Research

Epidemiological evaluation of Intracranial Ependymoma

¹Ravi Shankar Prasad, ¹Kulwant Singh, ¹Vivek Sharma

• ¹Department of Neurosurgery, Institute of Medical Sciences Varanasi-221005, India

• Submitted:March 2, 2013
• Accepted:March 23, 2013
• Published:March 25, 2013

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Introduction

Ependymomas are infrequently seen tumours that have been defined as neoplasms arising from ependymal cells lining the ventricles and the central canal of the spinal cord.

Material and method

This is a retrospective study conducted at Department of Neurosurgery, Sir Sunderlal Hospital, Banaras Hindu University, Varanasi from August 2007 to December 2012. The diagnosis was made with CT scan and/or MRI brain and confirmed histologically.

Results

Ependymomas constitutes 5.6% of total intracranial tumours. Mean age of all patients was 17.63 years, Fifty eight patients had headache at presentation. Nausea, vomiting, papilloedema and cognitive impairment were present in majority of patients. This can occupy supratentorial or infratentorial compartment of brain.

Conclusion

Intracranial ependymoma constitute a significant proportion among brain tumours so high index of suspicion is required for early detection and better management.

Introduction

Ependymomas are infrequently seen tumours that have been defined as neoplasms arising from ependymal cells lining the ventricles and the central canal of the spinal cord. The four most prevalent locations are supra and infratentorial, spinal and conus-cauda filum. It is the third most common infratentorial tumour of childhood, but its frequency is considerably less than that of cerebellar astrocytomas and medulloblastomas [1, 2]. They constitute 2 to 9% of all intracranial tumours and upto 12% of pediatric brain tumours [3, 4, 5, 6, 7]. At least half of ependymomas present in the first two decades of life [8].

Typical cases in children and adults differ considerably. For example, most childhood ependymomas are intracranial, whereas most adult tumours are spinal. About 65% of ependymomas occur in posterior fossa; these malignancies are found more often in children, and most arise from the floor of fourth ventricle. The remaining 35% are found equally distributed between the spinal cord and higher in the brain.

Material and Method

This is a retrospective study conducted at Department of Neurosurgery, Sir Sunderlal Hospital, Banaras Hindu University, Varanasi from August 2007 to December 2012. The diagnosis was made with CT scan and/or MRI brain and confirmed histologically .Patient’s age/ sex, presenting symptoms and signs and tumour location were recorded for analysis. Supratentorial tumours were classified as a) Intraparenchymal tumours b)Tumours arising in lateral ventricles c) Tumours of the third ventricles. Infratentorial tumours were seen for a) Invasion of floor of IVth ventricle b) Lateral extension into foramen of Luschka.

Result

Total number of patients of tumours was 1125 out of which 696 were supratentorial tumours and 429 were infratentorial. Out of these cases, 81 patients were diagnosed as ependymoma based on CT scan or MRI but only 63 patients were histologically proved to be cases of ependymoma. This constitutes 5.6% of total intracranial tumours. Among the remaining 18 cases, 3 turned up as subependymoma, 5 as medulloblastoma, 3 as metastasis, 4 as astrocytomas and 3 as oligodendrogliomas and all these cases were excluded from the study. We considered only 63 histologically proven cases of ependymoma.

Mean age of all patients was 17.63 years, minimum age of presentation was 2 years and maximum age was 60 years. Patients were mostly children and less than 30 years age. 13 patients were less than 5 years of age. About 90% patients were less than 30 years age. (Table 1) shows the age distribution.

Table 1: Age distribution

Age group (years)	No. of cases (n=63)	Percentage
0-5	13	20.63
5-10	12	19.04
10-15	6	9.52
15-20	10	15.87
20-25	7	11.11
25-30	8	12.7
30-35	0	0
35-40	3	4.76
40-45	0	0
45-50	3	4.76
50-55	0	0
55-60	1	1.59

Male-female ratio was 1.5:1 (Table 2). Pediatric patients were mostly males while adults were both males and females almost equal in numbers. There was no significant correlation found between sex and age at presentation (Table 3).

Table 2: Sex distribution (n=63)

Sex	No. of cases (n=63)	Percentage
Male	38	60.32%
Female	25	39.68%

Table 3: Comparison between paediatric and adult with sex

Sex	<10 years (n=25)	>10 years (n=38)
Male	16	22
Female	9	16

x² = 0.235; p=0.628

Headache was the most common presentation. Fifty eight patients had headache at presentation. Nausea, vomiting, papilloedema and cognitive impairment were present in majority of patients due to raised intracranial tension irrespection of the location of the tumor infratentorial or supratentorial. Twenty two patients of posterior fossa ependymoma had ataxia, ten had nystagmus and nineteen had vertigo. Patients of supratentorial tumors presented with Aphasia, convulsion and weakness of limbs. One patient of third ventricular ependymoma presented as Perinaud syndrome. Seizure was present in ten out of fourteen cases of supratentorial ependymomas which constitute 71.4% (Table 4).

Table 4: Presenting complaints / signs (n=63)

	No. of cases	Percentage
Headache	58	92%
Nausea/vomiting	31	49.2%
Papilloedema	40	63.5%
Aphasia	1	1.6%
Seizure	10	15.9%
Motor impairment	4	6.3%
Vertigo	19	30.2%
Cognitive impairment	43	68.3%
Perinaud Syndrome	1	1.6%
Ataxia	22	34.9%
Nystagmus	10	15.9%

CT scan was done in 52 cases and MRI was done in eleven cases. Fourteen cases were in supratentorial region, out of which four were parenchymal, eight were in lateral ventricle and two patients had tumor in the third ventricle. Forty nine patients had ependymoma in infratentorial region. In ten patients, tumour was extending laterally to CP angles and cisterns. In nineteen cases, tumor was extending in floor of fourth ventricle. Few cases were located purely inside the fourth ventricle without any lateral extension or extension to floor (Table 5).

Table 5: Location (n=63)

Location	No. of cases (n=63)	Percentage
Supratentorial Parenchymal Third ventricle Lateral ventricle	14 4 2 8	22.2% 6.3% 3.2% 12.7%
Infratentorial Lateral extension Floor extension Lateral + Floor extension No extension	49 10 19 15 5	77.8% 15.9% 30.2% 23.8% 7.9%

Discussion

Ependymomas are infrequently seen tumours and they arise from ependymal cell lining the ventricles and central canal of the spinal cord. It constitutes 2-9% of all intracranial tumours according to literature [3, 4, 5, 6, 7]. In our study, we had 63 patients of ependymoma among the total 1125 intracranial tumours during January 2001 and August 2009. This constitutes 5.6% cases.

At least half of ependymomas present in the first two decades of life [8]. In our study 41 patients were less than 20 years age, that is about 65% patients were in first two decades. About 90% patients were less than 30 years age.

The male-female ratio in literature is 1.4:1 [3, 4, 5, 6, 7] and we found 1.5:1 ratio. The male-female ratio in less than 10 years age group was 1.8 and in more than 10 years age group, it was 1.3. This difference may be due to ignorance of female child in India.

About 65% of ependymomas occur in posterior fossa according to literature. In our study, 77.8% tumours were in posterior fossa. Ependymoma of third ventricle are very rare according to literature. We also had only 2 cases of third ventricular ependymoma. We had 4 cases of supratentorial parenchymal ependymomas, this is supported by the theory that ependymoma arise from rests of ependymal cells remaining within the brain parenchyma during embryological development [3, 4].

Ependymomas frequently fill the entire fourth ventricle, insinuating themselves into the foramina of luschka as well as out through the foramen of Magendie onto the posterior aspect of the spinal cord [9]. They can also frequently extend into the CP angle through a distended foramen of luschha [9]. We also came across extensive posterior fossa ependymomas, 10 were extending to CP angle and lateral cistern, 19 were involving the floor of fourth ventricle extensively and 15 were extensively involving CP angles as well the floor. Only 5 posterior fossa ependymomas had neither lateral nor the floor extension.

According to Piepmeier JM et al [10] most consistent neurological problem associated with ependymoma is cognitive impairment associated with hydrocephalus. We came across 43 patients who had cognitive impairment. This may be due to the supratentorial tumour itself or a result of hydrocephalus. Headache, nausea, vomiting and papillaedema were also very frequent finding, partly due to mass effect of the tumour itself and partly due to hydrocephalus due to obstruction to CSF flow. Among all the symptoms, we found headache to be the most persistent symptom which was seen in 92% of patients. Supartentorial epndymomas may also cause focal deficits such as aphasia and motor impairment [5]. We also had 1 aphasic patient and 4 patients who had weakness of limbs. Seizure has been reported to occur in one third of the supratentorial ependymomas [5] but in our study it was present in 71.4% cases of supratentorial tumours.

In cases of third ventricle ependymomas, patients may present with vertigo and parinaud syndrome [11]. We also had 1 patient of third ventricle tumour presenting with parinaud syndrome. Our patients complain of vertigo very frequently. It was present in 19 cases. The more common infratentorial tumours may produce nausea, vomiting and manifestations of cerebellar compression, mainly ataxia and nystagmus [4, 5]. Our 22 patients had ataxia and 10 had nystagmus.

Conclusion

Intracranial ependymoma constitute a significant proportion among brain tumours. It is more common in young people. Posterior fossa region is most commonly involved. So we should always suspect it in young patients who complain of headache, vomiting, convulsion, cognitive impairment or any focal neurological deficit and should investigate radiologically for early detection and better management.

Conflict of interest

The authors declare that they have no competing interests.

Author’s Contribution

RSP: Concept and Analysis of cases study, Literature search and preparation of manuscript.
KS: Concept and design of study.
VS: Concept of study and final approval of manuscript.

Ethical Consideration

This study was approved by Institute Ethics committee

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