.0 366 61.4 3312
49.4 1874 156
54.1 2026
59.8 2126
120

AFP: alpha-fetoprotein; HCG: human chorionic gonadotropin; ND: not done; N: normal (within reference range); E: elevated (higher than reference range)

maining 1 patient had a germinoma at the basal years of age. An anaplastic oligodendroglioma with
ganglia.5 Thirteen children had NFII. All of them had tumoral hemorrhage was found 20.5 years after CSI for
bilateral vestibular schwannomas. Cranial meningio- medulloblastoma in a girl aged 9 years. A left frontal
mas were also recorded in 6 patients. Twelve patients pole meningioma was diagnosed 20 years in a girl after
had tuberous sclerosis with subependymal giant cell CSI for medulloblastoma at 5.1 years of age. Atypical
astrcytomas (SEGAs). Ten of them had histologic di- meningioma of the tentorium was found 13.1 years
agnosis. Two patients had neurocutaneous melanosis after local radiation of a residue pilocytic astrocytoma
and histologic proof of intracranial malignant mela- of the posterior temporal region. A glioblastoma mul-
nomas. Basal cell nevus syndrome (Gorlin syndrome) tiforme of pons was diagnosed 8 years after radiation
was observed in 2 children with medulloblastomas therapy for a craniopharyngioma in a girl 4 years old.
(Table 5). Angiosarcoma in soft tissue of occipital region oc-
curred at 2.8 years after radiotherapy for a pineal
Radiation-induced tumors. We observed 7 patients germinoma. These tumors were excluded from the
who developed second tumors after radiation therapy study series of primary brain tumors.
for primary brain tumors. Their primary tumors were
cerebellar medulloblastomas in 4 patients, sellar cra- Locations of tumors
niopharyngioma in 1 patient, pineal germinoma in 1 Including 91 cases of pineal– quadrigeminal tumors,
patient, and posterior temporal residue pilocytic as- there were 575 (58.3%) supratentorial tumors and 405
trocytoma in 1 patient. The durations of time after (41.1%) infratentorial tumors. Another 6 (0.6%) pa-
resection and radiation therapy to diagnosis of second tients had both supra- and infratentorial tumors and
tumors were from 2.8 –21.1 years. These radiation- all of them had traits of neurofibromatosis (Table 6).
induced tumors included basal cell epithelioma of Infratentorial tumors predominated only in the
scalp and meningioma of cerebral convexity devel- groups aged 5, 6, and 8 years. There was almost equal
oped in a girl at 16.3 years after radiation for her distribution of supratentorial and infratentorial tu-
medulloblastoma at 2 years old. Multiple supratento- mors in the age groups of 4 and 18 years. Supratento-
rial meningiomas were found at 21.1 years after cra- rial tumors predominated in all of the other year
niospinal irradiation (CSI) for medulloblastoma at 5.5 groups (Fig. 2). The neuroanatomic distributions of
 10970142, 2005, 10, Downloaded from https://acsjournals.onlinelibrary.wiley.com/doi/10.1002/cncr.21430 by Readcube-Labtiva, Wiley Online Library on [16/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
2162 CANCER November 15, 2005 / Volume 104 / Number 10

TABLE 5 TABLE 6
Phacomatoses and Brain Tumors Distribution of Pediatric Brain Tumors in Taiwan

No. of No. No. No. supra- &

Phacomatoses cases Intracranial tumors supratentorial infratentorial infratentorial
tumors (%) tumors (%) tumors (%)
NFI 10 Optic gliomas 5
pilocytic astrocytoma 2 Shih et al. NDMC, Taipei,
no histologic diagnosis 3a 1977a N ⫽ 126 67 (53.2) 59 (46.8)
Pilocytic astrocytoma 3 Ho et al. NTUH, Taipei,
thalamus, cerebellar vermis, pons 1969–1978b N ⫽ 87 53 (60.9) 34 (39.1)
Astrocytoma 1 Wong et al. Taipei VGH,
hypothalamus 1978–1985 Age ⱕ 15
Germinoma 1 yrsc N ⫽ 171 99 (57.9) 72 (42.1)
Basal ganglia Wong et al. Taipei VGH,
NFII 13 Bilateral vestibular schwannomas 1978–1994 Age ⱕ 15
histologic diagnosis 6 yrsd N ⫽ 501 291 (58.1) 207 (41.3) 3 (0.6)
no histologic diagnosis 7b Wong et al. Taipei VGH,
Meningiomas 6 1975–2004 Age ⱕ 15
histologic diagnosis 6 yrse N ⫽ 935 540 (57.8) 390 (41.7) 5 (0.5)
Tuberous sclerosis 12 Subependymal giant cell Wong et al. Taipei VGH,
astrocytomas 10 1975–2004 Age ⱕ 18
histologic diagnosis 10 yrsf N ⫽ 986 575 (58.3) 405 (41.1) 6 (0.6)
no histologic diagnosis 2c
a
Basal cell nevus syndrome 2 Medulloblastomas 2 If 7 arteriovenous malformations and 4 arachnoid cysts were excluded, the ratio of supratentorial and
Cerebellar vermis 1 infratentorial tumors would be 49.6–50.4%.
b
Cerebellar hemisphere 1 If 22 histologically unverified brain stem gliomas were included, the ratio of supratentorial and
Neurocutaneous melanosis 2 Malignant melanomas 2 infratentorial tumors would be 48.6–51.4%.
c
Data from study of 171 cases of pediatric brain tumors of ⬍ 15 yrs of age after the CT brain scan was
a
Three sellar region tumors in NFI patients without histologic diagnosis were classified as optic used as the diagnostic tool in Taipei VGH.
gliomas. d
Data from study of 501 cases of pediatric brain tumors of ⬍ 15 yrs of age with diagnostic images.
b
Bilateral tumors in internal meatus of 7 NFII patients without histologic diagnosis were classified as e
Data from study of 935 cases of pediatric brain tumors of ⬍ 15 yrs of age with diagnostic images.
vestibular schwannomas. f
Data from study of 986 cases of pediatric brain tumors of ⬍ 18 yrs of age with diagnostic images.
c
Among 13 patients with SEGA, 12 patients presented clinical features of tuberous sclerosis, and 2 of
these SEGAs had no histologic diagnosis.

Types of GCTs in this area were germinomas (52.8%),

immature teratoma (2.8%), mixed GCTs (16.7%), pure
tumors were cerebellum 24.2% (239), cerebral hemi- yolk sac tumors (13.9%), unclassified GCT (2.8%), and
sphere 19.7% (194), sellar-suprasellar–anterior third tumors diagnosed by tumor markers (11.1%). Two
ventricular– hypothalamus region 19.2% (189), brain types of tumors occurred in the hypothalamus, the
stem 12.2% (120), ventricles 8.3% (82), single pineal astrocytic tumors and the hamartomas. For pineal
region tumors 7.5% (74), tumors of both suprasellar tumors, there were 74 patients with tumors in the
and pineal regions 1.0% (10), thalamus 4.7% (46), dura pineal region alone. Another 10 patients had both
1.5% (15), and bilateral vestibular schwannoma 1.3% suprasellar and pineal tumors. The average age at
(13) (Table 7). diagnosis of these tumors was 10.3 years with a male
to female ratio of 5.5:1. Tumors in the pineal or su-
Types of tumors in specific neruoanatomic locations. prasellar–pineal regions were GCTs (79.8%), pine-
Common tumors in cerebral hemisphere were astro- oblastomas (9.5%), astrocytic tumors (2.4%), and pine-
cytic tumors (49.0%), GCTs (13.9%), ependymal tu- ocytoma (1.2%). Common tumors in thalamus region
mors (10.3%), gangliogliomas (7.2%), and oligoden- were astrocytic tumors (71.7%) and PNETs (15.2%).
droglioma (6.2%). In cerebral hemisphere, the Cerebellar tumors were mainly medulloblastomas
majority of GCTs (23/25 patients) originated in the (54.8%), astrocytic tumors (33.5%), and AR/RTs (7.1%).
deep hemisphere—the basal ganglia region. Common In cerebellar vermis, 65.3% tumors were medulloblas-
tumors of sellar–anterior III ventricular– hypothalamic toma. In cerebellar hemisphere, 63.5% were astrocytic
region were craniopharyngiomas (43.4%), astrocytic tumors. For brain stem tumors, among 120 patients of
tumors (21.2%), GCTs (19.1%), pituitary adenomas primary brain stem tumors, only 46 (38.3%) had his-
(5.3%), and hamartomas (4.2%). For gliomas in this tologic diagnosis, and the majority was astrocytic tu-
location, most were optic pathway gliomas, and 3 of mors. These tumors were distributed at the midbrain
37 patients had no histologic confirmation. In supra- (7), pons (94), and the medulla and cervicomedullary
sellar region, 28 of 36 GCTs had histologic diagnosis. junction (18). The distribution of 46 histologically
 10970142, 2005, 10, Downloaded from https://acsjournals.onlinelibrary.wiley.com/doi/10.1002/cncr.21430 by Readcube-Labtiva, Wiley Online Library on [16/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
Primary Pediatric Brain Tumors/Wong et al. 2163

TABLE 7 (48.6%), sellar–anterior third ventricle– hypothalamic

Neuroanatomic Locations of Pediatric Brain Tumors region (26.1%), and basal ganglia and cerebral lobes
(19.6%). All medulloblastomas occurred within cere-
Cerebral hemisphere 19.7%
Lobe 15.2% bellum, and the majority of them in cerebellar vermis
Deep hemisphere 4.5% (86.3%). AT/RTs, which may mimic medulloblasto-
Sellar-anterior III ventricle-hypothalamus 19.2% mas, in cerebellum (71.4%), but they were also distrib-
Hypothalamus 1.7% uted in cerebral hemisphere, ventricle, and pineal–
Thalamus 4.7%
quadrigeminal region. Supratentorial PNETs were
Pineal 7.5%
Suprasellar-pineal 1.0% found mainly in thalamus (58.3%), cerebral hemi-
Cerebellum 24.2% sphere (16.7%), and sellar region (16.7%). All cranio-
Vermis 17.2% pharyngioma in this series were sellar or suprasellar
Hemisphere 6.4% tumors with various extensions and directions of ex-
Brachium 0.2%
tension. Neuronal and mixed neuronal glial tumors
NOSa 0.2%
Brainstem 12.2% were mainly in cerebral hemisphere (66.7%), cerebel-
Midbrain 0.7% lum (14.3%), brain stem (14.3%), and hypothalamus
Pons 9.5% (4.8%). Meninigiomas were tumors arising from dural
Medulla 1.5% matter (64.3%), but there were also tumors in ventricle
Cervicomedulla 0.3%
or cerebral hemisphere. Meningeal sarcomas all orig-
NOSa 0.1%
Ventricle 8.3% inated from dura of anterior temporal region (Table 9).
Extraparenchymatous 1.9%
Bil, vestibular schwannoma 1.3% DISCUSSION
Dura 1.5% In this study, specific tumor data that highlight the
a
character of this series were 1) average age of diagno-
Not otherwise specified in location.
sis ⫽ 7.8 years; 2) male to female ratio of 1.4/1; 3) high
incidence of germ cell tumors (14%); 4) AT/RTs rep-
resenting 2.1% of tumors in this series; 5) Thirty-nine
proved tumors in brainstem were 41 astrocytic tumors (3.9%) patients with traits of phacomatoses; 6) seven
(midbrain 3, pons 23, medullar/cervicomedullary 15), patients who developed second tumors after radio-
3 gangliogliomas (pons 2, medulla 1), 1 ependymo- therapy for primary tumors; 7) supratentorial tumors
blastomas (pons 1), and 1 hemangioblastoms (pons 1). (58.3%) predominated over infratentorial tumors
These histologic findings demonstrated that the ma- (41.8%); 8) neuroanatomical locations of tumors com-
jority of brainstem tumors (89.1% in this series) were mon in cerebellum, cerebral hemisphere, sellar–ante-
actually brainstem astrocytic tumors. Ventricular tu- rior third ventricular– hypothalamic region and brain
mors were mainly posterior fossa ependymomas stem; 9) tumors in pineal and basal ganglia regions
(43.9%), choroids plexus tumors (23.2%), SEGAs mostly germ cell tumors.
(14.6%), GCTs (8.5%), and meningiomas (4.9%). The Increased incidence of brain tumors in children
two major types of tumors of meninges were menin- has been observed and reported in the West. Also
giomas (56.3%) and meningeal sarcomas (31.3%) (Ta- reported is an increased incidence of astrocytoma and
ble 8). medulloblastoma and/or primitive neuroectodermal
tumor (M/PNET).6 –11 In Japan, a study of incidence of
Location distributions of specific primary brain tu- childhood brain tumors in Hokkaido Prefecture
mors. Astrocytic tumors and gangliogliomas distribute showed increased incidence.12 According to the regis-
both supratentorially and infratentorially in brain. ter of the childhood brain tumor (age ⬍ 18 yrs) from
Other types of primary brain tumors in children tend 17 representative hospitals by the Childhood Cancer
to arise in specific neuroanatomic locations. In this Foundation, R.O.C. Taiwan from 1995 to 2002, the
series, the most common distributions of astrocytic annual registered cases varied from 116 to 144 with
tumors were cerebral hemisphere (30.9%), cerebellum annual incidence rate of 1.84, 2.02, 2.03, 2.4, 2.09, 2.25,
(26.1%), brain stem (13.4%), optic appraratus-sellar– 2.03, 2.23/100,000, respectively. There was no evi-
hypothalamus region (13.0%), thalamus (10.7%), and dence of trend increase. This collection represented
SEGAs in the lateral ventricle (4.2%). Ependymal tu- the majority of childhood brain tumors diagnosed in
mors distributed in cerebral hemisphere (35.7%) and Taiwan but was still not a national registration.
in fourth ventricle of the posterior fossa (64.3%). All We compared the reported data of Childhood
oligodendrogliomas and mixed gliomas originated in Brain Tumor Consortium (CBTC), hospital series of
cerebral hemisphere. The 3 most common locations University Hospital of Munster (UHM), the collection
for GCTs were pineal and suprasellar–pineal region of Brain Tumor Registry of Japan (BTRJ), the hospital
 10970142, 2005, 10, Downloaded from https://acsjournals.onlinelibrary.wiley.com/doi/10.1002/cncr.21430 by Readcube-Labtiva, Wiley Online Library on [16/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
2164 CANCER November 15, 2005 / Volume 104 / Number 10

TABLE 8
Types of Tumors in Specific Neuroanatomic Locations

No. of Cases of tissue Cases of clinical

Locations cases diagnosis diagnosis Types and percentage (%) of tumors

Cerebral hemisphere 194 179 7 Ast GCT Epen Gang Olig

49.0 13.9 10.3 7.2 6.2
Sellar-anterior IIIV-Hypothalamus 189 166 17 Cran Ast GCT Pit Ham
43.4 21.2 19.1 5.3 4.2
Pineal & suprasellar-pineal 74 54 15 GCT PB Ast PC
10 8 2 79.8 9.5 2.4 1.2
Thalamus 46 41 0 Ast PNET
71.7 15.2
MDB Ast AT/RT Gang D.I. Gang
Cerebellum 239 236 0 54.8 33.5 7.1 0.8 0.4
Vermis 170 167 0 65.3 22.9 6.5 0.6
Hemisphere 63 63 0 25.4 63.5 6.4 1.6
Brachium 4 4 0 25 50 25
NOS* 2 2 0 100
Ast Gang EB Hem
Brainstem 120 46 0 34.2 2.5 0.8 0.8
Midbrain 7 3 0 42.9
Pons 94 27 0 24.5 2.1 1.0
Medulla/cervicomedulla 18 16 0 83.3 5.6
NOS* 1 0 0
Ventricle 82 79 2 Epen CPT SEGA GCT Men
43.9 23.2 14.6 8.5 4.9
Meninges 15 15 0 Men Sar Chon Ast
53.3 33.3 6.7 6.7

Ast: astrocytic tumor; AT/RT: atypical teratoid/rhabdoid tumor; Chon: chondroma; CPT: choroid plexus tumor; Cran: craniopharyngioma; D.I. Gang: desmoplastic infantile gangliglioma; EB: ependymoblastoma;
Epen: ependymal tumor; Gang: ganglioglioma; GCT: Germ cell tumor; Ham: hamartoma; Hem: hemangioblastoma; MDB: medulloblastoma; Men: meningioma; Olig: oligodendroglial tumor; PB: pineoblastoma; PC:
pineocytoma; Pit: pituitary adenoma; PNET: primitive neuroepithelial tumor; Sar: sarcoma; SEGA: subependymal giant cell astrocytoma.
a
NOS: not otherwise specified in location.

hood brain tumors under the age of 15 years (age 0 –14

FIGURE 2. Chart shows the distribution of supratentorial ( including pineal–
quadrigeminal) versus infratentorial tumors.
series of Seoul National University Hospital (SNUH) of
Korea, and our series.13–16 The CBTC collected data on
3291 children with CNS brain tumors. These patients
received first tumor surgery at one of the CBTC cen-
ters before January 1, 1979 before age 21 years. The
series of UHM consisted of 319 cases of brain tumors
of up to 17 years of age (age 0 –17 yrs) with biopsy
samples. Patient data were collected over a 17-year
period (1984 –2000) by the Institute of Neuropathology
Munster from 7 regional neurosurgical departments.
In BTRJ (1969 –1996), there were 4280 cases of child-
yrs). Among them, 210 patients had no histologic di-
agnosis. In the SNUH series, a series of 677 patients
with age younger than 16 years old, only surgically
treated patients were included. The Taipei VGH series,
a series of 986 patients, age younger than 18 years, and
all of the patients with computed tomography (CT) or
MRI diagnosis were recruited. Among them, 953 pa-
tients were younger than 16 years of age. The three
series in Asia had similar mean age and gender ratio of
patients. The mean age of diagnosis, gender ratio, and
relative frequency of common tumor entities of the
five comparative series are listed in Table 10. Except
the series of CBTC, the other 4 series were predomi-
nant in supratentorial tumors that were 53.3%, 52.8%,
60.0% and 58.3%, respectively. In the recently reported
series of primary pediatric brain tumors in children,
supratentorial tumors predominated over infratento-
rial tumors.14,16,17 By using chi-square test, statistical
comparison of relative incidence of tumors in these
series was performed (data not shown). Except cho-
roid plexus tumor and pineal parenchymal tumor,
significant statistical difference (P ⬍ 0.05) of relative
 10970142, 2005, 10, Downloaded from https://acsjournals.onlinelibrary.wiley.com/doi/10.1002/cncr.21430 by Readcube-Labtiva, Wiley Online Library on [16/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
Primary Pediatric Brain Tumors/Wong et al. 2165

TABLE 9
Location Distribution of Specific Primary Brain Tumors in Children

Neuroanatomic location and percentage of tumors

Cases with Sellar-anterior

No. of histologic Cerebral III V- Pineal and Brain
Types of tumors cases diagnosis Hem hypothalamus suprasellar-pineal Ventricle Cerebellum stem Thalamus Meninges

Ast 307 305 30.9 13.0 0.7 4.2 26.1 13.4 10.75 0.3
Epen 56 56 35.7 64.3a
Olig 12 12 100
M. glioma 7 7 100
GCT 138 105 19.6 26.1 48.6 5.1 0.7
MDB 131 131 100b
AT/RT 21 21 9.5 4.8 4.8 81.0c
PNET 12 12 16.7 16.7 8.3 58.3
Cran 82 81 100
NMNGT 21 21 66.7 9.5 14.3 4.8
Men 14 14 7.1 28.6 64.3
M. Sa 5 5 100

Ast: astrocytic tumor, AT/RT: atypical teratoid/rhabdoid tumor; Cran: crnaiopharyngioma; Epen: ependymal tumor; GCT: germ cell tumor; MDB: medulloblastoma; M. glioma: mixed glioma; NMNGT: neuronal and
mixed neuronal glial tumor, Men: meningioma; M. Sa: meningeal sarcoma; Olig: oligodendroglial tumor; PNET: primitive neuroepithelial tumor.
a th
4 ventricular ependymoma.
b
cerebellar vermis 86.3%, cerebellar hemisphere 12.2%, cerebellar location undetermined 1.5%.
c
cerebellar vermis 11 (52.4%), cerebellar hemisphere 4 (19.1%), brachium cerebellum 2 (9.5%).

TABLE 10
Comparison of Case Collections for Average Age, Gender Ratio, and Relative Frequency of the Most Common Childhood Brain Tumors

CBTC UHM BTRJ SNUH Taipei VGH

Series before 1979 (1984–2000) (1969–1996) (1959–2000) (1975–2004)

No. of patients 3291 319 4080 699 986

Gender ratio M/F 1.2 1.6 1.3 1.4 1.4
Age range in yrs 0–20 0–17 0–14 ⬍ 16 ⬍ 18
Average age 7.8 7.8
Supratentorial tumors 40.9% 53.3% 52.8% 60.0% 58.3%
% % % % Mean age M/F % Mean age M/F
Astrocytic tumor 40.3 47.3 28.2 25.7 7.4 1.0 31.0 7.5 1.0
Medulloblastoma 20.0 16.3 11.9 17.9 7.7 1.7 13.1 7.0 1.9
Germ cell tumor 2.3 2.5 15.3 11.2 10.0 1.7 14.0 10.6 3.8
Craniopharyngioma 6.8 5.6 8.9 12 8.0 1.5 8.3 7.7 1.5
Ependymal tumor 10.9 3.8 4.5 5.0 5.1 1.4 5.7 6.0 1.3
Oligodendroglial tumor 1.3 — 0.9 3.5 8.8 0.8 1.2 7.9 1.4
Choroid plexus tumor 2.0 0.9 1.9 1.3 4.1 3.5 1.9 2.2 1.4
Pineal parenchymal tumor 1.0 0.9 0.9 1.3 8.3 1.3 0.9 5.2 0.8

Case collection of Childhood Brain Tumor Consortium (CTBC), the hospital series of University Hospital of Munster (UHM), Brain Tumor Registry of Japan (BTRJ), Seoul National University Hospital of Korea (SNUH)
and the Taipei VGH series of Taiwan (Taipei-VGH).

incidence existed in astrocytic tumor, medulloblas- countries and geographic regions for astrocytic tumor
toma, germ cell tumor, craniopharyngioma, ependy- and other tumors. We have no explanation for the
moma, and oligodendroglioma. Comparing our series relatively higher incidence of astrocytic tumors in the
with the series of CBTC and the series of UHM indi- two series from Western countries. For the extreme
vidually, significant statistical difference (P ⬍ 0.0125) significant difference of incidence in germ cell tumor
of relative incidence existed in both astrocytic tumors between the two series of Western countries and the 3
and germ cell tumors. There was no such statistical Asian series (P ⬍ 0.0001), genomic difference should
difference between our series and the series of BTRJ be considered.
and the series of SNUH. The results showed geo- In this series, we classified part of those histologic
graphic differences in incidences existed between unverified tumors through their own specific clinical

2166 CANCER November 15, 2005 / Volume 104 / Number 10
features. These tumors were GCTs, specific entities of
CNS tumors of phacomatoses, and hypothalamic
hamartomas. For tumors of neither histologic diagno-
sis nor clinical classification, the majority of these
tumors were brainstem tumors, and most of them
were actually astrocytic tumors. For the present clin-
ical practice, histologic diagnosis is not absolutely
necessary for diagnosis and management of GCTs,
optic pathway gliomas, vestibular schwannomas, SE-
GAs, hypothalamic hamartomas, and pontine gliomas.
Primary intracranial GCTs consist of a variety of
tumor types with different degrees of malignancy. The
reported incidences of GCTs in children were high in
Japan and Korea as compared with incidence in West-
ern countries.14 –16,18 We had a similar high incidence.
For GCTs, over 40% of these tumors were germino-
mas.15,19 It was 54.3% in our series in children. Intra-
cranial GCTs mainly appear in pineal, sellar region,
basal ganglia and ventricules. In the pineal region,
usually ⬎ 70% tumors were GCTs.20,21 In our series, it
was 79.8%. The region of basal ganglia is a significant
location for germ cell tumors. In a reported series of
107 patients with primary germ cell tumors, 16 were
located in the basal ganglia or the thalamic region.22
In our series, among 138 children with GCTs, 25 were
located in the basal ganglia, which constituted of
67.6% of tumors in the basal ganglia.
Serum titers of AFP and beta-hCG in 82 GCTs in
this series correlated with their histologic entities and
an earlier report from this institution.23 Serum AFP
levels were usually higher in pure yolk sac tumors than
in immature teratomas and mixed GCTs. The serum
AFP titers in pure yolk sac tumors were more than
1000 ng/mL in 9 of 9 cases versus 1 of 9 in immature
teratoma and 3 of 13 in mixed GCTs. Normal serum
levels of AFP were observed in 5 of 5 mature terato-
mas. In 2 of 40 germinomas, a low level of elevation of
serum AFP titers was found, but staining for AFP was
negative in these 3 tumors. The serum hCG levels were
high in tumors with syncytiotrophoblastic giant
cells.23 In this series, there was no histologic diagnosis
of choriocarcinomas. Elevated serum titers of beta-
hCG occurred in 7 of 37 germinomas (range, 44.6 –
6555 mIU/mL), 3 of 8 pure yolk sac tumors (range,
28.8 – 804 mIU/mL), and 7 of 14 mixed GCTs (range,
10.5–3312 mIU/mL). Serum beta-hCG titers were nor-
mal in 7 of 7 mature teratomas. Only 1of 7 immature
teratoma had a low elevation of serum beta-hCG. (Ta-
ble 4).
CNS AT/RT was a rare entity of embryonal tu-
mors. The incidence in children in 2 reported series
were 1.3% and 1.6%.14,24 In this series, it was 2.1% of
all primary intracranial tumors in children. These tu-
mors are most common in infants of ⬍ 2 years of age.
The average ages of diagnosis were 17 months and 29
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months in 2 reported series as compare with 4.2 years
in ours.25,26 The diagnosis relied on the specific inter-
est of experienced neuropathologists to separate this
entity of tumor from PNET/MB. The diagnosis criteria
included morphologic features (rhabdoid cells, small
PNET/MB cells, epithelial components, spindle cells),
and immunohistologic profiles.26,27
Phacomatoses represents a diverse group of syn-
dromes that is characterized by retinal lesion, cutane-
ous signatures, and CNS manifestation, especially
tumors. In the current series, the incidence of phaco-
matoses was 3.9% including NF1, NF2, tuberous scle-
rosis, BCNS, and neurocutaneous melanosis. Clinical
diagnosis for brain tumors could be made in some of
these syndromes, such as optic gliomas in NF1, me-
ningiomas and bilateral vestibular schwannomas in
NF2, and SEGAs in tuberous sclerosis. From a series in
Denmark, among 911 children under 15 years of age,
there were 21 patients who had neurofibromatosis.
Among them, 16 had an astrocytoma (12 in the optic
pathway), 2 had a supartentorial meningioma, and 1
had a vestibular schwannoma.17 von Hippel–Lindau
disease with CNS hemangioblastomas usually pre-
sented in adult patients. This entity did not occur in
the current series.
Radiation-induced brain tumor is a potential
complication of radiation therapy for brain tumors in
adult and children. In the current series, radiation-
induced meningiomas, glioblastoma, anaplastic oligo-
dendroglioma, and soft tissue angiosarcoma were ob-
served in 7 patients after radiotherapy for malignant
or benign brain tumors. All of these second tumors
have been reported in the literature. Among them,
radiation-induced gliomas and meningiomas were
most common.28 –31
Conclusion
Epidemiologic studies of pediatric brain tumors are
usually based on population-based surveys of child-
hood cancer registries, childhood brain tumor regis-
tries, or hospital series. Annual incidence rates or age-
specific incidence rates in children can be obtained in
a country or in a geographic region. The pitfalls for
population-based survey of brain tumors in children
through childhood cancer registries or childhood
brain tumor registries were incompleteness of patient
registration that may lead to underestimation. Bias
also exists for not including benign tumors or tumors
without histologic verification for registry.32,33 For
hospital series, incompleteness occurred in series in-
volving only tumors with surgery or with histologic
diagnosis. In the current series from Taipei VGH, we
collected data on 986 patients with primary pediatric
brain tumors in the years of 1975–2004. Among them,
842 (85.4%) patients had histologic diagnosis. This

large hospital series included all primary brain tumors
diagnosed by CT or MRI. Although it is not a study of
population-based brain tumor registry, it may still be
data representative of primary pediatric brain tumors
in Taiwan.
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