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Year : 2012  |  Volume : 55  |  Issue : 2  |  Page : 262-264
Clear cell meningioma of the cauda equina in an adult


1 Department of Pathology, National Institute of Pathology, ICMR, Safdarjung Hospital Campus, New Delhi, India
2 Department of Neurosurgery, Safdarjung Hospital, New Delhi, India

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Date of Web Publication3-Jul-2012
 

How to cite this article:
Mallya V, Singh A, Sharma K. Clear cell meningioma of the cauda equina in an adult. Indian J Pathol Microbiol 2012;55:262-4

How to cite this URL:
Mallya V, Singh A, Sharma K. Clear cell meningioma of the cauda equina in an adult. Indian J Pathol Microbiol [serial online] 2012 [cited 2020 Feb 20];55:262-4. Available from: http://www.ijpmonline.org/text.asp?2012/55/2/262/97904


Sir,

Clear cell meningioma (CCM), WHO grade 2, is a rare histological variant of meningioma which is usually seen in young females and has a predilection for infratentorial region. We report a case of clear cell meningioma arising from the cauda equina in an adult female.

A 50-year-old female visited the department of neurosurgery with complaints of progressive weakness in both the lower limbs since three months. She also complained of urinary retention and constipation. On neurological examination, there was decreased power in both lower limbs. Magnetic resonance imaging (MRI) showed an isointense lesion on T1 and T2- weighted images that were homogeneously enhancing on contrast administration [Figure 1] and seen extending from the lower thoracic vertebrae till the sacral region causing osteolysis of the posterior elements of sacrum and the lower lumbar vertebrae involving the entire dural space. A presumptive clinical diagnosis of chordoma was entertained. A laminectomy was performed at S1 level and an intradural exposure showed an elliptical tumor that was sent for histopathological examination.
Figure 1: T2-W image of an MRI showing a homogeneously enhancing tumor at cauda equina

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The histopathology of the tumor showed polygonal cells with clear cytoplasm arranged in groups and sheets [Figure 2]a. There was no pleomorphism, vascular endothelial proliferation, mitosis, or necrosis. Psamomma body or whorl formation was rarely seen. The cytoplasm of these clear cells stained strongly with PAS stain [Figure 2]b and PAS positivity was diastase sensitive. Immunohistochemistry was performed and tumor cells were positively labeled with epithelial membrane antigen (EMA) [Figure 2]c and were negative for glial fibrillary acidic protein (GFAP), S100, cytokeratin, and synaptophysin. Proliferation marker Ki 67 labelling showed an index of 3.0% [Figure 2]d. A diagnosis of CCM was made.
Figure 2: Photomicrograph showing tumor cells with (a) clear cytoplasm (H&E, 200), (b) cytoplasmic PAS positivity (PAS, ×400), (c) EMA positivity (EMA, ×100), (d) nuclear Ki-67 positive cells (Ki-67, 400)

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CCM was described for the first time by Zorludemir et al,[1] in 1995 and since then about 50 cases have been published in literature. These constitute only about 0.2% of all meningiomas. [2] These occur mostly in the pediatric age group [3],[4],[5] and involve the cerebellopontine angle region in posterior fossa and intradurally in the spine. [4] They are associated with a high rate of recurrence and despite their bland histomorphology, can have an aggressive clinical course. [1],[4] The treatment of choice is complete excision, while adjuvant radiotherapy is reserved for recurrent CCM.

Histologically, CCM need to be distinguished from oligodendrogliomas which show a chickenwire pattern of blood vessels and are EMA negative. Similarly, extraventricular neurocytoma also falls into the differential diagnosis of CCM and can be excluded by positive staining with synaptophysin in neurocytomas. [6] Other mimics of CCM can be clear cell ependymoma especially in the spine and it can be excluded because of its GFAP positivity. Metastatic clear cell carcinomas, especially renal cell carcinoma also needs to be distinguished from CCM. Cerebellar hemangioblastomas can also be rarely confused with CCM because of their lipid-rich polygonal cells and need to be excluded by immunohistochemistry. MIB1 labeling index is variable and ranges from 3.3-25% and is usually a good indicator of recurrence and brain invasion. [1]

To conclude, a pathologist should be aware of this entity and proper use of immunohistochemistry can help to confirm the diagnosis of CCM. In the absence of proper diagnosis and hence subtotal excision, this tumor has propensity not only for recurrence and local spread but also metastasis to a distant site.

 
   References Top

1.Zorludemir S, Scheithauer BW, Hirose T, Van Houten C, Miller G, Meyer FB. Clear cell meningioma. A clinicopathological study of a potentially aggressive variant of meningioma. Am J Surg Pathol 1995;19:493-505.  Back to cited text no. 1
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2.Ko JK, Choi BK, Cho WH, Choi CH. Non-dura based intaspinal clear cell meningioma. J Korean Neurosurg Soc 2011;49:71-4.  Back to cited text no. 2
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3.Epstein NE, Drexler S, Schneider J. Clear cell meningioma of the cauda equina in an adult: case report and literature review. J Spinal Disord Tech 2005;18:539-43.  Back to cited text no. 3
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4.Dubois A, Sevely A, Boetto S, Delisle MB, Manelfe C. Clear-cell meningioma of the cauda equine. Neuroradiology 1998;40:743-7.  Back to cited text no. 4
    
5.Ranjan R, Sethuraman S. Supratentorial clear cell meningioma in a child: A rare tumor at unusual location. J Pediatr Neurosci 2010;5:141-3.  Back to cited text no. 5
[PUBMED]  Medknow Journal  
6.Pizzoni C, Sarandria C, Pierangeli E. Clear-cell meningioma of the anterior cranial fossa. Case report and review of the literature. J Neurosurg Sci 2009;53:113-7.  Back to cited text no. 6
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Correspondence Address:
Avninder Singh
213-Pocket B, Sukhdev Vihar, New Delhi- 110 025
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.97904

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