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|LETTER TO EDITOR
|Year : 2016 | Volume
| Issue : 2 | Page : 338-340
A primary intrapetrous osteolytic meningioma in a child: A rare report and review of literature
Damodar Rout1, KM Geetha Sharmi1, Rajeswaran Rangasami2, Preeti Priyadarshini2, Srinivas Rangan1, Ravi Kumar Arunachalam3
1 Department of Neurosurgery, Sri Ramachandra University, Chennai, Tamil Nadu, India
2 Department of Radiology, Sri Ramachandra University, Chennai, Tamil Nadu, India
3 Department of Ear Nose Throat, Sri Ramachandra University, Chennai, Tamil Nadu, India
|Date of Web Publication||3-Mar-2016|
Department of Neurosurgery, Sri Ramachandra University, Chennai, Tamil Nadu
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Rout D, Geetha Sharmi K M, Rangasami R, Priyadarshini P, Rangan S, Arunachalam RK. A primary intrapetrous osteolytic meningioma in a child: A rare report and review of literature. Neurol India 2016;64:338-40
|How to cite this URL:|
Rout D, Geetha Sharmi K M, Rangasami R, Priyadarshini P, Rangan S, Arunachalam RK. A primary intrapetrous osteolytic meningioma in a child: A rare report and review of literature. Neurol India [serial online] 2016 [cited 2019 Aug 25];64:338-40. Available from: http://www.neurologyindia.com/text.asp?2016/64/2/338/177613
We report a rare case of a primary intrapetrous osteolytic meningioma in a 5-year-old male child, with a review of the relevant literature. The child presented with left facial paresis and complete hearing loss on the left side. Computed tomography (CT) and magnetic resonance imaging (MRI) were suggestive of either a chondroma or an eosinophilic granuloma. However, the tumor biopsy following surgery, surprisingly, turned out to be a psammomatous meningioma.
Primary meningiomas arising outside the subdural compartment are termed as primary extradural meningiomas (PEMs). While bony invasion by an intracranial meningioma is a well-recognized entity, its primary origin from within a bone is a rare occurrence. This entity is called as primary intraosseous meningioma (PIM).
PEMs account for <2 % of all meningiomas. PIMs constitute only two-thirds of PEMs. An intraosseous meningioma presenting as a purely osteolytic lesion is not common. Such a lesion in an intrapetrous ,
location in a child has not yet been reported.
A 5-year-old male child, while undergoing a routine eye checkup for his refractive errors, was found to be having incomplete closure of the left eyelid, by the ophthalmologist. This had not been noticed by his parents anytime before. A few days later, his parents had a suspicion regarding his hearing ability. Pure-Tone Audiometry (PTA) and Brainstem Evoked Response Audiometry (BERA) revealed complete hearing loss on the left side. Hence, a computed tomographic (CT) scan of the temporal bones was performed, which revealed an expansile lytic lesion of the left petrous bone with a mild bulge into the cerebellopontine angle cistern [Figure 1]a,[Figure 1]b,[Figure 1]c,[Figure 1]d]. Following the abnormality observed on CT scan, his parents brought him to us for further management. He had no history of headache, seizures, giddiness, ear discharge, or trauma. His mother had gestational diabetes mellitus, and he was born using vacuum-assisted vaginal delivery.
|Figure 1: (a and b) Axial noncontrast CT shows a lesion (arrow) with calcification (broken arrow) in the left petrosal bone. (c and d) The corresponding axial CT scan images in the bone window shows an expansile lytic lesion within the left petrosal bone (arrow). The lesion is bordered posteriorly by a thin shell of bone (arrowhead). (e and f) T1- and T2-weighted axial MR images show a T1 hypointense lesion (arrow) within the left petrosal bone that appears mildly hyperintense on T2-weighted image (arrow). (g and h) Postcontrast fat-suppressed T1 axial images show homogeneous mild enhancement of the lesion (arrow)|
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On examination, he had a left facial paresis and complete hearing loss on the left side. Subsequently, magnetic resonance imaging (MRI) of the brain was performed [Figure 1]e,[Figure 1]f,[Figure 1]g,[Figure 1]h. The preoperative radiological differential diagnosis was either a chondroma or an eosinophilic granuloma.
The child underwent a left retromastoid partial petrosectomy and radical excision of the tumor. The frozen-section biopsy showed a spindle cell lesion with extensive calcification. The final histopathological report revealed the presence of a psammomatous meningioma [Figure 2].
|Figure 2: Psammomatous meningioma: Cellular spindle cell tumor with interspersed collagenous tissue. Psammoma bodies are also noted (HPE × 100)|
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Winkler, in 1904, first described a primary extradural meningioma. The first report of an entirely intraosseous osteolytic meningioma was by McWhorter in 1976. Although there are other reports of intraosseous/intrapetrous , meningiomas much before the availability of CT and MRI, the possibility of secondary petrous involvement by these lesions could not be excluded. The first case of a primary intraosseous meningioma with CT evidence was reported in 1996.
The age at presentation of a PEM is bimodal—a peak during the second decade and another peak between the fifth and seventh decades. The presence of a primary intraosseous meningioma in a child is a very rare entity. Kuzeyli et al., reported a PIM in a 6-year-old boy who had a progressively enlarging scalp mass since the age of 9 months. To our knowledge, our patient is the youngest of the patients presenting with a PIM reported in literature. However, the duration of symptoms remains questionable, as his parents were unaware of his illness. Male and female individuals are almost equally affected in PEM. However, a female preponderance is often detected in a PIM. In a review of 163 PEMs reported in literature, Mattox et al., found that skull base PEMs constitute only 3.8% of all PEMs.
The origin of PEM is controversial. There are only hypotheses regarding its origin: (1) Rests of arachnoidal cap cells are trapped in the cranial sutures during head molding at birth; Lang et al., however, found that only 8% of calvarial meningiomas were associated with a cranial suture. (2) Entrapment of dura occurs in the fracture lines; however, most PEMs reported in literature, including our case, do not have a history of trauma. PEMs arise directly from multipotent mesenchymal cells in reaction to a yet unknown stimulus.
Intraosseous meningiomas are often misdiagnosed preoperatively as secondary malignant deposits.,,,, A few other misdiagnoses reported in literature are due to the tumor being misrepresented as an osteoid osteoma, an eosinophilic granuloma, or as Paget's disease. This is due to its variable presentation in imaging studies which may be osteoblastic, osteolytic, or mixed. The differential diagnoses for an osteolytic lesion, as in our case, are chondroma, chondrosarcoma, epidermoid cyst, osteogenic sarcoma, myeloma, eosinophilic granuloma, and metastasis. The extent of a PIM can be well identified by a CT scan with bone window. MRI is important to study the dural involvement and intracranial extension. However, the findings in the imaging studies at present are insufficient to clinch the diagnosis of an intraosseous meningioma. Therefore, the diagnosis of PIM is not as easy as may be possible for its intracranial counterpart that is often associated with a dural tail sign. In petrous tumors particularly, interference by bones makes interpretation of CT scan images difficult. Hence, MRI is a better choice because of its multiplanar display and the absence of bony artifacts. However, CT and MRI remain complementary in ascertaining the possible nature and the extent of the lesion.
Primary intraosseous meningiomas have nonspecific clinical and radiological features. Owing to their rarity, they are often overlooked while the differential diagnoses of an intraosseous lesion is being considered. We would like to highlight the fact that their consideration in the differential diagnoses plays a crucial role in planning surgery. Stereotactic radiosurgery or a course of fractionated radiation therapy for residual lesions achieves a better outcome. In children, longer follow-ups are strongly recommended.
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[Figure 1], [Figure 2]