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British Journal of Healthcare and Medical Research - Vol. 10, No. 2
Publication Date: April 25, 2023
DOI:10.14738/jbemi.102.14400.
Eneh, C. I., & Egwuonwu, A. C. (2023). Paediatric Pontine Glioma in Southeast Nigeria: A Case Report. British Journal of Healthcare
and Medical Research, Vol - 10(2). 302-310.
Services for Science and Education – United Kingdom
Paediatric Pontine Glioma in Southeast Nigeria: A Case Report
Eneh, C. I.
Department of Paediatrics,
Enugu State University of Science and Technology and
Enugu State University Teaching Hospital (ESUT-TH)
Parklane, P.M.B. 1030, Enugu State, Nigeria
Egwuonwu A. C.
Enugu State University Teaching Hospital (ESUT-TH) Parklane,
P.M.B. 1030, Enugu State, Nigeria
Keywords: Paediatric, pontine glioma, Southeast, Nigeria, poor prognosis.
BACKGROUND
A pontine glioma is a diffuse intrinsic brainstem tumour1,2. Histologically it has a high degree of
tumour anaplasia with necrosis (high grade III-IV). The prognosis for intrinsic brainstem
glioma is extremely poor as the tumour is inoperable, radiotherapy and chemotherapy are not
curative and median survival is less than one year.
INTRODUCTION
Gliomas are tmours formed from glial cells-these form supporting tissues carrying oxygen,
nutrient, and protect the brain from infections3. Brainstem gliomas are among the four most
common types of infratentorial tumours in children. Pontine glioma is a rare tumour that occurs
in the most delicate part of the brain stem-the Pons1 It occurs almost exclusively in children.
Anatomically the pons lies antero-superior to the other structures of the brainstem (midbrain
and medulla oblongata)2. These structures connect the cerebellum to the spinal cord and has
control over important functions in the body- respiratory centre, blood pressure, CN III, VIII, X
chiefly. The falx cerebri separates the brain into two compartments with the brainstem lying in
the infratentorial compartment. Infratentorial tumours form approximately fifty percent of
childhood CNS tumours and are most common in children aged one to ten years.
CASE REPORT
Seven-year-old female C.C presented to our unit with history of progressive unsteadiness of gait
while walking then while standing for one week. This was followed by child becoming
progressively withdrawn. She had fever and dizziness but no visual or speech impairments.
There was no history of head-trauma, drug-use or seizures
Physical examination showed somnolent, afebrile child with signs of cerebellar dysfunction,
otherwise normal CNS findings. Patient later developed features of raised intracranial pressure
for which she was managed accordingly. She had normal fundus, cerebrospinal fluid and skull
x-ray but computerized tomography of the brain showed a diffuse pontine mass with
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Eneh, C. I., & Egwuonwu, A. C. (2023). Paediatric Pontine Glioma in Southeast Nigeria: A Case Report. British Journal of Healthcare and Medical
Research, Vol - 10(2). 302-310.
URL: http://dx.doi.org/10.14738/jbemi.102.14400.
obstructive hydrocephalus of the fourth ventricle (see images). MRI was requested for.
However, the patient died before either MRI and subsequent radiotherapy could be
commenced.
Brain tumours are the second most common childhood tumours in Caucasians but the true
prevalence in sub-Saharan Africa is unknown. This case report is mainly to create awareness of
the existence and prognostic feature of pontine gliomas in Southeast Nigeria of Sub-Saharan
Africa.
CT SCAN OF PATIENT
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Eneh, C. I., & Egwuonwu, A. C. (2023). Paediatric Pontine Glioma in Southeast Nigeria: A Case Report. British Journal of Healthcare and Medical
Research, Vol - 10(2). 302-310.
URL: http://dx.doi.org/10.14738/jbemi.102.14400.
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Eneh, C. I., & Egwuonwu, A. C. (2023). Paediatric Pontine Glioma in Southeast Nigeria: A Case Report. British Journal of Healthcare and Medical
Research, Vol - 10(2). 302-310.
URL: http://dx.doi.org/10.14738/jbemi.102.14400.
LITERATURE REVIEW
Classification: Histologically, brainstem gliomas are classified into: Diffused infiltrative pontine
glioma (DIPG), Focal glioma, exophytic glioma and cervicomedullary glioma4.
Eighty percent of DIPG are high grade and anaplastic, while the other 20% are low grade5.
Aetiology: This is unknown. There is paucity of data on the cause of pontine gliomas. Some
recent evidence points to platelet derived growth factor (PDGF) and its receptors (PDGFR) as
among the major driving forces of tumourigenesis in majority of cases7. Another study
implicated epidermal growth factor receptor (EGFR)- implicated in a majority of high grade
DIPG and about 20% low grade DIPG by immune-histo-chemistry staining techniques8.
Approximately 50% DIPG had p53 gene mutation. Three groups reported loss of a region of 17p
containing the p53 gene in 31%, 57%, and 64% respectively9. Possible risk factors for
brainstem glioma include Neurofibromatosis type1.
Epidemiology: Incidence in Africa is not known. Very little has been published or is known
about Paediatric brain tumours in Nigeria especially the South-Eastern part10 previous reports
being from the southwest zone11-13. According to Central brain tumour registry in US, there
were about 400-500 cases of brainstem glioma per year. DIPG represents 10.7% of primary
central nervous system tumour 14. The common age at diagnosis for DIPG is 5-9yrs, median age
of 6.5yrs. There are no sex predilection15.
Clinical features: Recognised symptoms of pontine glioma include loss of balance and gait
anomalies, aggressiveness or somnolence, excessive sleepiness, behavioural changes, headache
(initially early morning and gets relieved by vomiting), vision and hearing problems as well as
difficulty in learning15.
Diagnosis: is suggested by a good history and physical exam with emphasis on a thorough
neurological exam eliciting some or all the clinical features. Definitive diagnosis is by MRI scan
which shows infiltrative expansion of the pons, typically hypointense on T2 weighted and
hyperintense on T1 weighted. MR spectroscopy helps rule out other differential diagnosis. CT
scan can be used in the absence of MRI. Albright and co-workers16 reported that two CT scan
features including a hypo-dense tumour prior to contrast administration and a tumour
involving the entire brainstem correlated with a significantly reduced survival time.s
In
addition, Stronk and co-workers17 identified four distinct groups of brain stem gliomas based
on CT scan characteristics including group I- iso-dense contrast enhancing with dorsal
exophytic component, group IIa- hypo-dense non-enhancing intrinsic tumours, group IIb- intrinsic tumours with hyper-dense exophytic components and group III- intrinsic cystic
contrast enhancing and group IV-focally intrinsic iso-dense contrast enhancing. Group IV was
reported to have the best prognosis with best response to surgical intervention followed by
group I with group II having the worst prognosis. DIPG shows increased choline /N-acetyl
aspartate and choline /creatinine ratios differentiating it from brainstem encephalitis,
demyelination and other inflammatory processes when MRI and clinical features are
inconclusive18.
Treatment: DIPG lesions are said to be inoperable and not so sensitive to chemotherapy.
Radiotherapy remains treatment of choice and must be instituted as soon as diagnosis is made.
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The dose is between 5400-6000cGy and it is given in daily fractions of 150-200cGy over six
weeks19.
Prognosis: DIPG has a poor prognosis – 0% 5-year survival rate with median overall survival
time of children diagnosed with DIPG being approximately nine months. The 1 and 2-year
survival rates are approximately 30% and less than 10% respectively 20. About 75-80% of
patients show some improvement after radiotherapy. However, DIPG almost always begins to
sprout in form of progression, relapse or recrudescence. Clinical trials report the time between
radiotherapy and progression is 5-8.8 months19. Children under age 3 years and young adults
over 18years and those with Neurofibromatosis type1 may have better prognosis.
DISCUSSION
Our study environment as in most developing countries are fraught with such environmental
hazards as unregulated gas flaring, crude oil and other petroleum product spillage on human,
aquatic and agricultural lives21. These pose risks to development of cancers and affect their
characteristics, presentation and management outcomes. In addition, paucity of personnel and
technology in resource limited settings such as ours did not permit immuno-histochemical or
genetic studies by which our index patient could have been classified4,5,7-9. The patient had no
feature of Neurofibromatosis as a risk factor.
An earlier review of Paediatric brain tumours in another center in the same setting 10 reported
Paediatric gliomas the most common paediatric brain tumors (37%) and the most common
supratentorial tumour. However, brain stem glioma was second to medulloblastoma among the
infratentorial tumours10. Thus, brain stem gliomas as seen in our index patient may be said to
be relatively common compared to other Paediatric brain tumours in our setting. The index
patient’s age of 7 years is consistent with the reported common age at diagnosis (5-9yrs,
median age of 6.5yrs) for DIPG15. These findings support the literature that infratentorial
tumours form approximately fifty percent of childhood CNS tumours with brainstem gliomas
among the four most common types of infratentorial tumours in children.and being most
common in children aged one to ten years. Pontine glioma may be reported in a female as in
this case and equally in a male child within the age range10 since there is no sex predilection15.
Several other investigators collaborate this finding16-18.
Regarding the commonly recognised symptoms of pontine glioma15, it is noteworthy that our
patient presented mainly with dizziness as well as gait anomalies (as did most of the patients
with infratentorial tumours (82%) in the earlier review, followed by vomiting (72%) and
altered consciousness (48%) in their series19. In contrast, our index patient though often
somnolent remained fully conscious and had neither complaints of headache nor vomitting
episodes except for a period of time during admission when she developed raised intracranial
pressure.
For the index patient the diagnosis was via history, physical examination and by CT scan. No
biopsy was performed in our patient. In the absence of the highly specific and diagnostic MRI,
the alternative and acceptable CT scan was carried out and shown in the case report images.
The sudden demise of the child has implications for the bad prognostic type; DIPG20.
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Eneh, C. I., & Egwuonwu, A. C. (2023). Paediatric Pontine Glioma in Southeast Nigeria: A Case Report. British Journal of Healthcare and Medical
Research, Vol - 10(2). 302-310.
URL: http://dx.doi.org/10.14738/jbemi.102.14400.
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