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|Year : 2013 | Volume
| Issue : 3 | Page : 303-305
Cervical extradural and extraspinal ependymoma mimicking dumb-bell schwannoma: An unusual tumor
VG Ramesh1, KV Karthikeyan1, K Ramesh Rao2, C Balasubramanian1
1 Department of Neurosurgery, Chettinad Superspeciality Hospital, Chettinad Health City, Kelambakkam, Chennai, Tamil Nadu, India
2 Department of Pathology, Chettinad Superspeciality Hospital, Chettinad Health City, Kelambakkam, Chennai, Tamil Nadu, India
|Date of Submission||05-Apr-2013|
|Date of Decision||14-Apr-2013|
|Date of Acceptance||30-May-2013|
|Date of Web Publication||16-Jul-2013|
V G Ramesh
Department of Neurosurgery, Chettinad Superspeciality Hospital, Chettinad Health City, Kelambakkam, Chennai - 603 103, Tamil Nadu
Source of Support: None, Conflict of Interest: None
Ependymomas are common in intramedullary location and extradural location of the spinal cord is very rare. A few cases in the lumbosacral region have been reported. This report presents a cervical dumb-bell ependymoma with a small intraspinal extradural component and a large extraspinal component in the posterior triangle of the neck. The tumor was excised in two stages. This is probably the first such case report in the cervical region in the world literature. Possible histogenesis of ependymoma in this location is also discussed.
Keywords: Cervical extradural and extraspinal ependymoma, dumb-bell ependymoma, extradural ependymoma, extraspinal ependymoma
|How to cite this article:|
Ramesh V G, Karthikeyan K V, Rao K R, Balasubramanian C. Cervical extradural and extraspinal ependymoma mimicking dumb-bell schwannoma: An unusual tumor. Neurol India 2013;61:303-5
|How to cite this URL:|
Ramesh V G, Karthikeyan K V, Rao K R, Balasubramanian C. Cervical extradural and extraspinal ependymoma mimicking dumb-bell schwannoma: An unusual tumor. Neurol India [serial online] 2013 [cited 2019 Oct 22];61:303-5. Available from: http://www.neurologyindia.com/text.asp?2013/61/3/303/115073
| » Introduction|| |
Ependymomas constitute 60% of all intraspinal glial tumors.  They occur in the intramedullary location and in the filum terminale and arise from the ependymal lining of spinal central canal and ependymal remnants in the filum terminale. Extradural occurrence of ependymomas are very unusual and have been confined mainly to the sacral region. ,, Until date there has been no report of a intraspinal extradural and extraspinal ependymoma presenting as a dumb-bell tumor at the cervical level. Probably this is first such report in the world literature.
| » Case Report|| |
A 24-year-old gentleman presented with gradually increasing swelling on the left side of neck of 2 years duration. He developed stiffness of all the four limbs with numbness 2 weeks before this admission. Neurological examination showed mild spastic quadriparesis with brisk deep tendon reflexes and positive Babinski response bilaterally and mild sensory impairment below C 4 dermatome on both sides. There was large firm 8 cm × 8 cm swelling in the posterior triangle of the neck on the left side. Magnetic resonance imaging (MRI) showed a large soft-tissue mass in the posterior triangle of the neck on the left side, which was bilobed, with intraspinal extension in the extradural plane at C 3 C 4 levels, with severe compression of the spinal cord. MRI was suggestive of a dumb-bell schwannoma [Figure 1] and [Figure 2].
|Figure 1: Coronal T2 magnetic resonance imaging of the neck showing the intraspinal and extraspinal component of the tumor|
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|Figure 2: Axial T2 magnetic resonance imaging of the neck at the level of C3 showing the intraspinal and extraspinal component of the tumor mimicking dumb‑bell schwannoma|
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A two-stage surgery was planned. In the first stage, a cervical laminectomy and excision of the intraspinal extradural component of the tumor was performed. In the second stage, after 2 weeks, the extraspinal component in the posterior triangle of the neck was excised completely. It had a bi-lobar extension between the scalene muscles. The tumor was very firm, vascular with extension through the C 3-4 intervertebral foramen on the left side. Histopathology showed features of ependymoma with perivascular pseudorosettes [Figure 3]. Immunohistochemical staining with glial fibrillary acidic protein (GFAP) showed focal cytoplasmic positivity. Epithelial membrane antigen (EMA) staining showed diffuse membrane positivity. S-100 staining showed diffuse nuclear and cytoplasmic positivity. This confirmed the diagnosis of ependymoma [Figure 3], [Figure 4], [Figure 5] and [Figure 6]. Patient made an uneventful post-operative recovery and is undergoing radiotherapy.
|Figure 3: Histopathology picture (H and E, ×100) showing tumor cells with perivascular pseudorosettes|
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|Figure 4: Glial fibrillary acidic protein staining showing focal cytoplasmic positivity|
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|Figure 5: Epithelial membrane antigen staining showing diffuse membrane positivity|
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|Figure 6: S‑100 staining showing diffuse nuclear and cytoplasmic positivity|
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| » Discussion|| |
The first case of primary extraspinal ependymoma was reported by Mallory in 1902.  Following this report, there have been several reports of extraspinal ependymoma in the sacral and sacrococcygeal region. Akpolat et al., reported a case of sacrococcygeal extraspinal ependymoma and reviewed 22 cases reported till then.  The first report of myxopapillary extradural ependymoma with extraspinal extension in presacral region from India was by Magu et al.  De Bonis et al., reported primary extramedullary ependymoma of the thoracic spine, mimicking a schwannoma.  Intra-abdominal ependymoma presenting as an abdominal mass was reported by Mogler et al.  Wang et al., reported ectopic ependymoma in the soft-tissue of the neck.  However, there has been no report of intraspinal extradural and extraspinal ependymoma mimicking a dumb-bell tumor at the cervical level until date.
Ependymomas are glial tumors of ependymal origin. They are classified as subependymoma (World Health Organization (WHO) grade I), myxopapillary ependymoma (WHO grade I), ependymoma (WHO grade II) and anaplastic ependymoma (WHO grade III). Microscopic features of ependymoma include cells, which are epitheloid cuboidal to columnar cells with arrangements around a blood vessel (perivascular pseudorosettes). Sometimes true rosette arrangements and ependymal canal may be seen. Immunohistochemically, they show positivity for S-100, GFAP and vimentin. A few of the ependymomas show punctuate positivity for EMA.
Ependymal cells are usually found lining the ventricles in the brain and central canal of the spinal cord with some extension into the filum terminale. They are not usually found in other sites. Hence, ependymomas are common in the intraventricular compartment of the brain, intramedullary compartment of the spinal cord and cauda equina. The origin of ependymomas in the extradural and extraspinal regions is intriguing and subject to speculation. Aktuð et al., while reporting a case of sacrococcygeal extraspinal ependymomas considered it to be a form of monophasic teratomas occurring in the sacrococcygeal region.  Bale studied 15 infants and showed islands of ependymal cells in the post-coccygeal region unconnected with cauda equina in 10 of them.  Magu et al., consider sacrococcygeal extraspinal ependymoma to arise from coccygeal medullary vestige and ependymal lined cavity in caudal portion of neural tube that lies beneath the skin of post-natal pit.  Some others consider the ependymal rests as heterotopias that occur as a result of incomplete closure of the neural arch. It is a known fact that glial migration occurs from the central nervous system along the axons, to the peripheral nervous system during embryogenesis. Ependymal cells are also thought to arise from post-mitotic radial glial cells.  We postulate that ependymoma in the extradural and extraspinal compartment as in our case could arise from ependymal cell rests, which could have migrated during embryogenesis along the axons to the peripheral nervous system.
| » References|| |
|1.||Chason JL. Nervous system. In: Kissane JM, editor. Anderson's Pathology. St. Louis: CV Mosby Company; 1985. p. 1918. |
|2.||Mallory FB. Three gliomata of ependymal origin; two in the fourth ventricle, one subcutaneous over the coccyx. J Med Res 1902;8:1-10. |
|3.||Akpolat N, Bozlak N, Kazez A, Köseoðullari AA. Sacrococcygeal extraspinal ependymoma: A case report. Turk J Pediatr 2003;45:276-9. |
|4.||Magu S, Jaswal TS, Mishra DS, Magu NK, Popli P, Sharma N. Extradural ependymoma with extraspinal extension: Letter to editor. Neurol India 2002;50:223-5. |
|5.||De Bonis P, Montano N, Cioni B, Colosimo C, Lauriola L, Papacci F, et al. Primary extramedullary extradural ependymoma of the thoracic spine mimicking a schwannoma. J Neurol Neurosurg Psychiatry 2009;80:579-81. |
|6.||Mogler C, Kohlhof P, Penzel R, Grenacher L, Haag GM, Schirmacher P, et al. A primary malignant ependymoma of the abdominal cavity: A case report and review of the literature. Virchows Arch 2009;454:475-8. |
|7.||Wang Z, Huang G, Yan P, Liang R, Wang J, Yan Q, et al. Ectopic cervical anaplastic ependymoma. Pathol Int 2005;55:781-4. |
|8.||Aktuð T, Hakgüder G, Sarioðlu S, Akgür FM, Olguner M, Pabuçcuoðlu U. Sacrococcygeal extraspinal ependymomas: The role of coccygectomy. J Pediatr Surg 2000;35:515-8. |
|9.||Bale PM. Ependymal rests and subcutaneous sacrococcygeal ependymoma. Pathology 1980;12:237-43. |
|10.||Spassky N, Merkle FT, Flames N, Tramontin AD, García-Verdugo JM, Alvarez-Buylla A. Adult ependymal cells are postmitotic and are derived from radial glial cells during embryogenesis. J Neurosci 2005;25:10-8. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
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