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Year : 2015  |  Volume : 11  |  Issue : 4  |  Page : 1038

Unusual presentations of intracranial meningiomas: Report of two cases and review of the literature

1 Department of Otolaryngology, Head and Neck Surgery, Maulana Azad Medical College and Lok Nayak Hospital, New Delhi, India
2 Department of Histopathology, Rajiv Gandhi Cancer Institute and Research Centre, Rohini, New Delhi, India

Date of Web Publication15-Feb-2016

Correspondence Address:
Jatin Sundersham Gandhi
Department of Pathology, Rajiv Gandhi Cancer Institute and Reseach Centre, Sector V, Rohini, New Delhi - 110 085
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.146133

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 > Abstract 

Meningiomas at extracranial sites are uncommon clinical presentations. They may present in the form of benign, slow.growing masses or may exhibit aggressive malignant behavior. We report two cases of intracranial meningiomas presenting at extracranial sites that are, at the sinonasal tract/external auditory canal and as a neck mass. The clinical presentations, histopathological features and appropriate management are discussed.

Keywords: Extracranial extension, meningioma, paranasal sinus tumors, parapharyngeal tumor

How to cite this article:
Dhingra S, Gandhi JS, Gupta D. Unusual presentations of intracranial meningiomas: Report of two cases and review of the literature. J Can Res Ther 2015;11:1038

How to cite this URL:
Dhingra S, Gandhi JS, Gupta D. Unusual presentations of intracranial meningiomas: Report of two cases and review of the literature. J Can Res Ther [serial online] 2015 [cited 2020 May 31];11:1038. Available from: http://www.cancerjournal.net/text.asp?2015/11/4/1038/146133

 > Introduction Top

The extracranial presentation of meningiomas is extremely rare. Only in 20% cases, intracranial meningiomas may progress to extracranial sites,[1] whereas primary occurrence in extracranial sites is far less common, the incidence being only 1-2%.[2] Various sites of extracranial presentations have been implicated such as the orbit, the external surface of calvarium, the neck, parapharyngeal space, nose, and paranasal sinuses and the temporal bone.[3]

We report two unique cases of meningiomas, one with the extension into the parapharyngeal and posterior cervical space and the other with the extension into the paranasal sinuses and the temporal bone with appropriate discussion and management.

 > Case Reports Top

Case report 1

A 22-year-old female patient presented with the complaints of a gradually progressive painful swelling in the right upper part of the neck since last 7 months with associated muffling of voice and difficulty in deglutition for the last 4 months. The swelling had extended to the posterior part of the scalp and the nape of the neck in the last 2 months. Physical examination of the neck revealed approximately 6 × 7 cm swelling, firm in consistency with a bulge in the oral cavity, pushing the tonsil to the left side [Figure 1]a. There was ipsilateral paralysis of the 9th, 10th, and 12th cranial nerves (CNs). Contrast-enhanced computed tomographic scan (CT) revealed 7.8 cm × 6.3 cm, homogenously enhancing soft tissue mass in the right posterior cervical space, displacing the parapharyngeal space and the carotid space anteriorly and infiltrating into the right parotid space. Superiorly the mass was causing destruction of the right temporal and occipital bones with presence of extra-axial soft tissue in the posterior fossa, along the right cerebellar hemisphere [Figure 1]b. The continuity of intracranial and parapharyngeal tumors was clearly visualized on coronal and sagittal reconstruction [Figure 1]c.
Figure 1: (a) 6 cm × 7 cm swelling in the right upper part of neck, (b) contrast-enhancing computed tomographic scan showing homogenously enhancing mass occupying right posterior cervical space, destroying the temporal and occipital bones, (c) coronal reconstruction showing continuity of the tumor with intracranial space

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Biopsy examination showed sheets of cells with a swirling pattern and had round to oval vesicular nuclei displaying moderate nuclear atypia and intranuclear inclusions. Few of the clusters showed psammoma bodies. The mitosis was high 6-8/10 hpf [Figure 2]. These cells on immunohistochemistry (IHC) [Table 1] were strongly positive for epithelial membrane antigen (EMA), vimentin with a weak expression for progesterone receptors (PgR) [Figure 3]. The MIB-1 proliferation index was approximately 12-14% that correlated with higher mitotic rate on light microscopy. In view of the histomorphology and IHC a diagnosis of atypical meningioma was offered.
Figure 2: (a) Section shows a meningothelial tumor with whorls and psammoma bodies (H and E; ×40), (b) section shows meningothelial cells arranged in whorls with few cells exhibiting mitosis (yellow arrow) (H and E; ×200), (c) section shows few of the cells with anaplastic nuclei having intranuclear inclusions (black arrow) (H and E; ×400)

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Table 1: List of Immunohistochemistry markers applied with their clones and dilutions

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Figure 3: (a) Tumor cells highlight strong immunoreactivity for epithelial membrane antigen (DAB; ×40), (b) tumor cells highlight strong immunoreactivity for vimentin (DAB; ×100), (c) tumor cells expressing a high Ki-67 index (approximately 12-14%) (DAB; ×200), (d) tumor cells expressing weak nuclear immunopositivity for progesterone receptors (DAB; ×200)

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Surgical excision of the tumor was planned; however, the patient and her family refused to permit the operation.

Case report 2

A 50-year-old female patient presented to the clinic with complaints of decreased hearing in the right ear, aural fullness with the associated diminution of vision in the right nasal field for the last 4 years. Otoscopic examination revealed a pinkish smooth polypoidal mass in the external auditory canal on the right side. A CT scan of the temporal bone revealed evidence of a soft tissue mass in the right external auditory canal with opacification of bilateral middle ear cavities and the mastoid antrum [Figure 4]a. Nasal endoscopic examination revealed evidence of polypoidal mass arising from the right spheno-ethmoidal recess. Magnetic resonance imaging of the brain was performed and revealed evidence of a small approximately 1.5 × 1.0 cm lobulated the lesion in the region of the right cerebello-pontine angle cistern, which showed enhancement on postcontrast scans. There was an extension of tumor into the sphenoid sinus with expansion and thinning of the bones [Figure 4]b. The biopsy from the auricular and the nasal mass was performed, both of which showed similar histomorphology of a tumor comprising of plump to spindle meningothelial cells in whorls and fascicles having vesicular nuclei. Occasional intranuclear inclusions and psammoma bodies were identified [Figure 4]c and [Figure 4]d. On IHC [Table 1] the tumor cells expressed positivity for EMA, vimentin and PgR. MIB-1 proliferation index was 1-2%. Overall histomorphology and IHC were in keeping with a meningothelial meningioma (grade 1).
Figure 4: (a) High resolution computed tomography temporal bone showing soft tissue mass in the right external auditory canal, (b) contrast-enhanced magnetic resonance imaging showing enhancing mass in the sphenoid sinus with enhancement of bilateral cavernous sinuses. Note diffuse plaque like thickening of meningeal layers, (c) section showing meningothelial cells arranged in whorls (H and E; ×40), (d) occasional cell showing intranuclear inclusions (H and E; ×400)

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Endoscopic debridement of the sphenoid mass was done to relieve the pressure on the optic nerves. The patient was referred for radiotherapy for the residual intracranial lesion.

 > Discussion Top

Meningiomas are the most common primary tumors of the central nervous system. They are thought to develop from the arachnoid cells in the core of the arachnoid villi that project into the dural sinuses. Although most meningiomas are limited to the intracranial space, few may extend extracranially. Various sites have been implicated such as the orbit, external surface of the calvarium, the skin, brachial plexus, neck, submandibular gland, inner canthus of eye, parapharyngeal space, temporal bone, nasopharynx, nose and paranasal sinuses.[3]

Our first case describes a patient with an intracranial meningioma with direct extension into the parapharyngeal space. The differential diagnosis of such a patient presenting with a neck mass usually includes salivary gland tumors, schwannomas and paragangliomas. The clinical presentation of meningiomas may be extremely subtle, and the patient may not present with symptoms unless the tumor has reached a significant size as was seen in our case. It was interesting to note that this tumor caused destruction of the temporal bone and occipital bone with lower CN involvement, raising suspicion of a malignant lesion which correlated well with an atypical meningioma.

Our second case describes a patient with meningioma in the sphenoid sinus with involvement of CP angle cistern and middle ear cavity. Meningiomas involving the temporal bone may originate either from arachnoid cell nests present within the temporal bone, or more frequently originate from arachnoid cell nests of posterior or middle cranial fossa which can extend along the path of least resistance and involve the temporal bone secondarily. This pathway may be along natural dehiscence in the tegmen tympani, the sulci of greater and lesser superficial petrosal nerves or the internal auditory canal. Arachnoid cell clusters travel along these CNs and blood vessels to give rise to meningiomas in ectopic locations.[4]

Controversy still exists around the exact site of origin of meningiomas within the sinus tract. These appear as polypoidal masses and may arise from the septum or the nasal bones.[5] These tumors may erode the bones of the sinuses and involve the surrounding soft tissues and the skull base. The differential diagnosis includes carcinoma, paraganglioma, melanoma and schwannomas. In general, the prognosis of meningiomas within the sinonasal tract is excellent. Surgical extirpation is the treatment of choice as they are generally considered to be radio resistant.[6] However, when recurrences develop, they, usually, arise in the same anatomical site as the primary tumor and represent residual disease.

 > Acknowledgment Top

Dr. Gurudutt Gupta, Department of Histopathology, Rajiv Gandhi Cancer Institute and Research Centre, Rohini, Delhi - 110 085, India.

 > References Top

Rietz DR, Ford CN, Kurtycz DF, Brandenburg JH, Hafez GR. Significance of apparent intratympanic meningiomas. Laryngoscope 1983;93:1397-404.  Back to cited text no. 1
Farr HW, Gray GF Jr, Vrana M, Panio M. Extracranial meningioma. J Surg Oncol 1973;5:411-20.  Back to cited text no. 2
Friedman CD, Costantino PD, Teitelbaum B, Berktold RE, Sisson GA Sr. Primary extracranial meningiomas of the head and neck. Laryngoscope 1990;100:41-8.  Back to cited text no. 3
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. 4
Pathak PN. Ectopic meningioma of the nose. J Laryngol Otol 1969;83:1115-8.  Back to cited text no. 5
Rubinstein LJ. Tumors of the central nervous system. Washington, DC: Armed Forces Institute of Pathology; 1970. p. 169-20.  Back to cited text no. 6


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

  [Table 1]


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