Gac Med Mex. 2016 Mar-Apr;152(2):158-62.
[Clinical presentation of supratentorial and infratentorial intracranial tumors in pediatric patients].
[Article in Spanish]
Author information
- 1
- Departamento de Pediatría, UMAE No. 25, Monterrey, N.L., México.
Abstract
BACKGROUND:
Brain tumors are the second most common malignancy in childhood and they are also the most common solid tumors in children and the most frequent cause of morbidity and mortality associated with cancer in this age.
OBJECTIVE:
To determine the clinical presentation of supratentorial and infratentorial intracranial tumors in pediatric patients.
MATERIAL AND METHODS:
An observational, retrospective, cross-sectional comparative study in which records the clinical manifestations of 51 pediatric patients diagnosed with intracranial tumor, according with its location, supra- or infratentorial.
RESULTS:
51 patients were analyzed. Tumor location was infratentorial in 32 patients (62.7%) and supratentorial in 19 (37.3%). Cerebellar syndrome occurred in 21 patients (65.6 %) with infratentorial tumor compared with six (31.5 %) with supratentorial tumor (p = 0.04; OR: 3.2; 95% CI: 1.1-12.8). Nystagmus was significantly more common in patients with infratentorial tumors (p = 0.029). Endocrine manifestations were present in four patients (21%) with supratentorial tumor and none of the infratentorial group (p = 0.03).
CONCLUSIONS:
Cerebellar syndrome was statistically significantly more common in patients with infratentorial when compared with patients with supratentorial. Nystagmus is one of the most common visual impairments in patients with infratentorial tumor. Endocrine disorders are significantly more frequent in patients with supratentorial tumor. The interval between onset of symptoms and diagnosis of intracranial tumor is significantly longer in children with supratentorial tumor because the symptoms are more insidious in onset and are mistaken for other benign conditions.
- PMID:
- 27160613
138
Clinical presentation of supratentorial and infratentorial
intracranial tumors in pediatric patients
Luz María Sánchez-Sánchez*, Javier Vázquez-Moreno, José Antonio Heredia-Delgado
and Ricardo Sevilla-Castillo
Department of Pediatrics, UMAE No. 25, Monterrey, N.L., Mexico
GACETA MÉDICA DE MÉXICO ORIGINAL ARTICLE
Correspondence:
*Luz María Sánchez-Sánchez
Departamento de Pediatría
UMAE No. 25
Salamanca, 5907
Col. Cumbres Santa Clara
C.P. 64346, Monterrey, N.L., México
E-mail: luzsanchez68@hotmail.com Date of modified version reception: 22-11-2014
Date of acceptance: 09-02-2015
Introduction
Intracranial tumors are the second most common
neoplasm in childhood, and their occurrence and potential causes have been the subject of debate. They
comprise between 16.6 and 21% of all malignancies in
children. In addition, they are the most common solid
PERMANYER
www.permanyer.com
Contents available at PubMed
www.anmm.org.mx Gac Med Mex. 2016;152:138-42
Abstract
Background: Brain tumors are the second most common malignancy in childhood and they are also the most common
solid tumors in children and the most frequent cause of morbidity and mortality associated with cancer in this age. Objective:
To determine the clinical presentation of supratentorial and infratentorial intracranial tumors in pediatric patients. Material and
Methods: An observational, retrospective, cross-sectional comparative study in which records the clinical manifestations of
51 pediatric patients diagnosed with intracranial tumor, according with its location, supra- or infratentorial. Results: 51 patients
were analyzed. Tumor location was infratentorial in 32 patients (62.7%) and supratentorial in 19 (37.3%). Cerebellar syndrome
occurred in 21 patients (65.6 %) with infratentorial tumor compared with six (31.5 %) with supratentorial tumor (p = 0.04; OR:
3.2; 95% CI: 1.1-12.8). Nystagmus was significantly more common in patients with infratentorial tumors (p = 0.029). Endocrine
manifestations were present in four patients (21%) with supratentorial tumor and none of the infratentorial group (p = 0.03).
Conclusions: Cerebellar syndrome was statistically significantly more common in patients with infratentorial when compared
with patients with supratentorial. Nystagmus is one of the most common visual impairments in patients with infratentorial
tumor. Endocrine disorders are significantly more frequent in patients with supratentorial tumor. The interval between onset of
symptoms and diagnosis of intracranial tumor is significantly longer in children with supratentorial tumor because the symptoms
are more insidious in onset and are mistaken for other benign conditions. (Gac Med Mex. 2016;152:138-42)
Corresponding author: Luz María Sánchez-Sánchez, luzsanchez68@hotmail.com
KEY WORDS: Tumor. Intracranial. Supratentorial. Infratentorial.
tumors at pediatric age and the most frequent cause
of cancer-related morbidity and mortality in this group of
patients1.3.
Intracranial tumors location differs considerably in percentage according to the studied population: infratentorial (IT) tumors range from 21 to 67%; supratentorial (ST)
tumors, from 30 to 64%, and those invading both spaces, from 2 to 15%, according to conducted studies4,5.
L.M. Sánchez-Sánchez, et al.: Clinic supratentorial and infratentorial intracranial tumors
139
In children, most intracranial tumors are of unknown
origin. Some risk factors are the following: being firstborn, having an adolescent or aged mother, high
weight for gestational age, dystocic deliveries, prior
abortions, in general, without most these factors being
statistically significant6-7.
In a study conducted with 451 patients with intracranial-origin primary tumors, presentation characteristics
were the following: intracranial hypertension (ICH), with
symptoms such as vomiting, headache, macrocephaly
and altered consciousness, which occurred in up to
49.3% ST and 83% IT tumors. Visual disturbances,
clinically apparent by decreased visual acuity (VA),
ptosis, ophthalmoplegia, exophthalmos and Parinaud
syndrome, have been reported in up to 30.9% of ST
and 24.9% of IT tumors. Ataxia is more commonly reported in IT that ST tumors (33 vs. 2.4%). Seizures are
usually more common in ST than in IT tumors (26.6 vs.
10.4%). Cranial nerves involvement occurs in IT-origin
tumors. Other manifestations, such as endocrine disturbances, have been found in similar percentage on
both locations: 5.3 (ST) versus 3.6% (IT)8.
The treatment of intracranial tumors depends on the
size and type of tumor, as well as on the child’s general health status. The goal of treatment is total resolution of the tumor, symptom cessation and function
improvement. Surgical intervention is necessary in
most tumors and some can be completely removed.
Chemotherapy and radiotherapy can be used for certain tumors9,10.
In this study, a thesis on intracranial tumors and their
clinical manifestations is used with the purpose to obtain useful and applicable information to establish clinical diagnosis of patients affected by symptoms consistent with neurological involvement that drive to the
suspicion of ST or IT tumors-related intracranial involvement, on any of their initial presentations, in order
to help accelerating opportune diagnosis and treatment.
Material and methods
A retrospective, cross-sectional, comparative, observational study was carried out at the UMAE No. 25 of the
Mexican Institute of Social Security, in Monterrey (Nuevo
León, Mexico). Pediatric patients of 0-15 years of age,
diagnosed with ST or IT intracranial tumors at the UMAE
25 of Monterrey during the period comprised between
January 2012 and October 2014 were studied.
Pediatric patients aged 0-15 years, with a diagnosis
of supra- or infratentorial tumor, diagnosed at the
UMAE 25 of Monterrey within the period encompassed
from January 2013 and June 2014 were included; patients with intracranial tumors whose location was not
well defined as being supra- or infratentorial were excluded. Those patients whose medical record was lost
o did not contain the medical history or initial admission
note were eliminated.
Results
Fifty one pediatric patients, diagnosed with endocranial tumors, with a mean age of 7.8 ± 3.7 years were
included; there were 23 patients (45%) of the female
and 28 (55%) of the male gender. Tumor location was
IT in 32 patients (62.7%) and ST in 19 (37.3%).
For IT tumors, histological varieties with confirmed
pathological report were the following: brain stem glioma 11 (34.3%), astrocytoma (7 [21.8%]), medulloblastoma (6 [18.7%]), ependymoma (4 [12.5%]) and
other 4 (12.5%). Histological varieties in patients with
IT tumors were: astrocytoma (5 [26%]), germinoma (3
[15.7%]), thalamic glioma (2 [10.5%]), craniopharyngioma (1 [5.2%]), teratoid rhabdoid tumor (1 [5.2%]) and
others (6 [31.5%]).
The patients were divided to be studied according
to tumor location. The age of the patients with IT tumors
was 7.3 ± 3.2 years, and in patients with ST tumors,
8.7 ± 4.3 (p = 0.152). Of these, 19 patients (59.4%)
with IT tumors and 9 (47.4%) with ST tumors were from
the male sex.
The time elapsed between the onset of symptoms and
the intracranial tumor diagnosis was 2.7 ± 2.7 months
for patients with IT tumors and 22.7 ± 7.6 months for
those with ST tumors (p = 0.00).
Headache occurred in 22 patients (68.7%) with IT
tumors and in 8 (42.1%) with ST tumors (p = 0.06). The
most common type of headache was holocranial in
both groups, with percentages of 75 and 87%, respectively. Cerebellar syndrome was characterized by ataxia, dysmetria, dysdiadochokinesia or all these symptoms, and occurred in 21 patients (65.6%) with IT
tumors and 6 (31.5%) with ST tumors (p = 0.04; odds
ratio [OR]: 3.2; 95% confidence interval [CI]: 1.1-12.8).
Seizures occurred in 3 patients (9.3%) with IT tumors
and 4 (21%) with ST tumors (p = 0.245). Partial seizures were more common in patients with ST tumors,
but there was no statistically significant difference. Visual disturbances were present in 11 patients (34.3%)
with IT tumors and 6 (31.5%) with ST tumors (p =
0.839). However, nystagmus was significantly predominant in the group with IT tumors (p = 0.029). Cranial
Gaceta Médica de México. 2016;152
140
Table 1. Clinical characteristics of 51 pediatric patients with intracranial tumors, classified according to their location, IT or
ST*
Total (n = 51) IT (n = 32) ST (n = 19) p
Age (years) 7.8 ± 3.7 7.3 ± 3.2 8.7 ± 4.3 0.152
Sex
Female 23 (45%) 13 (40.6%) 10 (52.6%) 0.400
Male 28 (54.9%) 19 (59.4%) 09 (47.4%)
Headache 30 (58.5%) 22 (68.7%) 8 (42.1%) 0.400
Holocranial 23 (76%) 16 (72.7%) 7 (87.5%)
Localized 7 (23.4%) 6 (27.3%) 1 (12.5%)
Cerebellar syndrome 27 (52.9%) 21 (65.6%) 6 (31.5%) 0.049
Ataxia 12 (44.4%) 9 (42.8%) 3 (50%)
D+D 3 (11.2%) 2 (9.5%) 1 (16.6%)
All 12 (44.4%) 10 (47.7%) 2 (33.4%)
Seizures 7 (13.7%) 3 (9.3%) 4 (21%) 0.245
Partial 5 (71.4%) 1 (33.3%) 4 (100%)
Generalized 2 (28.6%) 2 (66.7%) 0
Visual disturbances 17 (33.3%) 11 (34.3%) 6 (31.5%) 0.839
Nystagmus 6 (35.3%) 6 (54.5%) 0 0.029
Strabismus 2 (11.7%) 2 (18.2%) 0
Diplopia 5 (29.4%) 2 (18.2%) 3 (50%)
Amaurosis 1 (5.9%) 1 (9.1%) 0
Decreased VA 3 (17.6%) 0 3 (50%)
CNP 16 (31.3%) 12 (37.5%) 4 (21%) 0.225
Endocrine 4 (7.8%) 0 4 (21%) 0.030
ICH 36 (70.5%) 25 (78.1%) 11 (57.8%) 0.194
Vomiting 28 (77.7%) 21 (84%) 7 (63.6%)
Somnolence 6 (16.6%) 3 (12%) 3 (27.2%)
Papilledema 2 (5.5%) 1 (4%) 1 (9.2%)
Hydrocephalus 31 (60.7%) 22 (68.7%) 9 (47.4%) 0.134
TSD 2.5 ± 9.9 2.7 ± 2.7 22.7 ± 7.6 0.000
DEC 1 9 (17.6%) 7 (21.8%) 2 (10.5%) 0.300
*Values presented as mean ± standard deviation o absolute frequency (percentage).
D+D: dysmetria and dysdiadochokinesia; CNP: cranial nerve palsy; TSD: time from symptoms onset to diagnosis; DEC 1: decease during the first year after diagnosis
nerve palsies occurred in 12 patients (37.5%) with IT
tumors and 4 (21%) with ST tumors (p = 0.225). In
patients with IT tumors, cranial nerves VI and VII palsy
was predominant (66%), although no statistically significant different was found when compared with those
with ST tumors. Endocrine anomalies, characterized by
hypothyroidism, hypocortisolism, and anti-diuretic hormone and growth hormone deficit, occurred in 4 patients (21%) with ST tumors, but in none with IT tumors
(p = 0.03). Endocranial hypertension manifested mainly as vomiting, somnolence and papilledema in 25
patients (78.1%) with IT tumors and 11 (57.8%) with ST
tumors (p = 0.194). Hydrocephalus was detected in 22
patients (68.7%) with IT tumors and 9 (47.4%) with ST
tumors (p = 0.134). Only one of the 51 patients showed
an important failure to thrive, resulting from growth
hormone deficit, in the group of ST tumors. Other less
common manifestations were: monoparesis, which
progressed to hemiparesis in 8 patients (25%) with IT
tumors and 4 (21%) with ST tumors, and neck pain in
4 patients (12.5%) with IT tumors and 1 (5.2%) with a
ST tumor.
Hydrocephalus was documented in 22 patients
(68.7%) with IT tumors and 9 (47.4%) with ST tumors.
The number of patients who were deceased within
the first year after diagnosis was 7 (21.8%) in the group
with IT tumors versus 2 (10.5%) in the group of ST
tumors (p = 0.300) (Table 1).
L.M. Sánchez-Sánchez, et al.: Clinic supratentorial and infratentorial intracranial tumors
141
Discussion
Intracranial tumors are the second most common
neoplasm in childhood; they account for 16.6 to 21%
of all malignant neoplasms in children. In addition, they
are the most common solid tumors at pediatric age and
the most frequent cause of cancer-related morbidity
and mortality in this group of patients11,12.
Intracranial tumors location differs considerably according to the studied population: IT tumors account
for 21 to 67%; ST tumors for 30 to 64%, and those
invading both spaces, for 2 to 15%, according to several studies13,14. In this study, 62.7% of examined patients had IT tumors and 37.3%, ST tumors, very similar to reports in previous studies.
Lacour et al. conducted a study in France with 8,473
children included in the national registry of children with
cancer, with data from 2000 to 2004, where children with
malignant hematological disorders and solid tumors
were evaluated, and found coincidence with the second
most common type of tumors being those of the central
nervous system, regardless of the children’s age. The
most common histological variant in this study was astrocytoma (37.6%), followed by medulloblastoma (15%)
and gliomas of different histological lineages (13.6%)15.
In this case series, astrocytomas were found in 23.5%
of patients, medulloblastomas in 11.7%, and gliomas of
different lineages in 25.4%, which is consistent with
reports of the aforementioned authors, with the exception that there were a lower percentage of astrocytomas.
With regard to gender, no factor associated with the
presence of intracranial tumors at any specific location,
or risk factor for the development of the tumor have
been considered6,7. In this study, there was no clear
predominance of any gender, which is consistent with
reports by other authors.
In a study conducted in 451 patients with intracranial-origin primary tumors, presentation characteristics
were the following; ICH, manifesting as vomiting, headache, macrocephaly and altered consciousness, which
occurred in up to 49.3% of ST and 83% of IT tumors5.
In our study, similar percentages to those previously
reported were found, but with no statistically significant
differences between both locations.
Visual disturbances, with symptoms such as decreased VA, ptosis, ophthalmoplegia, exophthalmos
and Parinaud syndrome, have been reported in up to
30.9% of ST and 24.9% of IT tumors8,16-18. In this study,
visual disturbances were found to occur in a third part
of patients with IT or ST intracranial tumors, just as
described in other studies.
Ataxia is more commonly reported in IT than in ST
tumors (33 vs. 2.4%). In this case series, ataxia occurred in 28.1% of children with IT, consistent with
findings reported by El-Gaidi8, but it also occurred in
15.7% of patients with ST, which is a much higher
figure than that reported by El-Gaidi.
Seizures are usually more frequent in ST than IT tumors (26.6 vs. 10.4%). In our study, no statistically
significant difference was found; however, the percentages found in this case series are similar to those reported by other authors.
Cranial nerves involvement is usually more common
in those patients with IT-located tumors. The study we
carried out is consistent with these findings, but with
no statistically significant differences.
Other manifestations, such as endocrine abnormalities, have been found at similar percentage for both
locations: 5.3 (ST) versus 3.6% (IT)19,20. Conversely,
in this case series we only found endocrine anomalies
in patients with ST-located tumors, but in none with IT
tumors, with a statistically significant difference.
Ansell et al., in a comparative study conducted between patients with intracranial tumors and patients
with symptoms but without an intracranial tumor diagnosis, found an up to 60-month diagnostic delay, due
to the atypical, heterogeneous and insidious presentation of some tumors19. In this case series, we found that
patients with ST tumors had more prolonged evolution,
and the time elapsed since the onset of symptoms and
definitive diagnosis was longer than in those with IT
location, although no statistically significant difference
was found.
Worldwide, the mortality rate due to malignant tumors of the brain is 2.8 for each 100-thousand in males
and 2.0 for each 100-thousand in females. Five-year
relative survival is quite varied, according to ethnicities
and races: on average, in Caucasians it is 33.5%, a
figure that discretely increases to up to 37% for African-Americans. In children, the 5-year relative survival
rate is considered to be lower, especially in the case
of the glioblastoma multiforme and ependymoma histological varieties20-22. In this study, mortality was
17.6% during the first year after diagnosis, in both locations, with no statistically significant differences.
Conclusions
Cerebellar syndrome is more frequently observed
in patients with IT tumors, with a statistically significant difference when compared with patients with ST
tumors.
Gaceta Médica de México. 2016;152
142
Nystagmus is one of the most common visual disturbances in patients with IT tumors.
Endocrine alterations are significantly more frequent
in patients with ST tumors.
The time elapsed between the onset of symptoms and
the intracranial tumor diagnosis is significantly longer
in children with ST tumors, since symptoms have a
more insidious onset and are confused with other
benign conditions.
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