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Year : 2016  |  Volume : 59  |  Issue : 3  |  Page : 386-388
Primary intraosseous atypical inflammatory meningioma presenting as a lytic skull lesion: Case report with review of literature

1 Department of Pathology, Maulana Azad Medical College, New Delhi, India
2 Department of Neurosurgery, Lok Nayak Hospital, Maulana Azad Medical College, New Delhi, India

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Date of Web Publication10-Aug-2016


Primary extradural meningiomas of the skull comprise 1% of all meningiomas, and lytic skull meningiomas are still rarer and are said to be more aggressive. We present a case of 38-year-old male with an extradural tumor which on histopathological examination showed features of inflammatory atypical meningioma (WHO Grade II). The intense inflammatory nature of osteolytic primary intraosseous meningioma has not been reported before. This entity deserves special mention because of the need for adjuvant therapy and proper follow-up.

Keywords: Atypical, intraosseous, meningioma

How to cite this article:
Bohara S, Agarwal S, Khurana N, Pandey P N. Primary intraosseous atypical inflammatory meningioma presenting as a lytic skull lesion: Case report with review of literature. Indian J Pathol Microbiol 2016;59:386-8

How to cite this URL:
Bohara S, Agarwal S, Khurana N, Pandey P N. Primary intraosseous atypical inflammatory meningioma presenting as a lytic skull lesion: Case report with review of literature. Indian J Pathol Microbiol [serial online] 2016 [cited 2022 Sep 25];59:386-8. Available from:

   Introduction Top

Primary meningiomas of the skull are an uncommon entity often appearing as hard osteoblastic tumors which show hyperdense areas of the calvarial bones in X-ray studies. Lytic skull meningiomas are still rarer.[1] It has been emphasized that meningiomas present with osteolysis are more aggressive than others.[2] There are only rare case reports of atypical primary extradural meningioma (PEM) and that with inflammatory change in such a location has not been described as far as the best of our knowledge.

   Case Report Top

We present a case of a 38-year-old male who presented with a scalp swelling for 1 year. Magnetic resonance imaging (MRI) showed altered signal intensity high parietal scalp with the presence of bony defect [Figure 1]a. Contrast-enhanced computed tomography of the head showed a bony defect although the brain parenchyma was uninvolved. Peroperatively, a firm fibrous and vascular tumor was noted which was largely extradural, only partly adhered to the dura in the midline along with a bony defect. The dura was opened and the involved portion was completely removed. The tumor was partially abutting the brain surface, and it was removed en bloc followed by cranioplasty.
Figure 1: (a) T1-weighted magnetic resonance imaging skull showing altered signal intensity high parietal scalp with the presence of bony defect, (b) scanner view showing bony trabeculae, marrow spaces replaced by sheets of tumor cells and few marrow spaces show necrosis (H and E, ×40), (c) sheets of atypical meningothelial cells showing prominent nucleoli (arrow) (H and E, ×400), (d) admixed inflammatory cells comprised histiocytes, lymphocytes and occasional plasma cells (H and E, ×100)

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The tumor tissue along with the involved bony tissue was excised and sent to the pathology laboratory for histopathological examination which showed fragments of mature bone, whereby some of the marrow spaces were replaced by a cellular tumor composed of oval- to spindle-shaped cells arranged in sheets [Figure 1]b. The cells showed mild to moderate degree of pleomorphism with oval nuclei, prominent nucleoli, and eosinophilic cytoplasm with indistinct cytoplasmic borders [Figure 1]c. The mitosis was up to 4–5/10 high power field with focal zones of necrosis [Figure 1]b. The bony bits included showed infiltration of marrow spaces by tumor cells. The meningothelial cells were admixed with the histiocytes, lymphocytes, along with few eosinophils and plasma cells [Figure 1]d. The tumor cells were positive for epithelial membrane antigen (EMA) and vimentin while the admixed histiocytes were positive for CD68 [Figure 2]. No brain tissue could be identified in any of the microscopic sections of the excised tumor tissue provided. The features were of an intraosseous atypical meningioma of an inflammatory nature (WHO Grade II).
Figure 2: Immunohistochemistry (a) epithelial membrane antigen, (b) vimentin, (c) CD68 positive histiocytes (immunohistochemistry: Avidin-biotin method with diaminobenzidine chromogen and hematoxylin counterstain, ×400)

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Postoperative recovery was uneventful and follow-up MRI 6 months after the surgery did not show any evidence of recurrence.

   Discussion Top

True primary intraosseous meningiomas are described as those lesions that do not involve the underlying dura.[3] Primary meningiomas of the skull without any involvement of the dura and intracranial cavity are rare entities comprising about 2% of all meningiomas. PEMs of the skull convexity comprise 52.5% of all the PEMs.[4] Of a rarer prevalence, the osteolytic intraosseous meningiomas are not radiologically suspected at the first instance due to its radiological picture. It has been suggested that only a meningioma originating from the outer layer of dura can evoke osteoclastic activity in the skull by causing mechanical pressure and erosion.[5]

The clinical presentation depends on the size and location of the tumor. Skull convexity PEMs present as scalp masses that grow slowly, in relation to cranial sutures, mostly in periorbital or frontoparietal region.[4]

It has been suggested that osteolysis in intracranial meningiomas correlates with its aggressive nature; however, others are of opinion that invasion to the extracranial portion and osteolysis might be related to matrix metalloproteinase-2 expression.[6]

Microscopically, PEMs demonstrate the pathognomic traits of intradural meningiomas.[4] The most common histopathological subtype is meningotheliomatous meningioma, occurring in about 60% of cases. Similar to intradural meningiomas, PEMs typically show positive immunostaining for vimentin and EMA, and they are occasionally positive for S-100.[4]

The inflammatory cell reaction to meningioma has been proposed to be a mechanism of host resistance. However, the relationship between inflammatory lesions and meningiomas remains uncertain.[7] Langerhans cell histiocytosis may also present as dural based lesion with the presence of lymphocytes infiltrating a fibrous stromal background and S-100 protein positive histiocytes; however, these histiocytes have indented/reniform nucleus, stain positive for CD1a and contain Birbeck granules ultrastructurally [8] which was not present in this case.

Surgical treatment of atypical or malignant PEMs is not consistently described in the literature. When possible, wide en bloc resection including a 1-cm negative margin is recommended.[4]

The use of radiotherapy as an adjuvant after Simpson Grade III–IV resections (subtotal resections) is frequently recommended. Incompletely resected or recurrent WHO II and III tumors, however, should be considered for postoperative radiotherapy, using doses as 13–20 Gy for stereotactic radiosurgery and 45–60 Gy for stereotactic radiotherapy.[9],[10]

Chemotherapy should be reserved for unresectable, growing WHO I and all WHO II and III PEMs. However, a single chemotherapeutic agent cannot be recommended.[4]

The postoperative imaging with MRI should be done within 48 h to establish extent of resection. Tumor recurrence or progression can be monitored with yearly MRI for 5 years, then every 2 years for benign PEMs.[4]

We present a rare case of atypical inflammatory meningioma in an intraosseous location. The treatment protocol includes wide resection of the tumor along with radiotherapy. The use of chemotherapy has been encouraged in the literature along with long-term follow-up.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Elder JB, Atkinson R, Zee CS, Chen TC. Primary intraosseous meningioma. Neurosurg Focus 2007;23:E13.  Back to cited text no. 1
Russel DS, Rubinstein LJ. Pathology of Tumors of the Nervous System. 2nd ed. Baltimore: Williams & Wilkins; 1989. p. 449-532.  Back to cited text no. 2
Todd S, Bette K, Kleinschmidt DM. Primary intraosseous meningioma: A case report. J Neurosurg 1995;83:912-5.  Back to cited text no. 3
Mattox A, Hughes B, Oleson J, Reardon D, McLendon R, Adamson C. Treatment recommendations for primary extradural meningiomas. Cancer 2011;117:24-38.  Back to cited text no. 4
Baek JU, Cho YD, Yoo JC. An osteolytic meningioma en plaque of the sphenoid ridge. J Korean Neurosurg Soc 2008;43:34-6.  Back to cited text no. 5
Moon HS, Jung S, Jung TY, Cao VT, Moon KS, Kim IY. Possible role of matrix metalloproteinase in osteolytic intracranial meningiomas. J Korean Neurosurg Soc 2010;47:11-6.  Back to cited text no. 6
Pandey R, Khurana P, Sethi S, Suneja S, Shankar J. Lymphoplasmacyte-rich meningioma mimicking tubercular meningitis: A case report. Internet J Radiol 2008;9.  Back to cited text no. 7
Wu M, Anderson AE, Kahn LB. A report of intracranial Rosai-Dorfman disease with literature review. Ann Diagn Pathol 2001;5:96-102.  Back to cited text no. 8
Huszar M, Fanburg JC, Dickersin GR, Kirshner JJ, Rosenberg AE. Retroperitoneal malignant meningioma. A light microscopic, immunohistochemical, and ultrastructural study. Am J Surg Pathol 1996;20:492-9.  Back to cited text no. 9
Higuchi M, Tsuji M, Fujimoto Y, Ikeda H. Spheno-orbital meningioma with unusual radiological features. Clin Neurol Neurosurg 1998;100:288-91.  Back to cited text no. 10

Correspondence Address:
Dr. Sangita Bohara
1/B, Martyr's Home, Choti Lal Kurti, Sadar Bazaar, Lucknow - 226 002, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.188131

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