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Table of Contents    
CASE REPORT
Year : 2021  |  Volume : 69  |  Issue : 4  |  Page : 1045-1047

Pineal Anaplastic Ependymoma - A Rare Entity


1 Department of Pathology, Chettinad Hospital and Research Institute, Kelambakkam, Chennai, Tamil Nadu, India
2 Department of Neurosurgery, Chettinad Hospital and Research Institute, Kelambakkam, Chennai, Tamil Nadu, India

Date of Submission24-Jan-2020
Date of Decision26-Jan-2020
Date of Acceptance06-Jun-2020
Date of Web Publication2-Sep-2021

Correspondence Address:
Devi Subbarayan
Department of Pathology, D Block, Chettinad Hospital and Research Institute, Kelambakkam, Kanchipuram - 603 103, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.325365

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 » Abstract 


Anaplastic ependymoma in the pineal region is rare. Here, we present a rare case of anaplastic ependymoma of the pineal region on a 42-year-old woman who came to our hospital with headache associated with blurring of vision since one month. MRI brain showed a contrast enhancing mass lesion measuring 30 × 30 × 35 mm in the pineal region with obstructive hydrocephalus. Initially, the ventriculoperitoneal (VP) shunt was done, followed by total tumor excision by the infratentorial supracerebellar approach. Histopathological and immunohistochemistry examinations of the tumor showed the features of anaplastic ependymoma (WHO Grade III). Patient made uneventful recovery and underwent radiotherapy. Only 12 cases of pineal ependymoma have been reported so far, of which only three have been anaplastic ependymoma.


Keywords: Anaplastic ependymoma, IHC, pineal ependymoma, pineal region tumors
Key Message: Although ependymoma is rare in the pineal region, it should be considered in the differential diagnosis. IHC is mandatory to differentiate from papillary tumor of pineal region.


How to cite this article:
Moorthy PE, Subbarayan D, Raghavan V, Kanna RN, Ramesh VG, Karthikeyan KV. Pineal Anaplastic Ependymoma - A Rare Entity. Neurol India 2021;69:1045-7

How to cite this URL:
Moorthy PE, Subbarayan D, Raghavan V, Kanna RN, Ramesh VG, Karthikeyan KV. Pineal Anaplastic Ependymoma - A Rare Entity. Neurol India [serial online] 2021 [cited 2021 Sep 18];69:1045-7. Available from: https://www.neurologyindia.com/text.asp?2021/69/4/1045/325365




Pineal region tumors are very rare and accounts for less than 1% intracranial space occupying lesions in adults.[1] A wide variety of histological tumor subtypes can arise in the pineal region most common being germ cell tumor followed by pineal parenchymal tumor. Very rarely, glial tumors arise in the pineal region. It is paramount to establish a definitive histological diagnosis because their management is quite different. Ependymoma usually arises from the ependymal lining of the ventricles and spinal cord.[2] Pineal ependymomas are extremely rare and usually arises from a specialized ependymal cells of circumventricular organs.[3]


 » Case Report Top


A 42-year-old lady presented with head ache and blurring of vision for one month. Neurological examination showed papilloedema on fundoscopy and restriction of upward gaze. MRI Brain revealed a mass in the pineal region and the region of tectal plate measuring 30 × 30 × 35 mm, hyperintense on T2/FLAIR images and hypointense on T1-weighted image enhancing with contrast [Figure 1]a. The patient underwent VP shunt initially. Four days later, midline suboccipital craniotomy and tumour excision via infratentorial supracerebellar approach were done. The postoperative period was uneventful and the patient underwent radiotherapy.
Figure 1: (a) Preoperative contrast enhanced T1 MRI axial and saggital images showing mass lesion (red arrow) in the pineal region. (b) Preoperative contrast enhanced T1 MRI axial and saggital images showing mass lesion (red arrow) in the pineal region. (c) Post operative contrast enhanced CT brain axial image show evidence of complete excision of the tumour

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Pathologic findings

Initially, squash cytology was sent intraoperatively and showed [Figure 2]a sheets of atypical cells with round to oval nuclei with vescicular chromatin, frequent mitosis, moderate pleomorphism in a fibrillary background. At places, the tumor cells were admixed with lymphocytes, focal calcification, and prominent endothelial cell proliferation. No necrosis was seen and the possibility of high-grade glioma was suggested. The microscopic examination of routine formalin-fixed specimen [Figure 2]b showed multiple fragments of tumour tissue composed of round to oval cells with speckled nuclear chromatin and fibrillary cytoplasm with interspersed numerous blood vessels. The cells were arranged radially around the blood vessels with perivascular fibrillary processes (pseudorosettes) at places. The cells exhibited moderate pleomorphism, few multinucleated giant cells, frequent mitosis (around 12-15/10HPF), and intranuclear inclusions. There was focal calcification, myxoid change, microcystic spaces, and intratumoral hemorrhage.
Figure 2: (a) Squash smear showing tumour cells showing round to oval hyperchromatic nucleus with salt and pepper chromatin in some (red arrow), moderate pleomorphism & frequent mitosis (yellow arrow). (b) Tumour showing round to oval nucleus with speckled chromatin, microcystic spaces (yellow arrow), increased proliferation of blood vessels.(H and E stain, 200X). (c) IHC- GFAP exhibiting diffuse strong positivity in tumour cells. (d) Ki 67 showing nuclear positivity (yellow arrows). (e) S100 – tumour cells showing nuclear & cytoplasmic positivity

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Immunohistochemically, tumor cells expressed diffuse cytoplasmic membranous positivity for GFAP (glial fibrillary acidic protein) (clone -EP13) and S100 (clone-EP 32) on both nuclear and cytoplasmic positivity. For Ki67 labelling index (clone-MMI) - 42% tumour cells show positivity based on MIB-1 index [Figure 2]c and [Figure 2]d. The features were suggestive of anaplastic ependymoma (WHO Grade III).

Following surgery, radiotherapy was given and the patient tolerated well. She is being followed up as an outpatient.


 » Discussion Top


Pineal gland tumor is an uncommon tumor accounting for 0.4–1%[2] of adult intracranial tumors and 3–8% in the pediatric age group.[3] Common neoplasm in this region being germ cell neoplasm with 40% frequency followed by pineal parenchymal tumor which accounts for approximately 20% of the cases. Glial tumors are very rare of which astrocytomas are common. Pineal region ependymomas are not very common; they usually arise from the specialized ependymal cells located in the circumventricular organs. The first case of pineal ependymoma was reported by Ringertz et al. (1954) and the second case was reported by Chor et al. (1998).[1] So far, 12 cases of pineal ependymoma including three anaplastic ependymomas have been reported in the literature.[1],[4],[5],[6]

Clinically, pineal gland tumors present with features of increased intracranial pressure, obstructive hydrocephalus, and Parinaud's syndrome. MRI brain with contrast is the investigation of choice. The definitive diagnosis of the tumor type can be made by histopathological and immunohistochemical examination of the excised tumor specimen.

Histologically, ependymomas are characterized by monomorphic cells with round to oval nuclei, speckled nuclear chromatin. The key features being pseudorosettes and true rosettes, the other features include myxoid degeneration, intratumoral hemorrhage, calcification, and microcystic changes. Our case showed all these features. The differential diagnosis of ependymoma in this region includes papillary tumor of pineal region, especially if the papillae and pseudorosettes are prominent, as both can show these features. Papillary tumor of the pineal region is a newly included entity in WHO 2016 guidelines and is characterized by eosinophilic cuboidal to columnar cells arranged in the pseudorosette in a distinctive papillary pattern and are thought to be of ependymal origin based on their ultrastructural features.

Immunohistochemically, ependymoma expresses diffused positivity for GFAP, whereas the papillary tumor of the pineal region shows only focal GFAP positivity around the vessels (perivascular). Since our case exhibited strong diffuse positivity for GFAP and negative for CK (cytokeratin), we ruled out the latter.

Anaplastic ependymoma usually has an unfavorable prognosis, high cellularity focally or throughout, nuclear pleomorphism and/or necrosis of which mitotic activity is an important criterion. In our case, MIB 1 labelling index showed around 40%. Pineal gland ependymoma on a total of 12 cases have been reported[1],[4],[6],[7] of which only three cases of anaplastic ependymoma in the pineal region are reported in the literature [Table 1].[4],[7] The present case is the thirteenth case of pineal ependymoma and fourth case of anaplastic ependymoma in the pineal region to be reported.
Table 1: Number of ependymoma cases in the pineal region reports so far in the literature.

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Ependymomas are usually managed by combined surgery with adjuvant radiotherapy.[7] Our case was managed by surgery with adjuvant radiotherapy. The postoperative CECT did not reveal any mass on follow up.

This case is being presented for its rare occurrence and should be considered in the differential diagnosis of pineal tumors. Histopathology and immunohistochemistry are essential for the diagnosis and appropriate management.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 » References Top

1.
Hyun SJ, Park SW, Nam TK, Hwang SN. A case of Pineal ependymoma. J Korean Neurosurg Soc 2007;42:56-8.  Back to cited text no. 1
    
2.
Patil M, Karandikar M. Papillary tumour of pineal region: A rare entity. Med J DY Patil Univ 2013;6:302-4.  Back to cited text no. 2
  [Full text]  
3.
Smith AB, Rushing EJ, Smirniotopoulos JG. Lesions of the Pineal region: Radiologic pathologic correlation. Radiographics 2010;30:2001-2020.  Back to cited text no. 3
    
4.
Ben Bouallègue F, Vaucht F, Menjot de Champfleur N, Mariano-Goulart D. Pineal anaplastic ependymoma with multifocal intradural extramedullary metastases on MRI and 18FDG-PET. Clin Nucl Med 2018;43:319-21.  Back to cited text no. 4
    
5.
Cho BK, Wang KC, Nam DH, Kim DG, Jung HW, Kim HJ, et al. Pineal tumors: Experience with 48 cases over 10 years. Child Nerv Syst 1998;14:53-8.  Back to cited text no. 5
    
6.
Sano K. Pineal region tumors: Problems in pathology and treatment. Neurol India 1983;30:59-91.  Back to cited text no. 6
    
7.
Abbassy M, Aref K, Farhoud A, Hekal A. The supracerebellar infratentorial approach in pineal region tumors: Technique and outcome in an underprivileged setting. Alexandria J Med 2018;54:725-9.  Back to cited text no. 7
    


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