
==== Front
Int J Surg Case Rep
Int J Surg Case Rep
International Journal of Surgery Case Reports
2210-2612
Elsevier

S2210-2612(24)00870-8
10.1016/j.ijscr.2024.110089
110089
Case Report
Anaplastic cortical ependymoma in 10 months girl: A case report
Kitaz Mohammad Nour a
Dabbagh Ezeddin ezzalldin.da@gmail.com
b⁎
Hallak Hasan a
Alali Kutaiba a
Wereekia Mahmoud a
Kadi Mohamad a
a Department of Neurosurgery, Aleppo University Hospital, University of Aleppo, Aleppo, Syrian Arab Republic
b Faculty of Medicine, University of Aleppo, Aleppo, Syrian Arab Republic
⁎ Corresponding author. ezzalldin.da@gmail.com
27 7 2024
10 2024
27 7 2024
123 11008928 3 2024
20 7 2024
25 7 2024
© 2024 Published by Elsevier Ltd on behalf of IJS Publishing Group Limited.
2024

https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Introduction and importance

Ependymomas arise from the ependymal cells that line the brain ventricles, and central canal. In children most of them are benign. However, cortical anaplastic ependymomas are very rare in pediatrics.

Case presentation

A 10 months girl presented with vomits for one week, increased head circumference, psychomotor development delay, left facial nerve (VII) palsy, and left hemiparesis 3/5. Magnetic resonance imaging (MRI) of the brain demonstrated a large parenchymal lesion filling most of the right hemisphere. She underwent a total excision of the lesion. The tumor had no connection to the ventricular ependymal lining. No adjuvant chemotherapy or radiotherapy was considered. The final diagnosis is Anaplastic Ependymoma (WHO Grade III).

Clinical discussion

Cortical anaplastic ependymomas are extremely rare. In pediatrics they affect frontal, frontoparietal, and parietal lobes, Temporal and occipital lobes are uncommon. Migration disorders from the germinal matrix and the differentiation of primitive neuroectodermal tumors along the ependymal lineage are considered two hypotheses that explain the pathogenesis of ectopic ependymomas.

Conclusion

Ependymomas should be considered a differential diagnosis in children, as the successful total removal of ependymomas in that age saves the patient from the need of adjuvant therapy such as radio­therapy or chemotherapy.

Highlights

• Ependymomas arise from the ependymal cells that line the brain ventricles, choroid plexus, filum terminal and central canal of the spinal cord

• Most of these tumors are histologically benign in the pediatric population

• Although ependymomas exhibit some biologic variability concerning growth rate

• Most of ependymomas cases are (WHO) grade II (70%).

• Myxopapillary ependymomas – WHO grade I lesions – present only 2% of the incidence, and the remainder are anaplastic ependymomas – WHO grade III lesions.

• Patient’s age is considered one of the most important prognosis factors of ependymomas, the worst prognosis appears on children younger than 3 years.

• Cortical anaplastic ependymomas are very rare in pediatrics and not well studied in this population

Keywords

Anaplastic ependymoma
Cortical
Pediatrics
==== Body
pmc1 Introduction

Ependymomas arise from the ependymal cells that line the brain ventricles, choroid plexus, filum terminal and central canal of the spinal cord. Most of these tumors are histologically benign in the pediatric population [1].

Although they exhibit some biologic variability concerning growth rate; most of them are (WHO) grade II (70 %). But myxopapillary ependymomas – WHO grade I lesions – present only 2 % of the incidence, and the remainder are anaplastic ependymomas – WHO grade III lesions [2].

The patient's age is considered one of the most important prognostic factors of ependymomas, as the worst prognosis appears on children younger than 3 years [3].

Cortical anaplastic ependymomas are very rare in pediatrics and not well studied in this population [1]. Here we present a rare case of cortical anaplastic ependymomas in a 10-month-old girl, using SCARE criteria [14].

2 Case presentation

A female infant aged 10 months was brought to our hospital clinic with a one-week history of vomiting. Physical assessment revealed an elevated head circumference, delayed psychomotor development, paralysis of the left facial nerve (VII), and left-sided hemiparesis graded 3/5. Her medical and family background were unremarkable.

Magnetic resonance imaging (MRI) of the brain demonstrated a large parenchymal lesion filling most of the right hemisphere, with heterogenous enhancement in contrast. The lesion measured 9 cm in the great dimension, and it accompanies by edema, which causes a mass effect on the ipsilateral hemisphere and midline shift (Fig. 1).Fig. 1 MRI of the brain demonstrated a large right parenchymal lesion, with heterogenous enhancement in contrast, and it accompanies by edema.

Fig. 1

She underwent a right trauma flap trans subtemporal sulci approach and gross total excision of the lesion. The tumor had no connection to the ventricular ependymal lining. It was greyish-white, soft, and suckable, with a cystic bleeding area of the necrotic tumor (Fig. 2). No adjuvant chemotherapy or radiotherapy was considered because a gross total resection was done.Fig. 2 Gross view of the tumor.

Fig. 2

Pathology: sections show dense cellularity and conspicuous mitotic activity accompanied by complex microvascular proliferation, extensive necrosis, perivascular pseudorosettes and true rosettes (Fig. 3).Fig. 3 Pathology sections show A: dense cellularity, conspicuous mitotic activity, perivascular pseudorosettes, true rosettes, and B: extensive necrosis.

Fig. 3

Immunostain show (GFAP, CD99, EMA) was positive. The final diagnosis is Anaplastic Cortical Ependymoma (WHO Grade III).

After three months of follow-up: the patient made a good recovery, vomiting stopped, and left hemiparesis improved (4/5), but (VII) nerve palsy remained. Computed tomography (CT) on the day after the operation showed no evidence of residual lesions.

3 Discussion and conclusion

Ependymomas are neuroectodermal tumors; which grow in the cerebral ventricles or the central canal of the spinal cord [4,5]. In children, ependymomas commonly arise in the infratentorial region. Third and lateral ventricles are commonplace in Supratentorial cases. Cortical anaplastic ependymomas are extremely rare [2].

Pediatric cortical ependymomas affect the frontal lobe, frontoparietal lobe, and parietal lobe. Temporal and occipital lobes are uncommon locations [6].

Glioblastoma multiforme, supratentorial PNET, ganglioglioma, and oligodendroglioma considerate as differential diagnosis for ependymomas [7].

On unenhanced T1-weighted MRIs, they usually are isointense to hypointense and hyperintense on T2-weighted MRI. Anyway, those findings are untypical [7].

Several hypotheses have been proposed to elucidate the development of supratentorial ectopic ependymomas. One theory posits that anomalies in germinal matrix migration lead to the formation of intraparenchymal or subarachnoid ependymal cysts, which subsequently progress into tumors. Alternatively, ependymomas are postulated to originate as a subtype of primitive neuroectodermal tumors that undergo differentiation along the ependymal lineage [8].

Patients younger than 15 years show lower overall survival. In such cases, subtotal resection and adjuvant therapy have been used [9].

in general, total resection is the ideal treatment, but it can be applied only in 30–40 % of cases; because of vital structures involvement [10].

In children, when ependymomas are completely eradicated, adjuvant radiation therapy is not indicated [11].

Incomplete surgical resection of ependymomas is considered an indicator of chemotherapy in patients under the age of two [12,13].

Although it is rare, ependymomas should be considered a differential diagnosis in children; because the successful total removal of ependymomas in that age makes the patient no longer require adjuvant therapy such as radio­therapy or chemotherapy [5].

Abbreviations

MRI magnetic resonance imaging

WHO World Health Organization

Consent

Written informed consent was obtained from the patient's parents/legal guardian for publication and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Ethical approval

This study is exempt from ethical approval in our institution (Aleppo University Hospital, Faculty of Medicine, University of Aleppo, Aleppo, Syria.

Funding

There are no sources of funding.

Author contribution

MK: Supervisor, Surgeon.

MW, KA, HH: Surgeon, analyzed and interpreted the patient data, wrote the manuscript, revision.

MNK: Analyzed and interpreted the patient data, wrote the manuscript, revision.

ED: Wrote the manuscript and was the corresponding author.

All authors read and approved the final manuscript.

Guarantor

Mohammad Nour Kitaz.

Research registration number

Not applicable in Syria.

Conflict of interest statement

The authors declare that they have no competing interests.

Availability of data and materials

All data generated or analyzed during this study are included in this published article and its supplementary information files.

Acknowledgements

Not applicable.
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