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Table of Contents
CASE REPORT
Year : 2019  |  Volume : 16  |  Issue : 3  |  Page : 161-163

Supratentorial ependymoma: A rare presentation in an adolescent


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 Submission05-May-2019
Date of Acceptance17-Aug-2019
Date of Web Publication11-Sep-2019

Correspondence Address:
Dilip Rajasekharan
Department of General Surgery, Apollo BGS Hospitals, Mysore - 570 023, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/am.am_19_19

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  Abstract 


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

How to cite this URL:
Rajasekharan D, Bopaiah KS, Moin A, Nagaraja JB. Supratentorial ependymoma: A rare presentation in an adolescent. Apollo Med [serial online] 2019 [cited 2019 Nov 18];16:161-3. Available from: http://www.apollomedicine.org/text.asp?2019/16/3/161/266772




  Introduction Top


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.[1],[2],[3] 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.[4] It has no gender predilection, and on the contrary, the more common intracranial variety tends to be found in the adult age group.[5],[6] Histologically, they are well-demarcated tumors with a capacity for infiltration into the surrounding tissue.[7] They are highly aggressive in nature with high recurrence rates. This leads to the potential malignant behavior exhibited by the tumor.[4]

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 Top


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.
Figure 1: Radiological image showing supratentorial ependymoma

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Operative course

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.

Histopathological findings

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 Top


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.[4] Supratentorial ependymomas usually arise from the ependymal cells in the third or lateral ventricle.[8] 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.[9] 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 [10] or originate from glial cells with progenitor properties.[4],[11]

The clinical presentation of intraparenchymal supratentorial lesions reflects that of focal neurologic deficits, headache, and seizures.[6] The common anatomic locations include frontal and parietal lobe.[12] The presentation of a supratentorial anaplastic ependymoma is usually that of a heterogeneous-enhancing solid lesion with separate cystic component if present.[13] The typical histologic characteristics are that of tumor cells arranged in perivascular pseudorosettes.[4]

Among the important prognostic factors include tumor location, histology, and extent of resection.[14] 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.[4]

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 Top


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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Alexiou GA, Moschovi M, Stefanaki K, Sfakianos G, Prodromou N. Epidemiology of pediatric brain tumors in Greece (1991-2008). Experience from the Agia Sofia Children's Hospital. Cent Eur Neurosurg 2011;72:1-4.  Back to cited text no. 1
    
2.
Alexiou GA, Stefanaki K, Moschovi M, Patereli A, Prodromou N, Karentzou O, et al. Immunohistochemical expression of cell cycle/apoptosis regulators and epidermal growth factor receptor in pediatric intracranial ependymomas. J Child Neurol 2011;26:195-8.  Back to cited text no. 2
    
3.
Alexiou GA, Moschovi M, Stefanaki K, Panagopoulos D, Tsotra M, Siozos G, et al. Supratentorial ependymomas in children: Analysis of nine cases. J Pediatr Neurosci 2013;8:15-8.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Beniwal M, Mishra A, Rao KV, Vazhayil V, Nandeesh BN, Somanna S, et al. Supratentorial pure cortical ependymoma: An unusual lesion causing focal motor aware seizure. J Neurosci Rural Pract 2018;9:264-7.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Mangalore S, Aryan S, Prasad C, Santosh V. Imaging characteristics of supratentorial ependymomas: Study on a large single institutional cohort with histopathological correlation. Asian J Neurosurg 2015;10:276-81.  Back to cited text no. 5
[PUBMED]  [Full text]  
6.
Koeller KK, Sandberg GD, Armed Forces Institute of Pathology. From the archives of the AFIP. Cerebral intraventricular neoplasms: Radiologic-pathologic correlation. Radiographics 2002;22:1473-505.  Back to cited text no. 6
    
7.
Rosenblum MK. Ependymal tumors: A review of their diagnostic surgical pathology. Pediatr Neurosurg 1998;28:160-5.  Back to cited text no. 7
    
8.
Niazi TN, Jensen EM, Jensen RL. WHO grade II and III supratentorial hemispheric ependymomas in adults: Case series and review of treatment options. J Neurooncol 2009;91:323-8.  Back to cited text no. 8
    
9.
Hamano E, Tsutsumi S, Nonaka Y, Abe Y, Yasumoto Y, Saeki H, et al. Huge supratentorial extraventricular anaplastic ependymoma presenting with massive calcification case report. Neurol Med Chir (Tokyo) 2010;50:150-3.  Back to cited text no. 9
    
10.
Ehtesham M, Kabos P, Yong WH, Schievink WI, Black KL, Yu JS, et al. Development of an intracranial ependymoma at the site of a pre-existing cavernous malformation. Surg Neurol 2003;60:80-2.  Back to cited text no. 10
    
11.
Hegyi L, Peston D, Theodorou M, Moss J, Olver J, Roncaroli F, et al. Primary glial tumor of the retina with features of myxopapillary ependymoma. Am J Surg Pathol 2005;29:1404-10.  Back to cited text no. 11
    
12.
Lehman NL, Jorden MA, Huhn SL, Barnes PD, Nelson GB, Fisher PG, et al. Cortical ependymoma. A case report and review. Pediatr Neurosurg 2003;39:50-4.  Back to cited text no. 12
    
13.
Furie DM, Provenzale JM. Supratentorial ependymomas and subependymomas: CT and MR appearance. J Comput Assist Tomogr 1995;19:518-26.  Back to cited text no. 13
    
14.
Youkilis AS, Park P, McKeever PE, Chandler WF. Parasagittal ependymoma resembling falcine meningioma. AJNR Am J Neuroradiol 2001;22:1105-8.  Back to cited text no. 14
    


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  [Figure 1], [Figure 2]



 

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