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 Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 2  |  Issue : 4  |  Page : 370-372

Primary extracranial sino-nasal tract meningioma in a child: A rare lesion with diagnostic pitfall


1 Department of Pathology, Bankura Sammilani Medical College, Bankura, West Bengal, India
2 Department of Pathology, Medical College, Kolkata, West Bengal, India
3 Department of Pathology, North Bengal Medical College, Darjeeling, West Bengal, India
4 Department of Pathology, School of Tropical Medicine, Kolkata, West Bengal, India

Date of Web Publication18-Sep-2015

Correspondence Address:
Adhikari Anindya
Basudevpur Village, PO-Banipur, P. S - Sankrail, Howrah - 711 101, West Bengal
India
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Source of Support: Department of Pathology, Medical College, Kolkata, West Bengal, India., Conflict of Interest: There are no conflicts of interest.


DOI: 10.4103/2348-3334.165740

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  Abstract 

Extracranial meningiomas are rare in children and are frequently misdiagnosed. We report a case of primary extracranial meningioma of the nasal cavity in a 2-year-old child who presented with a recurrent, gradually increasing swelling over the nasal bridge. Computed tomography scan revealed a mass protruding through a rent in the nasal bridge. Fine-needle aspiration cytology showed features suggestive of a benign mesenchymal neoplasm. Histologically, a diagnosis of meningothelial meningioma was made which was confirmed by immunohistochemistry. The neoplastic cells were strongly positive for vimentin and epithelial membrane antigen, weakly positive for S-100 and negative for neuron specific enolase, and glial fibrillary acidic protein. The case is documented for the rarity of its occurrence and varied prognosis.

Keywords: Child, extracranial meningioma, immunohistochemistry


How to cite this article:
Anindya A, Parul J, Kumar MP, Nandita B. Primary extracranial sino-nasal tract meningioma in a child: A rare lesion with diagnostic pitfall. CHRISMED J Health Res 2015;2:370-2

How to cite this URL:
Anindya A, Parul J, Kumar MP, Nandita B. Primary extracranial sino-nasal tract meningioma in a child: A rare lesion with diagnostic pitfall. CHRISMED J Health Res [serial online] 2015 [cited 2019 Nov 14];2:370-2. Available from: http://www.cjhr.org/text.asp?2015/2/4/370/165740


  Introduction Top


Meningiomas represent 10–15% of primary intracranial tumors. The most common localizations of extracranial meningiomas are the skull bones, nose, orbit, paranasal sinuses, middle ear, neck, skin, etc.[1],[2] These are rare in children and their treatment and prognosis differs from other lesions in those sites. In children, they may be associated with Von Recklinghausen neurofibromatosis (Type-I). Meningiomas pose a diagnostic problem for the surgeon and surgical pathologist by reason of extracranial manifestation and the varying histological patterns seen in these tumors. However, due to many clinical, topographical, radiological, and surgical factors, the histology is not solely decisive for the prognosis of the meningiomas. To the best of our knowledge, literature has <50 cases of primary meningiomas of paranasal sinuses in children.


  Case Report Top


A 2-year-old girl presented with complaints of gradually increasing swelling over nasal bridge and features of nasal obstruction. She had a past history of similar swelling over the nasal bridge at 6 months of age which was excised [Figure 1]a. Previous diagnosis on biopsy was hamartoma (slides were not available). An X-ray of paranasal sinuses showed opacity in the posterior nasal cavity. Computed tomography (CT) scan revealed a mass in the posterior nasal cavity with protrusion through the nasal bridge [Figure 1]b. X-ray chest and ultrasonography of whole abdomen were within normal limits. Similar family history could not be elicited. Fine-needle aspiration cytology (FNAC) from the swelling showed round to oval cells with pale cytoplasm, indistinct cell borders, ovoid nucleus with finely granular chromatin [Figure 1]c. At places, they were arranged in a syncytial pattern. Focal areas showed clustering and whorl like the arrangement of cells [Figure 1]d. No calcification was seen. The mass was excised surgically with combined access through external and endonasal ethmoidectomy. On gross examination, it was grayish white, firm in consistency, measuring 1.5 cm × 1.5 cm × 1 cm [Figure 2]a. Microscopically, the tumor showed sheets of neoplastic cells with indistinct cell borders; round to oval, vesicular nuclei, inconspicuous nucleoli [Figure 2]b. These were focally arranged in whorls and had nuclear pseudo inclusions. Necrosis or mitoses were absent. On immunohistochemistry (IHC), the neoplastic cells were strongly positive for vimentin [Figure 2]c and epithelial membrane antigen (EMA) [Figure 2]d; weakly positive for S-100 and negative for neuron specific enolase and glial fibrillary acidic protein. A final diagnosis of meningothelial meningioma (World Health Organization [WHO] Grade I) was made. The child was disease free till 8 months of follow-up.
Figure 1: (a) Clinical photograph showing tumor over nasal bridge. (b) Picture of computed tomography scan showing the tumor. (c) Picture of fine needle aspiration cytology smear (LG, ×100). (d) Picture of fine needle aspiration cytology smear (LG, ×400)

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Figure 2: (a) Gross picture of the tumor. (b) Picture of histopathology (H and E, ×400). (c) Picture of immunohistochemistry showing epithelial membrane antigen positivity (×100). (d) Picture of immunohistochemistry showing vimentin positivity (×400)

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  Discussion Top


Meningiomas are common tumors of central nervous system and derived from arachnoid cells. Primary extracranial meningiomas are rare tumors, but have been reported at sites from head and neck region to lung to foot.[1],[2],[3],[4] Occurrence of primary extracranial meningiomas is probably related to the transformation of embryonic arachnoid cell remnants of ectopic meningocytes derived from pluripotent mesenchymal cells. They may also occur in rare cases of traumatic event or intracranial hypertension that dislocates arachnoid cells. They can also metastasize to a different site from a benign or malignant. A genuine extracranial primary meningioma is to be confirmed after a CT scan or magnetic resonance imaging (MRI), to rule out intracranial mass or any underlying bony erosion of the skull base and proper history of the patient.[1] The MRI supplies more precise information related to the extension and tumoral invasion of adjacent structures than CT scan.[5]

FNAC of the lesion can be deceptively mistaken and final diagnosis is usually made on the basis of histological examination of the excised mass. The diagnosis becomes complicated as meningiomas are notorious for their varied cytohistological presentations. The WHO histopathologically classifies meningiomas as Grade I: Meningothelial, fibrous (fibroblastic), transitional (mixed), psammomatous, angiomatous, microcystic, secretory, lymphoplasmacyte-rich, metaplastic, Grade II: Atypical, clear cell, chordoid meningioma, and Grade III: Rhabdoid meningioma, papillary meningioma, anaplastic (malignant) meningioma.[6] Considering the site and the age in our case, the differential diagnosis included dermoid cyst, teratoma, hemangioma, mucocele, olfactory neuroblastoma, and hamartoma.

IHC of these tumors shows strong positivity with vimentin and EMA, as seen in this case. Other markers which may be inconsistently positive include S-100, cytokeratin, E-cadherin. Ultrastructurally, both intra- and extra-cranial meningiomas have abundant intermediate filaments (vimentin), complex interdigitating cellular processes, and joined by desmosomes.[5],[6]

Meningiomas are usually slow growing and have a good prognosis with younger age and complete resection.[5],[7] Recurrence and possible fatal outcome are mainly related to incomplete resection of the tumor. Hence, awareness of its occurrence at an unusual site along with imaging, microsurgery, and histopathological examination is mandatory for proper management and survival of the patient.

Financial support and sponsorship

Department of Pathology, Medical College, Kolkata, West Bengal, India.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Sharma JK, Pippal SK, Sethi Y. A rare case of primary nasoethmoidal meningioma. Indian J Otolaryngol Head Neck Surg 2006;58:101-3.  Back to cited text no. 1
    
2.
Thompson LD, Gyure KA. Extracranial sinonasal tract meningiomas: A clinicopathologic study of 30 cases with a review of the literature. Am J Surg Pathol 2000;24:640-50.  Back to cited text no. 2
    
3.
Tomaru U, Hasegawa T, Hasegawa F, Kito M, Hirose T, Shimoda T. Primary extracranial meningioma of the foot: A case report. Jpn J Clin Oncol 2000;30:313-7.  Back to cited text no. 3
    
4.
Lockett L, Chiang V, Scully N. Primary pulmonary meningioma: Report of a case and review of the literature. Am J Surg Pathol 1997;21:453-60.  Back to cited text no. 4
    
5.
Ferraz-Filho JR, Floriano VH, Felipe LF, Rocha-Filho JA. Clinical-radiological aspects of primary extracranial meningioma of the ethmoid sinus in a child. Arq Neuropsiquiatr 2008;66:274-5.  Back to cited text no. 5
    
6.
Perry A, Louis DN, Scheithauer BW, Budka H, von Deimling A. Meningiomas. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, editors. WHO Classification of Tumours of the Central Nervous System. 4th ed. Lyon: IAR Press; 2007. p. 164-72.  Back to cited text no. 6
    
7.
Rushing EJ, Bouffard JP, McCall S, Olsen C, Mena H, Sandberg GD, et al. Primary extracranial meningiomas: An analysis of 146 cases. Head Neck Pathol 2009;3:116-30.  Back to cited text no. 7
    


    Figures

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