|Year : 2018 | Volume
| Issue : 3 | Page : 186-190
Paediatric brain tumours managed in Enugu, Southeast Nigeria: Review of one centre experience
Chika Anele Ndubuisi, Samuel C Ohaegbulam, Gabriel O Ejembi
Department of Neurosurgery, Memfys Hospital for Neurosurgery, Enugu, Nigeria
|Date of Web Publication||26-Sep-2018|
Chika Anele Ndubuisi
Department of Neurosurgery, Memfys Hospital for Neurosurgery, P. O. Box 2292, Enugu
Source of Support: None, Conflict of Interest: None
Background: Nothing has been published about paediatric brain tumours (PBTs) in Southeast Nigeria. This pioneer study analyses the tumour characteristics and management outcome of PBTs in Enugu, Southeast Nigeria. Methodology: Retrospective longitudinal study utilising information from medical, radiology and histopathology records of all paediatric tumour cases managed at Memfys Hospital for Neurosurgery, Enugu, a major referral centre, from 2006 to 2017. Results: The total number of cases was 54. Peak age group at the diagnosis were 0–5 years (37.0%) and >10 years (40.8%). Frequent presenting symptoms of supratentorial tumours were limb weakness (70%), convulsions (50%) and altered consciousness (48%). Infratentorial tumours presented mostly with gait disturbance (82%), vomiting (72%) and altered consciousness (48%). There were 51 (94.4%) primary and three metastatic tumours. Supratentorial tumours were 28 (51.9%) and infratentorial tumours were 26 (48.1%). Histopathologic types were glioma 20 (37.0%), medulloblastoma 13 (24.1%), craniopharyngioma 11 (20.4%), meningioma 2 (3.7%) and others 8 (14.8%). The most common supratentorial tumours were gliomas 11 (39.3%) and craniopharyngioma 11 (39.3%), and the most common infratentorial tumour was medulloblastoma 13 (50.0%). Gross total resection (GTR) was achieved in 28 (51.9%) and subtotal resection (STR) in 26 (48.1%). Patients with Karnofsky score ≥70% had 16 (65.2%) GTR and patients with ≤60% score had more STR 18 (58.1%). At 1-year post-surgery, 16 (64%) of those with GTR lived normal lives compared with only 4 (20%) of STR. Among GTR group, 7 (28.0%) died compared to the 11 (55.0%) deaths recorded following STR. Overall, 55.6% of patients were at least independent at 1 year. Conclusion: Glioma, medulloblastoma and craniopharyngioma are the most common PBTs. The management outcome is good and affected by extent of tumour resection.
Keywords: Brain tumour, Nigeria, paediatric, Southeast
|How to cite this article:|
Ndubuisi CA, Ohaegbulam SC, Ejembi GO. Paediatric brain tumours managed in Enugu, Southeast Nigeria: Review of one centre experience. Niger Postgrad Med J 2018;25:186-90
|How to cite this URL:|
Ndubuisi CA, Ohaegbulam SC, Ejembi GO. Paediatric brain tumours managed in Enugu, Southeast Nigeria: Review of one centre experience. Niger Postgrad Med J [serial online] 2018 [cited 2019 Feb 20];25:186-90. Available from: http://www.npmj.org/text.asp?2018/25/3/186/242202
| Introduction|| |
Paediatric brain tumour (PBT) accounts for 20%–25% of paediatric tumours worldwide and a significant cause of childhood morbidity and mortality., Very little has been published or is known about this very important disease in Nigeria. These tumours were in the past considered uncommon among the Negroes, and the frequency of PBT from the published series supports this view., A previous report from Morocco had a significant volume of patients managed for PBT although this was a nationwide based study. This frequency of diagnosed PBTs is expected to improve with the improvement in the number of neurosurgery experts, neurodiagnostic services and health-seeking behaviour of those living in the developing countries. In the study environment, there are peculiar factors like financial constraints that still make early access to health-care services challenging. There are also some environmental hazards of undetermined risk level such as unregulated gas flaring, crude oil and other petroleum product spillage on human, aquatic and agricultural lives. These factors might affect the tumour characteristics, pattern of presentation and outcome of cases managed. This study analysed the tumour characteristics, pattern of clinical presentation and management outcome of PBTs in Enugu, Southeast Nigeria. This is the first report on PBT from this large part of Nigeria as previous reports from the country were based on the experience from the southwest zone.,,
| Methodology|| |
A retrospective longitudinal study carried out at Memfys Hospital for Neurosurgery, Enugu, from 2006 to 2017. The study centre is a major neurosurgical referral tertiary hospital serving a large catchment population of the Southeast Nigeria and neighbouring states. Information was obtained from patients' folders and histopathology records. Patients included in this study were aged from 1 month to <18 years old and with conclusive histology confirming brain tumour after surgery. All patients had a post-contrast-enhanced brain magnetic resonance imaging (MRI) investigation. Brain computed tomography (CT) scan was accepted for the diagnosis when MRI investigation could not be carried out. The spine was also screened for drop metastasis when indicated as in posterior fossa and intraventricular tumours. Demography, imaging finding, clinical history, surgery details, anatomy location, the tumour histology grade and clinical outcome were analysed. Patients were followed up for a minimum of 1 year. Anticonvulsants and steroids were administered to all patients before surgery. Anticonvulsants were continued for minimum of 6 months before being tailed off while low-dose steroids were generally continued until edema improves or after the completion of radiotherapy and tailed off. In the immediate post-operative period, patients were managed in the intensive care unit and later transferred to the ward for optimisation. At discharge, cases that needed rehabilitation were stepped down to secondary facilities. All patients with malignant lesions were referred to oncologist for adjuvant therapy while those with impaired hormone function were referred to the paediatric endocrinologist for long-term management. Radiotherapy was administered where appropriate for patients who were at least 5 years old. The world health organization classification was used for the tumour grading. All patients had post-operative neuroimaging investigation before discharge from the hospital. The extent of tumour resection was assessed with both the intraoperative microscope aided decision by the surgeon and patients post-operative brain CT scan. The clinic follow-up protocol was at 4 to 6 weeks, 3 months, 6 months and subsequently yearly. Neuroimaging follow-up investigations were obtained when clinically indicated. Functional status was assessed using the Karnofsky Performance Score (Ks). The pre-operative Ks was benchmarked at the time of tumour excision not necessarily the pre-admission Ks. Patients' outcome at 1 year was analysed against the extent of tumour resection. Data were analysed using descriptive statistics.
| Results|| |
The total number of cases was 54 representing 13% of all brain tumours managed in the centre. Averages of three cases were managed annually between the years 2007 and 2013 but this increased to eight cases per year between the year 2014 and 2017. Males were 26 (48.1%) and females 28 (51.9%). The mean age at presentation was 9 years. Peak age group at the diagnosis were 0–5 years (37.0%) and >a10 years (40.8%) [Table 1].
The most frequent presenting symptoms of supratentorial tumours were limb weakness (70%), convulsions (50%) and altered consciousness (48%). Infratentorial tumours presented mostly with gait disturbance (82%), vomiting (72%) and altered consciousness (48%). Primary tumours were 51 (94.4%) and metastatic tumours were 3. Supratentorial tumours were 28 (51.9%) and infratentorial tumours were 26 (48.1%) [Table 2].
The histologic types were glioma 20 (37.0%), medulloblastoma 13 (24.1%), craniopharyngioma 11 (20.4%) and meningioma 2 (3.7%). Other tumours include seminoma, pineoblastoma, neuroblastoma, neurofibroma, rhabdomyosarcoma and pituitary adenoma accounted for 8 (14.8%)[Table 3].
The most common supratentorial tumours were gliomas 11 (39.3%) and craniopharyngioma 11 (39.3%), whereas the most common infratentorial tumour was medulloblastoma 13 (50.0%) followed by glioma 10 (38.5) [Table 4].
|Table 4: Histology in different compartments analysed against age and sex|
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Fifty-seven per cent of patients had surgery at Karnofsky score of 60% or below, and the majority of these patients had subtotal resection (STR) of tumour. Gross total resection (GTR) was achieved in 28 (51.9%), STR and biopsy in 26 (48.1%). Patients with Karnofsky score ≥70% had more number of GTR 16 (65.2%), while those with scores ≤60% had more STR 18 (58.1%) [Table 5].
|Table 5: Karnofsky performance score at the time of tumour excision analysed against extent of resection|
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At 1-year post-surgery, 16 (64%) of those that had GTR lived normal lives while only 4 (20%) of STR lived normal life. Among the GTR group, only 7 (28.0%) died compared to the 11 (55.0%) deaths recorded following STR. Overall, 55.6% of patients were at least independent at 1 year [Table 6].
| Discussion|| |
This study is a retrospective review of the pattern of PBTs managed at a neurosurgical hospital in Enugu, Southeast Nigeria. The frequency of paediatric tumours appears to be low, accounting for about 13% of all brain tumours. However, in the past 3 years of this study, the average frequency of cases managed per year has almost tripled.
Although awareness seems to be increasing in the last few years of this study, significant proportion of cases still presented with advanced disease. As much as 48% of the patients had alteration of the level of consciousness at presentation usually from obstructive hydrocephalus and >70% of the cases presented with a definite focal deficit. These delays in presentation may be linked to the non-existence of universal health insurance which makes health care unaffordable to most families in Nigeria. This might also partly explain the low frequency of PBTs. The overall frequency of PBT of about five cases per year in this study still seems low, and it is hoped that the increasing awareness of neurological diseases improved availability of neuroscientists, and substantial improvements in diagnostic facilities such as CT and MRI in the catchment area will improve this statistics in the coming years. The slightly higher frequency of about 7 per year recorded at Ibadan was a pathology department-based study that included autopsy cases. These low-frequency rates appear to support the view that these tumours are uncommon among the black population. However, judging by the increased diagnosis in the past 3 years, the low overall low frequency of PBT in the region may also be a reflection of the relatively poor referral chain and primary health-care services in the country. A previous report from Morocco had a significant volume of patients managed for PBT although this was a nationwide based study.
Glioma, medulloblastoma and craniopharyngioma were the most common tumours managed in this study. However, medulloblastoma was the most common infratentorial tumour, while craniopharyngioma and glioma were the most common supratentorial tumours. Based on the age analysis, the findings in this study suggest that medulloblastoma followed by glioma is the most common in the first decade of life. However, in the second decade of life, the patients are more likely to have supratentorial tumour with craniopharyngioma followed by glioma as the predominant lesions.
In this study, there was relatively an even distribution of supratentorial and infratentorial tumours. This is similar to the findings from the southwest Nigeria and some parts of Europe.,, However, many studies reported higher proportions of supratentorial tumours while few others reported more infratentorial tumours.,,
Gliomas contributed 37% of the entire PBTs. The preponderance of gliomas is consistent with the findings in other studies based on 0–20 years of age., However, it is reported from some studies that analysed only the age group of 0–14 years that medulloblastomas predominate (Morocco). Racial differences have been reported in the distribution of histological types of PBTs and in their survival rates.
Metastatic tumours were very rare in this study despite the relative prevalence of systemic malignancies managed in this age group. There were three cases of metastatic brain tumours arising from two cases of neuroblastoma and one rhabdomyosarcoma, and all were in the under-five age group and with the poor outcome. This finding is not peculiar to the paediatric age group in the study environment as previous clinical studies carried out among the adults had a similar trend of predominant primary brain tumours. Another study from southwest Nigeria also recorded no cases of metastases of 77 cases of PBTs. Although craniopharyngiomas constituted about 7.5% of all intracranial tumours managed in the hospital, it represents as high as 19.6% of the PBT managed, a relatively high proportion when compared to the findings from Ibadan southwest Nigeria (11.7%) and other countries such as India (10.2%).,
There were more females (51.1%) than males (48.9%) in this study. The common knowledge is that PBTs usually affect more males than females., Although the latest census figure from the study population recorded more females, this increasing number of females diagnosed and managed in this study as compared to other previous study from Nigeria may be an indication of the improvement of sociocultural gender attitude of the society towards the girl child.
The pattern of presentation from this study revealed a significant delay by the patients in accessing neurosurgery care. Although there was significant dearth of neurosurgeons in the study environment, there is still a strong need to channel effort towards grassroots health awareness education for the population and further strengthening of the patient's referral chain. All the patients presented with significant symptoms. About 50% of cases presented in coma and associated hydrocephalus suggesting an advanced disease. Interestingly too, the seizure was a prominent symptom. There is therefore the need to emphasize early neuroimaging investigations for paediatric patients presenting with seizures or central nervous system symptoms to exclude sinister lesions like brain tumours.
The late presentation of patients has adverse effect on the outcome. GTR of tumour was achieved in only 51.9% of cases resulting in the better outcome when compared to those in whom only STR could be achieved. Adjuvant treatment is still not well developed, and we prefer to avoid radiotherapy in these children since the quality of service available is more hazardous than the residual tumor.
A major challenge in the management of these cases is that majority presented with a poor pre-operative Karnofsky Performance Score and this affects the extent of resection (EOR). EOR is also an indicator of long-term outcome as this study has revealed. The overall outcome of this study at 1 year was fair considering the poor pre-operative Karnofsky score. The 1-year mortality rate was 40% with more proportion of deaths among those with poor Karnofsky score and incomplete resections of tumour. However, 64% of cases with complete tumour excision were normal at 1 year compared with just 20% in those with incomplete excision of tumour, suggesting that early access to care when the conditions are favourable for complete tumour excision will improve outcome.
| Conclusion|| |
Glioma is the most common PBT, followed by medulloblastoma and craniopharyngioma in Enugu, Nigeria. Glioma and craniopharyngioma are the most common supratentorial tumours while medulloblastoma is the most common infratentorial tumour. There is no sex difference in the frequency of tumours managed. Most patients present late and this affects the extent of tumour resection. The management outcome is affected by the extent of tumour resection.
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Conflicts of interest
There are no conflicts of interest.
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[Table 1], [Table 2], [Table 3], [Table 4], [Table 5], [Table 6]