LGCmain
Indian Journal of Pathology and Microbiology
Home About us Instructions Submission Subscribe Advertise Contact e-Alerts Ahead Of Print Login 
Users Online: 4388
Print this page  Email this page Bookmark this page Small font sizeDefault font sizeIncrease font size
IJPM is coming out with a Special issue on "Genitourinary & Gynecological pathology including Breast". Please submit your articles for these issues


 
CASE REPORT Table of Contents   
Year : 2010  |  Volume : 53  |  Issue : 4  |  Page : 787-789
Recurrent posterior fossa anaplastic ependymoma with prominent chondroid metaplasia: A case report and review of literature


1 Department of Pathology and Transfusion Medicine, Sri Sathya Sai Institute of Higher Medical sciences, Bangalore, Karnataka, India
2 Department of Neurosurgery, Sri Sathya Sai Institute of Higher Medical sciences, Bangalore, Karnataka, India
3 Department of Neurosciences, Sri Sathya Sai Institute of Higher Medical sciences, Bangalore, Karnataka, India
4 Department of Radiology, Sri Sathya Sai Institute of Higher Medical sciences, Bangalore, Karnataka, India

Click here for correspondence address and email

Date of Web Publication27-Oct-2010
 

   Abstract 

We report an unusual case of a recurrent fourth ventricular anaplastic ependymoma with prominent chondroid metaplasia in a 16-year-old male. On initial presentation, the patient had a WHO Grade II tumor. However, at recurrence 1 year later, the tumor progressed to WHO Grade III tumor with more cellularity, necrosis and brisk mitotic activity. Chondroid metaplasia was present in both the initial and recurrent tumors.

Keywords: Adolescent, anaplastic ependymoma, chondroid metaplasia, recurrent, fourth ventricle

How to cite this article:
Ghosal N, Murthy G, Dadlani R, Hegde AS, Singh D. Recurrent posterior fossa anaplastic ependymoma with prominent chondroid metaplasia: A case report and review of literature. Indian J Pathol Microbiol 2010;53:787-9

How to cite this URL:
Ghosal N, Murthy G, Dadlani R, Hegde AS, Singh D. Recurrent posterior fossa anaplastic ependymoma with prominent chondroid metaplasia: A case report and review of literature. Indian J Pathol Microbiol [serial online] 2010 [cited 2019 Dec 8];53:787-9. Available from: http://www.ijpmonline.org/text.asp?2010/53/4/787/72092



   Introduction Top


Gliomas containing foci of cartilaginous metaplasia with or without associated bone formation are very rare. Mackay et al. [1] were the first to describe a case in a 4.5-year-old boy who had an ependymoblastoma in the fourth ventricle. Since then, only seven cases have been documented in the literature. [2] We report a case of ependymoma with prominent cartilaginous metaplasia (WHO Grade II) in an adolescent male aged 16 years. The patient developed recurrent tumor (twice), 1 year after the surgical excision of the tumor. The recurrent tumor showed features of anaplastic ependymoma (WHO Grade III) with increased cellularity, brisk mitotic activity and necrosis with similar chondroid metaplasia.


   Case Report Top


A 16-year-old male presented with features of raised intracranial pressure of 3 months duration. He had cerebellar ataxia, right hemiparesis of grade 4+/5 and blurring of vision of 1 month duration. Magnetic resonance imaging (MRI) revealed a well-defined, lobulated heterogenous lesion in the fourth ventricle, measuring 4.7 × 3.6 × 5.3 cm, hypointense on T1 weighted images and hyperintense on T2 weighted images with areas of hemorrhage. The mass demonstrated moderate enhancement in post contrast studies [Figure 1]a, b. The lesion was causing mass effect over the brain stem anteriorly, compressing and elevating the cerebellar hemisphere posterosuperiorly. Inferiorly, there was mild extension into the posterior subarachnoid space at the level of foramen magnum.
Figure 1 :(a) Axial T1 post contrast image at the level of fourth ventricle, showing homogenously enhancing mass occupying the ventricle; (b) saggital T1 post contrast image showing the craniocaudal extension and mass effect on the brain stem and cerebellum

Click here to view


The patient underwent a midline suboccipital craniectomy and tumor decompression. Intraoperatively, the lesion was firm and gritty with some parts soft and suckable. A small residue was left, which was infiltrating the floor of the fourth ventricle on the left side.

The tissue was sent for further histopathologic evaluation. The tissue was fixed in 10% formalin and routinely processed. Five micron thick sections were cut and stained with hematoxylin and eosin stain. On light microscopy, the tumor was found to be composed of two components intimately mixed with each other. One of the components was composed of round to oval cells with hyperchromatic nuclei showing a prominent perivascular and a few ependymal rosettes along with the other prominent component of cartilaginous metaplasia [Figure 2], [Figure 3]. The nuclei showed coarse clumping of chromatin with mitotic activity [4-5/10 high power field (hpf)]. The cytoplasm at many places showed cytoplasmic fibrillary extensions [Figure 2]b. Chondroid areas showed typical uninucleate chondrocytes with pericellular lacunae and focal areas of calcification. There was no pleomorphism, multi-nucleation or mitotic activity in the cartilaginous areas. The cartilaginous islands were unencapsulated. No necrosis was seen. Immunohistochemical analysis was done by Avidin Biotin Complex immunoperoxidase method with antibody dilution of 1:50 for glial fibrillary acidic protein (GFAP) and epithelial membrane antigen (EMA) (Dakopatts Laboratories, Copenhagen, Denmark). The tumor cells were strongly positive for GFAP [Figure 4]a and negative for EMA [Figure 4]b. A final histopathologic diagnosis of ependymoma with chondroid metaplasia (WHO Grade II) was done.
Figure 2 :(a, b) Paraffin section showing ependymoma with prominent chondroid metaplasia (H and E, ×100, and H and E, ×400 for a and b, respectively)

Click here to view
Figure 3 :Paraffin section showing (a) ependymal rosettes (b) chondroid metaplasia (H and E, ×400)

Click here to view
Figure 4 :Immunohistochemistry for (a) GFAP is strongly positive in the tumor cells and also in few chondrocytes; (b) negative reaction with EMA (Avidin Biotin Complex peroxidase method; ×400)

Click here to view


Postoperatively, the patient was lost to follow up and did not comply with the recommended radiotherapy. He presented to our emergency services, a year later, with gradually progressive history of raised intracranial pressure of 1 month duration. A repeat MRI revealed a heterogenously enhancing lesion T2 hyperintense and T1 hypointense mass measuring 4.8 × 3.5 × 4.1 cm in the fourth ventricle, invading the left cerebellar hemisphere. The lesion was layering the floor of the fourth ventricle and extending through the foramen of Magendie. Moderate obstructive hydrocephalus was present. The patient underwent re-explorative surgery and tumor decompression. Intraoperatively, the tumor was more vascular than the initial surgery. There was a poor plane of cleavage between the tumor and the fourth ventricle floor. The anterior inferior cerebellar artery was encased by the tumor on the left side. In view of the infiltrative nature of the tumor, only subtotal decompression could be achieved.

The tissue was sent for histopathologic evaluation. On light microscopy, the tumor this time showed more cellularity, brisk mitotic activity (≥20/hpf) and large areas of necrosis [Figure 5]a, b. There were perivascular rosettes as well as occasional true ependymal rosettes. Moderate cellular and nuclear pleomorphism was seen along with a few tumor giant cells. The recurrent tumor also showed chondroid metaplasia; however, no pleomorphism or mitotic activity was seen in this component. A final histopathologic diagnosis of recurrent anaplastic ependymoma with chrondroid metaplasia (WHO Grade III) was made. Postoperatively, the patient received craniospinal radiotherapy, subsequent to which he had a second recurrence 5 months later. Re-exploratory surgery was done, subsequent to which he was lost to follow up. Histopathologically, it was anaplastic ependymoma with chrondroid metaplasia (WHO Grade III).
Figure 5 :Paraffin section of the recurrent tumor showing (a) large areas of necrosis with (b) marked cellularity, pleomorphism and brisk mitotic activity (H and E, ×400)

Click here to view



   Discussion Top


Gliomas containing foci of cartilaginous metaplasia with or without associated bone formation are very rare. [3] They are characteristically described in the midline location, mostly in the fourth ventricle ependymomas or more rarely in pontine astrocytomas. [4] Till date, seven cases of ependymomas of different WHO grades have been described in the literature, showing chondroid metaplasia with or without osseous differentiation. [5],[6],[7],[8],[9] Of the total seven, four were in children and three in adult patients (age ranged from 4.5 to 61 years with male:female ratio of 5:2). They occurred in different locations like fourth ventricle (4), suprasellar and prepontine (1), frontal lobe (1) and temporal lobe (1). Review of the literature shows the fourth ventricle to be the favored site. The present case is in an adolescent boy aged 16 years, with a fourth ventricular tumor. At the initial presentation, the tumor showed histomorphology of WHO Grade II; however, on recurrence after 1 year, it upgraded to WHO Grade III with numerous mitotic figures, more cellularity and chondroid metaplasia. It is noteworthy that only the ependymal component progressed to higher grade and the chondroid component retained the benign morphology. This is the first case in literature where the tumor was of lower grade at presentation, but on recurrence, transformed to the anaplastic variant and had multiple frequent recurrences despite tumor decompression and postoperative radiotherapy. Mridha et al. [8] described anaplastic ependymoma with osseocartilaginous metaplasia in a 9-year-old male, located in suprasellar, prepontine and left cerebellopontine angle region. Bannykh et al. [2] described a left frontal lobe anaplastic ependymoma in a 61-year-old male. At the initial presentation, there was no chondroid differentiation, but on recurrence, the tumor showed large areas of chondroid differentiation.

Several mechanisms have been hypothesized for the origin of chondroid metaplasia in gliomas and other tumors. These could be teratomatous changes, heteroplasia, ossification in a mucoid matrix, transformation of neuroepithelial cells to mesenchymal cells or mixed mesenchymal-neuroepithelial changes in gliosarcomas. [10],[11] However, the most accepted theories are either origin of metaplastic cells from the neoplastic glial cells or chondroid metaplasia of connective tissue within the tumor. Kepes et al. [7] have described transformation of neuroepithelial cells to mesenchymal cells in four cases. They observed the presence of transitional elements between glial and cartilaginous tissue. The presence of GFAP-immunopositive chondrocytes confirmed their astrocytic origin. They also postulated that such a phenomenon might be due to the ability of malignant astrocytes to produce basement membrane material and other mucopolysaccharides. Our case also showed GFAP positivity in the tumor cells as well in a few chondrocytes. Cartilaginous differentiation in this tumor can also be a secondary phenomenon under the influence of some cytokines secreted by neoplastic cells, the nature of which is still not known.

To conclude, we present a rare case of fourth ventricular anaplastic ependymoma with marked chondroid metaplasia with tumor progression from a Grade II to a Grade III (WHO grading). To the best of our knowledge, this is the eighth case of ependymoma with chondroid differentiation, but the only case to be reported with evidence of tumor progression. This particular variant has been found to have a more aggressive clinical course and poor prognosis, necessitating a close follow up and aggressive adjuvant therapy.

 
   References Top

1.Mackay RP. Ependymoblastoma in the fourth ventricle with new bone formation. Arch Neurol Psychiatr (Chicago) 1935;34:844-53.  Back to cited text no. 1
    
2.Bannykh S, Baehring JM. Images in neuro-oncology: Rapid development of osseous and chondrous metaplasia in recurrent anaplastic ependymoma. J Neurooncol 2007;81:257-8.  Back to cited text no. 2
[PUBMED]  [FULLTEXT]  
3.Banerjee AK, Sharma BS, Kak VK, Ghatak NR. Gliosarcoma with cartilage formation. Cancer 1989;63:518-23.  Back to cited text no. 3
    
4.Mclendon ER, Enterline SD, Tien DR, Thorstad LW, Bruner MJ. Tumors of central neuroepithelial origin. In: Bigner DD, Mclendon ER, Bruner MJ, editors. Russell and Rubinstein's Pathology of tumors of the nervous system. 6 th ed. London: Arnold; 1998. p. 398-9.  Back to cited text no. 4
    
5.Jain A, Rishi A, Suri V, Garg A, Sharma MC, Sarkar C, et al. Recurrent ependymoma with cartilaginous metaplasia in an adult: Report of a rare case and review of literature. Clin Neuropathol 2009;28:101-4.  Back to cited text no. 5
[PUBMED]  [FULLTEXT]  
6.Siqueira EB, Bucy PC. Case report: Chondroma arising within a mixed glioma. J Neuropathol Exp Neurol 1966;25:667-73.  Back to cited text no. 6
[PUBMED]    
7.Kepes JJ, Rubinstein LJ, Chiang H. The role of astrocytes in the formation of cartilage in gliomas. An immunohistochemical study of four cases. Am J Pathol 1984;117:471-83.  Back to cited text no. 7
[PUBMED]  [FULLTEXT]  
8.Mridha AR, Sharma MC, Sarkar C, Garg A, Singh MM, Suri V. Anaplastic ependymoma with cartilaginous and osseous metaplasia: Report of a rare case and review of literature. J Neurooncol 2007;82:75-80.  Back to cited text no. 8
[PUBMED]  [FULLTEXT]  
9.Feyza KG Erhan E, Cicek B. Ependymoma with cartilage formation: A case report. Turk Neurosurg 2005;15:12-7.  Back to cited text no. 9
    
10.Kawamata T, Kubo O, Kawamuru H, Iwala Y, Kagawa M, Kitamura K. Ossified choroid plexus papilloma, case report. No Shinkei Geka 1988;16:989-94.  Back to cited text no. 10
    
11.Maleci A, Giordano MT, Bianco F, Di Lorenzo N. Intramedullary astrocytoma with cartilage formation, case report. J Neurosurg Sci 1996;40:157-60.  Back to cited text no. 11
    

Top
Correspondence Address:
Nandita Ghosal
Department of Pathology and Transfusion Medicine, Sri Sathya Sai Institute of Higher Medical sciences, EPIP Area, Whitefield, Bangalore, Karnataka
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.72092

Rights and Permissions


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

This article has been cited by
1 Anaplastic ependymoma of the third ventricle
Alberto Feletti,Elisabetta Marton,Matteo Bendini,Lucia Zanatta,Laura Valori,Angelo Paolo Dei Tos,Francesco Di Paola,Pierluigi Longatti,Sabrina Rossi
Brain Tumor Pathology. 2014;
[Pubmed] | [DOI]
2 Rare histological variants in ependymomas: Histopathological analysis of 13 cases
Gessi, M., Kuchelmeister, K., Lauriola, L., Pietsch, T.
Virchows Archiv. 2011; 459(4): 423-429
[Pubmed]
3 Rare histological variants in ependymomas: histopathological analysis of 13 cases
Marco Gessi,Klaus Kuchelmeister,Libero Lauriola,Torsten Pietsch
Virchows Archiv. 2011; 459(4): 423
[Pubmed] | [DOI]



 

Top
 
  Search
 
  
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
    Introduction
    Case Report
    Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed4597    
    Printed112    
    Emailed0    
    PDF Downloaded61    
    Comments [Add]    
    Cited by others 3    

Recommend this journal