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    Histopathology Image of Meningioma of the Middle Ear

            

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Primary extracranial meningiomas have been documented in the ear, skin, orbit, parapharyngeal space, parotid gland, mediastinum, and rarely the paranasal sinuses.

Site: The tumours affected the middle ear ,external auditory canal  or a combination of temporal bone and middle ear.

Meningiomas arising in or presenting as middle ear lesions are relatively uncommon.

The great majority of tumours that arise in the internal auditory canal are schwannomas of the eighth cranial nerve (acoustic neuromas). Meningiomas constitute the second largest group of posterior fossa tumors.    Vestibular Schwannoma of the Ear

Meningiomas arise from arachnoid villae, the apparatus responsible for cerebrospinal fluid absorption, in proximity to a major vein or dural sinus in most cases. These structures are present in many places in the temporal bone.

Arachnoid villae are present along neural foramena at the base of the skull. They have been observed histologically in the internal auditory canal and are the probable site of origin of meningiomas in this location.

Meningiomas of the jugular foramen manifest the same signs and symptoms as glomus jugulare tumours. They arise from arachnoid cells lining the jugular bulb and grow slowly, infiltrating the temporal bone and posterior fossa. These lesions, however, are more clinically treacherous than glomus tumours. Meningiomas infiltrate surrounding bone and nerve tissue and require wide margins of resection to prevent recurrence.

Primary meningioma most commonly occurs in the middle ear cleft.

Clinical presentation:   Patients often complain of vestibulocochlear nerve disturbance at presentation. Patients clinically present with hearing changes, otitis, pain, and/or dizziness/vertigo.

Gross appearance: The tumours ranged in size from 0.5 to 4.5 cm in greatest dimension. Gross features are those of granular or gritty mass.

Microscopic features:  Histologically, the tumours demonstrated features similar to those of intracranial meningiomas, including meningothelial, psammomatous, fibroblastic and atypical meningioma. An associated cholesteatoma may identified in some cases.

The most common form of meningioma in the middle ear is of meningothelial type.

Immunohistochemical studies confirm the diagnosis of meningioma with positive reactions for epithelial membrane antigen and vimentin.

The differential diagnosis includes paraganglioma, schwannoma, carcinoma, melanoma, and Middle Ear Adenoma.

                  

Temporal bone secretory meningioma presenting as a middle ear mass.Pathol Res Pract. 2006;202(6):481-4.

A 44-year-old woman presented with a history of increasing left hypoacusis and sporadic vertigo. CT scan revealed a tumor occupying the mastoid, middle ear, and external auditory canal. After surgical removal, a typical secretory meningioma was diagnosed. The histological hallmark and the immunohistochemical profile of secretory meningiomas are reviewed. The differential diagnosis of this tumor in this location is also commented on. As far as we know, primary temporal bone meningiomas with secretory histology have not been previously reported in the medical literature.

Cerebellopontine angle meningioma resulting in middle-ear polyp.J Laryngol Otol. 2006 Sep;120(9):786-8.

Extracranial spread of meningiomas to involve the middle ear is very rare. We present the case of a 43-year-old woman with a known cerebellopontine angle meningioma who subsequently presented with left-sided otalgia and a middle-ear mass extruding through the tympanic membrane due to local invasion of the meningioma. The tumour was excised surgically. A discussion of the relevant literature is also presented.

Meningioma of the internal auditory canal: a case report.J Med Assoc Thai. 2005 Nov;88 (11 ):1707-11.

Meningioma of the internal auditory canal is very rare. There are only 15 previous reports of intracanalicular meningioma. The authors add a case report of a patient with meningioma of the internal auditory canal. A 31-year-old woman presented with a one-year history of headache, dizziness, hearing loss and left facial paralysis. An MRI of the temporal bone demonstrated a tiny isointense intracanalicular tumor with inhomogeneous enhancement. In the operative field carried out by translabyrinthine approach, the tumor was found in the IAC without intracranial involvement. Pathology revealed a meningioma. The patient was followed up for 2 years without recurrence.

Meningiomas of the cerebellopontine angle with extension into the internal auditory canal. J Neurosurg. 2005 Jan;102(1):17-23.

OBJECT: Only some meningiomas of the cerebellopontine angle (CPA) extend into the internal auditory canal (IAC) or arise from its dural lining. The authors investigated cases of CPA tumors in which the meningioma was inserted in the dura mater in or at the ICA or infiltrated a cranial nerve. METHODS: The authors reviewed patient charts including surgical and clinical records, intraoperative recordings of auditory evoked potentials, records of postoperative auditory examinations, and imaging studies. In a series of 421 patients harboring CPA meningiomas, 72 patients in whom there was dural involvement of the IAC were investigated. Total tumor resection was achieved in 86.1%. In 34 patients, opening of the IAC was required for total tumor removal; this procedure did not influence the patient functional outcome. Among patients with secondary involvement of the IAC, anatomical preservation of the facial and cochlear nerves was obtained in 94%, whereas among patients in whom the lesion arose from the dura in or at the IAC these values were 80 and 75%, respectively. Functional preservation of the seventh and eighth cranial nerves in cases of tumor extension within the IAC was 86 and 77%, respectively, whereas in cases in which the IAC was involved it was only 60%. In four of five patients in whom the tumor had its origin in the dura mater within the IAC, the seventh or eighth cranial nerve had to be sacrificed to achieve tumor removal because of the lesion's infiltrative behavior. Facial nerve reconstruction by sural grafting was performed in the same operative procedure. CONCLUSIONS: Meningiomas of the CPA involving the IAC require special surgical management. Dural involvement of the IAC requires opening by using a diamond drill, a procedure that does not influence cranial nerve outcome. The increased rate of cranial nerve morbidity is attributed to the infiltrative behavior of these meningiomas. If affected nerve segments have to be sacrificed, immediate reconstruction enables satisfactory long-term results.

Meningiomas of the internal auditory canal. Neurosurgery. 2004;55(1):119-27.

OBJECTIVE: Meningiomas arising primarily within the internal auditory canal (IAC) are notably rare. By far the most common tumors that are encountered in this region are neuromas. We report a series of eight patients with meningiomas of the IAC, analyzing the clinical presentations, surgical management strategies, and clinical outcomes. METHODS: The charts of the patients, including histories and audiograms, imaging studies, surgical records, discharge letters, histological records, and follow-up records, were reviewed. RESULTS: One thousand eight hundred meningiomas were operated on between 1978 and 2002 at the Neurosurgical Department of Nordstadt Hospital. Among them, there were 421 cerebellopontine angle meningiomas; 7 of these (1.7% of cerebellopontine angle meningiomas) were limited to the IAC. One additional patient underwent surgery at the Neurosurgical Department of the International Neuroscience Institute, where a total of 21 cerebellopontine angle meningiomas were treated surgically from 2001 to 2003. As a comparison, the incidence of intrameatal vestibular schwannomas during the same period, 1978 to 2002, was 168 of 2400 (7%). There were five women and three men, and the mean age was 49.3 years (range, 27-59 yr). Most patients had signs and symptoms of vestibulocochlear nerve disturbance at presentation. One patient had sought treatment previously for total hearing loss before surgery. No patient had a facial paresis at presentation. The neuroradiological workup revealed a homogeneously contrast-enhancing tumor on magnetic resonance imaging in all patients with hypointense or isointense signal intensity on T1- and T2-weighted images. Some intrameatal meningiomas showed broad attachment, and some showed a dural tail at the porus. In all patients, the tumor was removed through the lateral suboccipital retrosigmoid approach with drilling of the posterior wall of the IAC. Total removal was achieved in all cases. Severe infiltration of the facial and vestibulocochlear nerve was encountered in two patients. There was no operative mortality. Hearing was preserved in five of seven patients; one patient was deaf before surgery. Postoperative facial weakness was encountered temporarily in one patient. CONCLUSION: Although intrameatal meningiomas are quite rare, they must be considered in the differential diagnosis of intrameatal mass lesions. The clinical symptoms are very similar to those of vestibular schwannomas. A radiological differentiation from vestibular schwannomas is not always possible. Surgical removal of intrameatal meningiomas should aim at wide excision, including involved dura and bone, to prevent recurrences. The variation in the anatomy of the faciocochlear nerve bundle in relation to the tumor has to be kept in mind, and preservation of these structures should be the goal in every case.

Meningioma of the internal auditory canal: case report. Arq Neuropsiquiatr.2003 Sep;61 (3A) : 659-62.

Meningiomas limited to the internal auditory canal (IAC) are rare. Acoustic neuroma is usually the diagnosis made when a tumor is found in this location because of its higher frequency. We report on a 58 year-old woman with a meningioma arising from the IAC and the difficulty to establish the pre-surgical diagnosis, based on clinical and radiological features. The perioperative suspicion and confirmation are very important to deal with the dura and bone infiltration in order to reduce tumor recurrence.

Primary ear and temporal bone meningiomas: a clinicopathologic study of 36 cases with a review of the literature. Mod Pathol. 2003;16(3):236-45.

"Primary" ear and temporal bone meningiomas are tumors that are frequently misdiagnosed and unrecognized, resulting in inappropriate clinical management. To date, a large clinicopathologic study of meningiomas in this anatomic site has not been reported. Thirty-six cases of ear and temporal bone meningiomas diagnosed between 1970 and 1996 were retrieved from our files. Histologic features were reviewed, immunohistochemical analysis was performed (n = 19), and patient follow-up was obtained (n = 35). The patients included 24 females and 12 males, aged 10-80 years (mean, 49.6 years), with female patients presenting at an older age (mean, 52.0 years) than male patients (mean, 44.8 years). Patients presented clinically with hearing changes (n = 20), otitis (n = 7), pain (n = 5), and/or dizziness/vertigo (n = 3). Symptoms were present for an average of 24.6 months. The tumors affected the middle ear (n = 25), external auditory canal (n = 4), or a combination of temporal bone and middle ear (n = 7). The tumors ranged in size from 0.5 to 4.5 cm in greatest dimension (mean, 1.2 cm). Radiographic studies demonstrated a central nervous system connection in 2 patients. Histologically, the tumors demonstrated features similar to those of intracranial meningiomas, including meningothelial (n = 33), psammomatous (n = 2), and atypical (n = 1). An associated cholesteatoma was identified in 9 cases. Immunohistochemical studies confirmed the diagnosis of meningioma with positive reactions for epithelial membrane antigen (79%) and vimentin (100%). The differential diagnosis includes paraganglioma, schwannoma, carcinoma, melanoma, and middle ear adenoma. Surgical excision was used in all patients. Ten patients developed a recurrence from 5 months to 2 years later. Five patients died with recurrent disease (mean, 3.5 years), and the remaining 30 patients were alive (n = 25, mean: 19.0 years) or had died (n = 5, mean: 9.5 years) of unrelated causes without evidence of disease. We conclude that extracranial ear and temporal bone meningiomas are rare tumors histologically similar to their intracranial counterparts. They behave as slow-growing neoplasms with a good overall prognosis (raw 5-y survival, 83%). Extent of surgical excision is probably the most important factor in determining outlook because recurrences develop in 28% of cases.

Meningioma in the internal auditory canal.J Laryngol Otol. 2001Jan;115(1): 48-9.

A case is presented of an entirely intracanalicular meningioma in a 48-year-old woman that was excised via a conventional translabyrinthine approach to the internal auditory canal (IAC). Pre-operative magnetic resonance imaging (MRI) suggested that the tumour was a vestibular schwannoma (VS). Histological examination confirmed the intra-operative impression that the tumour was a meningioma. Although VS is by far the commonest intracanalicular tumour, the differential diagnosis includes meningioma. MRI is unable to distinguish between these two entities when the tumour is located entirely in the internal auditory canal.

Middle ear meningiomas. Ann Diagn Pathol. 2000 Jun;4(3):149-53.

Meningiomas arising in or presenting as middle ear lesions are relatively uncommon. This study retrospectively reviews the clinicopathologic features of six meningiomas arising in or extending into the middle ear. The patients comprise five women and one man ranging in age from 45 to 67 years (median, 55 years) at the time of surgery. Five tumors arose in the posterior fossa or temporal bone region and one tumor arose from the auditory canal itself. Three tumors arose on the right side and three on the left. Duration of symptoms before surgery involving the middle ear was known in five patients and ranged from 2 to 13 years (median, 10 years). Symptoms at presentation included gait or balance problems (n = 3), chronic otitis media (n = 2), diplopia (n = 2), hearing loss (n = 2), pain (n = 1), aural polyp (n = 1), and tinnitus (n = 1). Histologically, all six tumors resembled a syncytial (meningotheliomatous) meningioma. Psamomma bodies were noted in two tumors and two tumors demonstrated mild nuclear pleomorphism. None of the tumors demonstrated histologic features of atypical meningioma. Follow-up information was available in five patients. Four patients had prior surgery for removal of posterior fossa temporal bone meningiomas and developed recurrences involving the auditory canal 60 to 84 months after surgery. At the time of most recent follow-up examination, three patients were alive with evidence of tumor (65, 112, and 214 months), one patient was alive with no evidence of tumor (99 months), one patient died in the postoperative period of sepsis and pneumonia following resection of a middle ear recurrence (64 months), and one patient was lost to follow-up analysis. Meningiomas arising in or extending to the middle ear canal are unusual. They more commonly arise in woman and in most cases involve extension of intracranial/cranial tumors into the canal.

Temporal meningiomas presenting as chronic otitis media.Am J Otol. 1993 Jul;14(4):403-6.

Two cases of temporal bone meningiomas presenting as chronic drainage ears are presented. One case was indicated by computer tomographic scan at the time of initial surgery to be limited to the middle ear, but recurred 9 months later with extensive cerebellopontine angle extension and cranial nerve palsies. The tumor in the second case extended from the middle ear to the cavernous sinus, with extensive intradural involvement. Meningiomas involving the middle ear must be considered to have intracranial extension, and magnetic resonance is the imaging study of choice.

Meningiomas of the jugular foramen.Otolaryngol Head Neck Surg. 1992 Feb;106(2):128-36.

Meningiomas of the jugular foramen manifest the same signs and symptoms as glomus jugulare tumors. They arise from arachnoid cells lining the jugular bulb and grow slowly, infiltrating the temporal bone and posterior fossa. These lesions, however, are more clinically treacherous than glomus tumors. Meningiomas infiltrate surrounding bone and nerve tissue and require wide margins of resection to prevent recurrence. Eight of these lesions have been managed in the past 5 years at the House Ear Clinic using modern imaging and skull base techniques. Two have recurred after "total" microsurgical removal. Presentation, radiologic evaluation, and management guidelines are reviewed.


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Meningioma of the internal auditory canal. Am J Otol. 1990;11(3):201-4.

The great majority of tumors that arise in the internal auditory canal are schwannomas of the eighth cranial nerve (acoustic neuromas). Meningiomas constitute the second largest group of posterior fossa tumors. Meningiomas arise from arachnoid villae, the apparatus responsible for cerebrospinal fluid absorption, in proximity to a major vein or dural sinus in most cases. Arachnoid villae are also present along neural foramena at the base of the skull. They have been observed histologically in the internal auditory canal (IAC), and are the probable site of origin of meningiomas in this location. Larger cerebellopontine angle meningiomas occasionally possess a significant intracanalicular component; however, these lesions usually originate from the meningeal lining of the posterior petrous face adjacent to the sigmoid, superior petrosal, or inferior petrosal sinuses and prolapse into the IAC. Two meningiomas have recently been observed that extensively involved the IAC, one of which arose from the lining of the IAC. The clinical manifestations of these meningiomas mimicked those of acoustic neuromas. Preoperative radiographic studies, including magnetic resonance imaging, were unable to differentiate these from acoustic neuromas. Meningiomas have a higher rate of recurrence than acoustic neuromas and should be excised with surrounding dura and several millimeters of subjacent bone. Meningiomas that extensively involve the IAC have a tendency to invade the inner ear and the deeper portions of the temporal bone. In meningiomas that involve the lateral portion of the IAC, consideration should be given to exenteration of the cochlea and semicircular canals.

Primary tumors of the external and middle ear. II. A clinicopathologic study of 14 paragangliomas and three meningiomas.Arch Otolaryngol. 1978 May;104(5):253-9.

Paragangliomas (chemodectomas, glomus jugulare tumors) represented 15% of all neoplasms of the ear in the period from 1964 to 1975 at the University of Minnesota, Minneapolis. Women in the fifth to sixthe decades of life were mainly affected, and hearing loss and tinnitus were the principal symptoms. Although most tumors had the typical histopathologic appearance, two neoplasms that were initially diagnosed as paragangliomas illustrated the problems in differential diagnosis. One of these latter two tumors had metastasized and was thought to represent a malignant paraganglioma. Retrospectively, both neoplasms were adenomatous tumors of the middle ear. For the remaining patients with typical jugular paragangliomas, surgery and/or irradiation therapy controlled the local tumor in 12 cases. Two of the three meningiomas occurring in the middle ear were preceded by or associated with an intracranial component. There were only four prior examples in the literature, excluding our one case, of primary meningiomas of the ear-mastoid region. Histologically, the features were virtually identical to the usual intracranial meningioma.

Meningiomas of the middle ear. Otolaryngol Pol. 1996;50(3):329-34.

The histological structure of meningiomas as well as symptomatology, diagnostics and treatment of those tumors originally localized in the temporal bone is discussed on the basis of literature. A case of a histologically confirmed intratympanic meningioma in a 43-year-old woman is presented. The original symptoms of the tumor imitated the symptoms of the chronic otitis media. Two operations of the ear were performed and the tumor which was completely removed has not given any symptoms of metastasis for six years.