Primary Extracranial Meningiomas Research Articles

Primary Extracranial Meningiomas of the Sinonasal Tract: A Systematic Review

Abstract Background Primary extracranial meningiomas (PEMs) of the sinonasal tract with no intracranial extension are rare. Our study presents the largest systematic review to date, providing a comprehensive overview and comparison of the characteristics, treatment, and prognosis of PEMs, with comparison to primary intracranial meningiomas (PIMs). Methods A systematic review was conducted according to the PRISMA guidelines on PubMed, Embase, and Google Scholar up to November 1, 2022. A total of 70 documented cases from 64 articles were included. Data analysis was performed to compare low-grade (World Health Organization [WHO] Grade I) and high-grade (Grade II, III) PEMs. Results Majority of patients were symptomatic with nasal symptoms (72.1%), followed by eye symptoms (41.2%) and facial swellings (41.2%). The nasal cavity was the most commonly affected site (70.0%). Most tumors were WHO Grade 1 (87.5%) with meningothelial histological subtype. PEMs have a predilection for involvement of surrounding structures, with more than half reporting erosion of sinonasal walls or orbital involvement (64.3%). Despite so, PEMs have a favorable prognosis, with high survival rates (95.3%) and low rate of recurrence (8.5%). Mainstay of treatment is surgical resection (98.6%). A total of 69/70 cases (98.6%) underwent surgical resection. Conclusions There are no clear defining clinical features of PEMs; hence, it is imperative for clinicians to prioritize histological diagnosis to guide further management. A conclusive diagnosis of PEMs, i.e., exclusion of PIMs, requires imaging and potential intraoperative assessment. Its prognosis is likely dependent on both WHO grading and the ability to achieve complete surgical extirpation. Endoscopic resection is recommended even in high-grade PEMs for localized and resectable disease. Radiotherapy may be reserved for unresectable or recurrent tumors as salvage therapy.

Palato-Antral Involvement of a Primary Extracranial Sinonasal Meningioma

Primary extracranial sinonasal meningiomas are one of the rarest tumours involving the head and neck region. Very few reports exist with additional involvement of the palato-antral region. This paper describes a case of primary extracranial sinonasal meningioma with palatal involvement and the management of the same. The patient was taken up for surgical excision via intra-oral and endoscopic approaches. Postoperative healing was uneventful with no recurrence noted over 2 years. A palatal obturator was used for rehabilitation. The diagnosis of this pathology needs additional immunohistochemistry testing for confirmation and treatment entails complete surgical excision which assures no recurrence or delayed presentation of residual disease in follow-up.
 Keywords: primary extra cranial meningioma; sinonasal; maxilla ; palate.

Primary Extracranial Meningiomas of the Head and Neck.

Meningiomas represent the most common benign histological tumor of the central nervous system. Usually, meningiomas are intracranial, showing a typical dural tail sign on brain MRI with Gadolinium, but occasionally they can infiltrate the skull or be sited extracranially. We present a systematic review of the literature on extracranial meningiomas of the head and neck, along with an emblematic case of primary extracranial meningioma (PEM), which provides further insights into PEM management. A literature search according to the PRISMA statement was conducted from 1979 to June 2021 using PubMed, Web of Science, Google Scholar, and Scopus databases, searching for relevant Mesh terms (primary extracranial meningioma) AND (head OR neck). Data for all patients were recorded when available, including age, sex, localization, histological grading, treatment, possible recurrence, and outcome. A total of 83 published studies were identified through PubMed, Google Scholar, and Scopus databases, together with additional references list searches from 1979 to date. A total of 49 papers were excluded, and 34 manuscripts were considered for this systematic review, including 213 patients. We also reported a case of a 45-year-old male with an extracranial neck psammomatous meningioma with sizes of 4 cm × 3 cm × 2 cm. Furthermore, whole-body 68Ga-DOTATOC PET/CT was performed, excluding tumor spread to other areas. Surgical resection of the tumor was accomplished, as well as skin flap reconstruction, obtaining radical removal and satisfying wound healing. PEMs could suggest an infiltrative and aggressive behavior, which has never found a histopathological correlation with a malignancy (low Ki-67, <5%). Whole-body 68Ga-DOTATOC PET/CT should be considered in the patient’s global assessment. Surgical removal is a resolutive treatment, and the examination of frozen sections can confirm the benignity of the lesion, reducing the extension of the removal of healthy tissue surrounding the tumor.

PRIMARY EXTRACRANIAL MENINGIOMA OF THE MANDIBLE: A CASE REPORT AND LITERATURE REVIEW OF THIS RARE PRESENTATION

Meningiomas are common benign neoplasms developing within the central nervous system and arise from the meningothelial cells. Extracranial (ectopic) meningiomas are rare and may represent an extracranial extension of an intracranial meningioma or distant metastasis from a primary intracranial meningioma, or they may originate from arachnoid cells in the sheaths of the cranial nerves that are exiting the skull through the foramina or sutures. Ectopic meningiomas can also arise as true primary extracranial meningiomas originating from ectopic arachnoid lining cells or multipotential mesenchymal cells. Primary extracranial meningiomas occurring in the jaws are extremely rare, with only 11 reported cases (2 cases in the maxilla and 9 cases in the mandible) in the literature. The infrequent occurrence of this entity presenting in this unusual location of the jaws often poses a diagnostic challenge to the oral pathologist, resulting in misdiagnosis. We present the 10th mandibular case of extracranial meningioma involving the right side of the mandible in a 56-year-old female patient. The clinical and radiographic presentation, histopathologic features, and histopathologic differential and immunohistochemical findings of this case are presented. The proposed pathogenesis, treatment options, and prognosis are also discussed along with a review of the literature.

Primary Ectopic Meningioma of Nasal Cavity: A Rare Presentation.

Primary ectopic meningiomas of the nasal cavity are rare tumours and thus, often not diagnosed and treated properly. In this case report we are going to discuss about our experience with a primary nasal meningioma involving bilateral nasal cavity with its diagnosis, management and histopathological features. A 28year old female presented with nasal obstruction and nasal discharge for the past 1year and swelling over right side of face for the past 8months. Patient underwent surgical resection by a combined endoscopic and external approach under general anaesthesia. Histopathological evaluation confirmed the diagnosis of Transitional meningioma WHO grade 1. A repeat nasal endoscopy done one week after surgery, showed no evidence of any residual tumour. Due to ectopic meningiomas being relatively less common one must always exclude the presence of a central meningioma, which makes radiological investigations a must in such cases. As primary extra-cranial meningiomas have an unpredictable behaviour, this study should further aid in diagnosis and management of these tumours.

Primary extracranial meningioma presenting as a forehead mass.

Meningioma originates from arachnoid cap cells and is the second most common intracranial tumor; however, it can also be found in an extracranial location. A very rare primary extracranial meningioma without the presence of an intracranial component has also been reported. Primary extracranial meningiomas have been found in the skin, scalp, middle ear, and nasal cavity. A computerized tomography or magnetic resonance imaging scan is necessary to determine the presence or absence of an intracranial meningioma, and a biopsy is essential for diagnosis. We report a case of primary extracranial meningioma located in the forehead skin of a 51-year-old male.

Primary Calvarial Meningioma

Introduction: Although meningioma is the most common non-glial tumor of intracranial space, primary extra cranial meningioma is rare and comprises less than 2% of all meningioma. Case Presentation: We report a case of slowly progressing extracranial meningioma that was presented in a young adult woman. The tumor was asymptomatic but large enough to cause cosmetic discomfort. Patient underwent successful and complete surgical resection of her tumor and eventually fully recovered. Conclusions: Primary Extracranial Meningiomas (PEMs) without intracranial origin are very rare and because of their slow growing nature are initially managed by primary care physicians.

Ectopic meningioma in the bilateral nasal olfactory cleft: A case report and literature review.

Certain nasal neoplasms, such as ectopic menigioma, present as nasal polyps, together with similar symptoms. The present study reports the diagnosis and treatment of ectopic meningioma in the bilateral nasal olfactory cleft in order to improve the diagnosis and treatment of ectopic meningioma in the nasal cavity. By retrospectively analyzing the clinical data and reviewing the associated literature, a detailed introduction to the clinical manifestation, diagnosis and treatment of ectopic meningioma of the nasal cavity was ascertained. The tumor was removed from the sinus by functional endoscopy surgery. Regular follow-up appointments were scheduled every three months, with no evidence of recurrence to date. The olfactory recovery and nasal ventilation were normal subsequent to surgery. Meningiomas are infrequently occurring tumors with unpredictable clinical behavior. A clear understanding of the etiology and appropriate diagnostic and management principles may aid in overcoming the challenges of treating primary extracranial meningiomas.

Atypical primary meningioma in the nasal septum with malignant transformation and distant metastasis

BackgroundPrimary extracranial meningiomas (PEMs) originating from the nasal septum are extremely rare, as are extracranial metastases of meningiomas.Case presentationA 44-year-old male presented with a 2-month history of left-side nasal obstruction and frequent episodes of epistaxis. A friable mass originating from the nasal septum was resected completely via an endoscopic endonasal approach. According to WHO criteria, the tumor was diagnosed as an atypical meningioma radiologically and histopathologically. Two years later, a tumor recurred at the primary site with the same histopathological findings, and the patient was given local external radiotherapy (6840 cGy in 38 fractions). Two months after this local recurrence, a left anterior chest wall mass and a left parietal area scalp mass were observed. The subcutaneous mass was resected and showed histological evidence of malignant transformation. Several months after the last operation, the patient died.ConclusionsWe describe the clinical, radiological, and bio-pathological features of this unique case and review the literature on atypical PEMs originating in the nasal septum. To our knowledge, this is the first reported case of an atypical PEM originating from the nasal septum that recurred with malignant transformation and extracranial metastasis.

Primary retroperitoneal meningioma: A case report

Primary extracranial meningiomas are rare disease entities, occurring primarily in the head and neck. We herein present a very rare case of primary extracranial meningioma of the retroperitoneum, observed in a 56-year-old man who presented with a retroperitoneal mass measuring 11 cm in its greatest dimension. Histological examination revealed sheets of spindle cells with frequent whorling patterns. The neoplastic cells had abundant light eosinophilic cytoplasm with minimal mitosis or cellular atypism. Immunohistochemical analysis demonstrated diffuse and strong positivity for vimentin and epithelial membrane antigen. Ultrastructural findings revealed abundant intermediate filaments, complex interdigitating cellular processes, and desmosome-like intercellular junctions, all of which are characteristic ultrastructural features of meningiomas. The patient was free of recurrence or metastasis 11 months after surgery. To the best of our knowledge, this is only the second reported case of primary retroperitoneal meningioma.

Primary Extracranial Meningiomas: An Analysis of 146 Cases

Primary extracranial meningiomas are rare neoplasms, frequently misdiagnosed, resulting in inappropriate clinical management. To date, a large clinicopathologic study has not been reported. One hundred and forty-six cases diagnosed between 1970 and 1999 were retrieved from the files of the Armed Forces Institute of Pathology. Histologic features were reviewed, immunohistochemistry analysis was performed (n = 85), and patient follow-up was obtained (n = 110). The patients included 74 (50.7%) females and 72 (49.3%) males. Tumors of the skin were much more common in males than females (1.7:1). There was an overall mean age at presentation of 42.4 years, with a range of 0.3–88 years. The overall mean age at presentation was significantly younger for skin primaries (36.2 years) than for ear (50.1 years) and nasal cavity (47.1 years) primaries. Symptoms were in general non-specific and reflected the anatomic site of involvement, affecting the following areas in order of frequency: scalp skin (40.4%), ear and temporal bone (26%), and sinonasal tract (24%). The tumors ranged in size from 0.5 up to 8 cm, with a mean size of 2.3 cm. Histologically, the majority of tumors were meningothelial (77.4%), followed by atypical (7.5%), psammomatous (4.1%) and anaplastic (2.7%). Psammoma bodies were present in 45 tumors (30.8%), and bone invasion in 31 (21.2%) of tumors. The vast majority were WHO Grade I tumors (87.7%), followed by Grade II (9.6%) and Grade III (2.7%) tumors. Immunohistochemically, the tumor cells labeled for EMA (76%; 61/80), S-100 protein (19%; 15/78), CK 7 (22%; 12/55), and while there was ki-67 labeling in 27% (21/78), <3% of cells were positive. The differential diagnosis included a number of mesenchymal and epithelial tumors (paraganglioma, schwannoma, carcinoma, melanoma, neuroendocrine adenoma of the middle ear), depending on the anatomic site of involvement. Treatment and follow-up was available in 110 patients: Biopsy, local excision, or wide excision was employed. Follow-up time ranged from 1 month to 32 years, with an average of 14.5 years. Recurrences were noted in 26 (23.6%) patients, who were further managed by additional surgery. At last follow-up, recurrent disease was persistent in 15 patients (mean, 7.7 years): 13 patients were dead (died with disease) and two were alive; the remaining patients were disease free (alive 60, mean 19.0 years, dead 35, mean 9.6 years). There is no statistically significant difference in 5-year survival rates by site: ear and temporal bone: 83.3%; nasal cavity: 81.8%; scalp skin: 78.5%; other sites: 65.5% (P = 0.155). Meningiomas can present in a wide variety of sites, especially within the head and neck region. They behave as slow-growing neoplasms with a good prognosis, with longest survival associated with younger age, and complete resection. Awareness of this diagnosis in an unexpected location will help to avoid potential difficulties associated with the diagnosis and management of these tumors.

Clinical-radiological aspects of primary extracranial meningioma of the ethmoid sinus in a child

Meningiomas are tumors of benign histological nature which represent 13 to 26% of all primary intracranial neoplasia1. Approximately 20% of intracranial meningiomas present extracranial dissemination at sites such as the orbit, middle ear, nasal cavity, nasopharynx and paranasal sinuses2. Primary extracranial meningiomas are histologically identical to intracranial meningiomas. They usually occur in 40 to 60-year-old patients and are rare in the pediatric age group. Primary extracranial meningiomas represent 1 to 2% of all meningiomas1-5 […] Clinical-radiological aspects of primary extracranial [...]

Scalp meningioma

Primary extracranial meningiomas occur very rarely. We present a rare case of extracranial meningioma of the transitional variant which was excised satisfactorily. There was no suggestion of any connection to the intracranial compartment or cranial nerves. The underlying galea was uninvolved, suggesting the true extracranial nature of this tumour. This rare diagnosis should nonetheless be kept in the differential diagnosis of scalp tumors.

Primary Pulmonary Meningioma First Suspected of Being a Lung Metastasis

Primary extracranial and extraspinal meningiomas are rare tumors. We describe a primary pulmonary meningioma first suspected of being a metastasis because it presented as a solitary subpleural pulmonary nodule in a patient with breast cancer. The absence of radiographic change after 6 months of chemotherapy led to resection of the breast and lung lesions. A complete central nervous system evaluation eliminated other locations of meningioma, allowing the diagnosis of primary pulmonary meningioma.

Primary Meningioma of the Fallopian Canal

Meningiomas are slow-growing lesions that represent approximately 20% of all intracranial tumors and are the second most common tumor of the cerebellopontine angle. In contrast, primary extracranial meningiomas are found relatively infrequently, and most cases have inadequate radiologic studies to determine if they were actually an extension from a primary intracranial source. Meningiomas of the intratemporal segment of the facial nerve have also been reported, but they are exceedingly rare and their pathophysiology remains unclear. We report a case of a meningioma of the facial nerve in the fallopian canal of a 7-year-old girl and review pertinent literature.

Primary Meningioma of the Fallopian Canal

Meningiomas are slow-growing lesions that represent approximately 20% of all intracranial tumors and are the second most common tumor of the cerebellopontine angle. In contrast, primary extracranial meningiomas are found relatively infrequently, and most cases have inadequate radiologic studies to determine if they were actually an extension from a primary intracranial source. Meningiomas of the intratemporal segment of the facial nerve have also been reported, but they are exceedingly rare and their pathophysiology remains unclear. We report a case of a meningioma of the facial nerve in the fallopian canal of a 7-year-old girl and review pertinent literature.

Primary extracranial meningioma of the soft palate

Extracranial meningiomas comprise two per cent of all meningiomas. Primary extracranial meningiomas are even less common. The authors report the first case of a primary extracranial meningiomas of the soft palate, which presented as an intraoral mass. This was treated by surgical excision and there was no evidence of tumour recurrence at four years of follow-up.

Tuberous sclerosis with primary meningioma of the maxillary antrum

Primary extracranial meningiomas are uncommon; they have been documented in the ear, skin, orbit, parapharyngeal space, parotid gland, mediastinum, and rarely the paranasal sinuses. Tuberous sclerosis is an autosomal dominant neurocutaneous syndrome characterized by multiple connective tissue tumours. We report the first case of a tuberous sclerosis patient with a maxillary antral meningioma.

Primary extracranial meningiomas of the head and neck

Extracranial meningiomas comprise approximately 2% of all meningiomas. Primary extracranial meningiomas are even less common. This report details our experience with these unusual tumors from 1972 to 1989. The diagnosis, surgical management, and significant histopathologic features are discussed. The correlation of primary extracranial meningiomas with neurofibromatosis type II and a treatment algorithm are presented.

Diagnostic and therapeutic problems in extracranial meningiomas

Extracranial meningiomas, either primary or secondary, may present in numerous sites about the head and neck. We have described primary extracranial meningiomas of the middle ear and jugular foramen-parapharyngeal regions. Surgical extirpation of such meningiomas should provide a good prognosis if removal is complete. For tumors in the infratemporal region, preliminary total parotidectomy exposes the facial nerve so that the surrounding tumor may be removed without injuring the nerve. With patients who have extracranial extensions of intracranial meningiomas, the surgeon may be reluctant to excise the extracranial component. However, follow-up study of cases in which extracranial excision was performed suggests that long-term relief from pain and disfiguration may be expected. The intracranial component may be removed at a second-stage procedure, or in certain instances, the patient may be observed. Consultation with a neurosurgeon is essential in determining the appropriate therapeutic course. Extracranial meningiomas, either primary or secondary, may present in numerous sites about the head and neck. We have described primary extracranial meningiomas of the middle ear and jugular foramen-parapharyngeal regions. Surgical extirpation of such meningiomas should provide a good prognosis if removal is complete. For tumors in the infratemporal region, preliminary total parotidectomy exposes the facial nerve so that the surrounding tumor may be removed without injuring the nerve. With patients who have extracranial extensions of intracranial meningiomas, the surgeon may be reluctant to excise the extracranial component. However, follow-up study of cases in which extracranial excision was performed suggests that long-term relief from pain and disfiguration may be expected. The intracranial component may be removed at a second-stage procedure, or in certain instances, the patient may be observed. Consultation with a neurosurgeon is essential in determining the appropriate therapeutic course.