|Year : 2019 | Volume
| Issue : 3 | Page : 161-163
Supratentorial ependymoma: A rare presentation in an adolescent
Dilip Rajasekharan1, Kanjithanda Subbaiah Bopaiah1, Aumir Moin2, Jayanth Bannur Nagaraja3
1 Department of Neurosurgery, Apollo BGS Hospitals, Mysore, Karnataka, India
2 Department of Neurology, Apollo BGS Hospitals, Mysore, Karnataka, India
3 Department of General Surgery, Apollo BGS Hospitals, Mysore, Karnataka, India
|Date of Submission||05-May-2019|
|Date of Acceptance||17-Aug-2019|
|Date of Web Publication||11-Sep-2019|
Department of General Surgery, Apollo BGS Hospitals, Mysore - 570 023, Karnataka
Source of Support: None, Conflict of Interest: None
Ependymomas are rare primary glial tumors that arise from ependymal cells found in the central nervous system. While usually intracranial, they rarely arise from the supratentorial parenchyma. They have a variable clinical course and are aggressive in nature. Among the important prognostic factors include tumor location, histology, and extent of resection. Total surgical excision is the rule. Diagnosis is confirmed by histopathology. Suspicion of an ependymoma must, however, be borne in mind while dealing with a supratentorial space-occupying lesion.
Keywords: Adolescent, supratentorial ependymoma, surgical management
|How to cite this article:|
Rajasekharan D, Bopaiah KS, Moin A, Nagaraja JB. Supratentorial ependymoma: A rare presentation in an adolescent. Apollo Med 2019;16:161-3
| Introduction|| |
Ependymomas are rare primary glial tumors that arise from ependymal cells found in the central nervous system. They were first described by Bailey in 1924. They may arise in the brain or the spinal cord. Ependymomas are the third most common primary brain tumor found in children and are usually found in the posterior fossa.,, Most of these ependymomas present as intracranial tumors.
In a small number of cases, the ependymoma arises from the supratentorial parenchyma. Supratentorial ependymomas are rare with a variable clinical course. It has no gender predilection, and on the contrary, the more common intracranial variety tends to be found in the adult age group., Histologically, they are well-demarcated tumors with a capacity for infiltration into the surrounding tissue. They are highly aggressive in nature with high recurrence rates. This leads to the potential malignant behavior exhibited by the tumor.
The usual presentations include that of pressure effects such as seizures which generally is the first warning sign. Relevant investigations include imaging. However, a definitive diagnosis can only be reached after surgical excision and histopathological examination. A complete in toto excision is the rule.
Only a few cases are reported in the literature. Due to the rarity of its presentation and its aggressive nature, the possibility of an ependymoma must be kept in mind when dealing with a space-occupying lesion in the supratentorial region. Here, we would like to present the case of a 17-year-old female patient who presented with seizures and was diagnosed with a supratentorial ependymoma.
| Case Report|| |
History and examination
A 17-year-old female patient presented to our hospital in May 2014 with a history of one episode of seizure and subsequent giddiness. The patient had no history of head injury or history of seizures. After initial evaluation, she was admitted for further management. A magnetic resonance imaging brain done revealed a solitary space-occupying lesion in the left parietal region [Figure 1]. After optimization, which included routine investigations, hydration, and other supportive measures, she was taken up to the operating room for a frontoparietal craniotomy and excision of the space-occupying lesion under general anesthesia on the very next day.
Under general anesthesia, the patient was placed in supine position and parts were painted and draped. The head was turned to the right with left shoulder support. A left frontoparietal horseshoe-shaped incision was made. Skin flaps were raised and subgaleal dissection was done. Free bone flaps were raised and dura was opened. The tumor was identified which was well defined and had a soft consistency. It was avascular with no cysts, septa, calcifications, or necrosis. The tumor was excised in toto. After confirmation of the well pulsatile nature of the brain, a watertight dural closure was done. The bone flap was replaced and the wound was closed in layers. Postoperative recovery was uneventful.
The excised space-occupying lesion was sent for histopathology. It was grossly found to have a gray-white, glistening, gelatinous soft tissue bits, largest measuring 2 cm × 1.5 cm × 1.5 cm. Microscopically, the sections showed elongated spindle cells with a dense meshwork of fibrillary cytoplasmic processes. Prominent pseudorosette formation was seen with few true rosettes. These cells exhibited round-to-oval nuclei with evenly distributed fine chromatin. Prominent myxoid background was noted. Tumor infiltration into surrounding brain parenchyma was also noted [Figure 2]. Final impression was of an ependymoma (WHO Grade II).
|Figure 2: Histopathological image showing the perivascular pseudorosette suggestive of ependymoma|
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| Discussion|| |
Ependymomas are rare primary glial tumors that arise from ependymal cells found in the central nervous system. The majority of ependymomas are found in children which arise in the infratentorial region. Supratentorial ependymomas usually arise from the ependymal cells in the third or lateral ventricle. Cortical supratentorial ependymomas are very rare.
The pathogenesis of supratentorial cortical ependymomas is still a matter of debate. It originates from the white matter and is hypothesized to develop from the embryonal ependymal remnants in the brain parenchyma. Some other speculations include association with a pre-existing lesion like cavernous malformation, which under effect of secreted angiogenic growth factors give rise to ependymomas  or originate from glial cells with progenitor properties.,
The clinical presentation of intraparenchymal supratentorial lesions reflects that of focal neurologic deficits, headache, and seizures. The common anatomic locations include frontal and parietal lobe. The presentation of a supratentorial anaplastic ependymoma is usually that of a heterogeneous-enhancing solid lesion with separate cystic component if present. The typical histologic characteristics are that of tumor cells arranged in perivascular pseudorosettes.
Among the important prognostic factors include tumor location, histology, and extent of resection. As grade of tumor and extent of resection are among the important indicators of survival, our aim should always be one of total resection following which favorable survival rates can be expected. The role of postoperative radiotherapy is controversial with definitive therapy indicated if the tumor is Grade III or above. The role of chemotherapy is not well elucidated.
In this report, we present the case of a 17-year-old female patient who presented with seizures and was diagnosed with a space-occupying lesion. Following surgery, the mass was subjected to histopathology that revealed an ependymoma (WHO Grade II).
| Conclusion|| |
Supratentorial ependymomas are rare tumors with a variable clinical course. Due to its aggressive clinical course, it is prudent to consider them as one of the differential diagnoses when dealing with a supratentorial space-occupying lesion.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]