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Purpose: To report a rare case of isolated intraconal meningioma. Case Report: A 24-year-old woman presented with painless proptosis in her left eye which started and progressed during her pregnancy about 10 months ago. Hertel exophthalomometry revealed anterior displacement of the globe with 4 mm of proptosis which was remarkable. Magnetic resonance imaging (MRI) demonstrated an intraconal circumscribed oval-shaped mass with hypointense signals on T1- weighted images and hyperintense signals on T2-weighted images, mimicking cavernous hemangioma. This mass, however, was free of any connections to optic nerve or bones. Due to the imaging characteristics, more prevalent diagnoses like cavernous hemangioma were placed on the top of the differential diagnoses list. However, during the surgical excision, the tumor’s consistency and gross features were not compatible with cavernous hemangioma. The pathologic findings instead determined meningotheliomatous meningioma, a very rare condition, which was far from our expectations prior to the surgery. Conclusion: Ectopic orbital meningiomas are rare tumors that are not easily diagnosed without postoperative histopathology. Despite its low prevalence, they should be considered in the differential diagnosis list of intraconal masses with hypointense signals on T1-weighted images and hyperintense signals on T2- weighted images.

Primary heterotopic meningiomas are lesions that are not associated with a cranial nerve foramen, vertebral canal, or intracranial structure. The most common histopathological subtype is meningeal epithelioma. In clinical practice, primary heterotopic nasal meningioma occurs relatively rarely, and its most common pathological type is psammomatous meningioma, whereas nasal fibrous meningioma is infrequent. In our case, a 31-year-old male patient was admitted to the hospital with “progressive nasal obstruction on the right side for half a year.” Computed tomography examination of the paranasal sinuses revealed a polyploid mass in the right nasal cavity. The patient underwent surgical resection of the mass under nasal endoscopy. Histopathological examination confirmed that the mass was an ectopic meningioma of the nasal cavity. The patient was regularly followed up for 2 years without recurrence of the tumor. Primary heterotopic fibrous meningioma of the nasal cavity is clinically rare but should be considered as a differential diagnosis for hemangioma of the nasal cavity, inverted papilloma, and nasal polyp. The final diagnosis is based on pathology and immunohistochemistry analysis results.

Extracranial meningioma is very rare with few cases reported, especially in the oral cavity. Its diagnosis is considered a challenge owing to the unusual site of occurrence. We report, to the best of our knowledge, the first case of extra-cranial meningioma as a primary tumor in the hard palate with no detected intracranial extension. A 59-year-old Egyptian female patient presented with a 22-year history of a large painless swelling at the right side of the hard palate, which could not be seen on radiographs. An incisional biopsy was taken and, after assessment with a panel of immunohistochemical markers, the lesion was diagnosed as extracranical grade I mengiothelial meningioma. The patient did not show up for surgical excision and follow-up was not performed because of the lose of contact with the patient. Intraoral meningioma is a rare tumor. Immuohistochemical markers are important for confirming this diagnosis.

Primary ectopic meningioma of the middle ear is relatively rare in clinical practice. It is often difficult to distinguish it from chronic otitis media or otitis media with effusion due to its similar and atypical clinical symptoms. We report a case of epithelial tympanic ectopic meningioma with the main complaints of otalgia, aural fullness, and hearing loss. It was accidentally discovered during tympanotomy due to the symptoms of recurring refractory secretory otitis media. This article briefly reviews the relevant literature in recent years, summarizes the characteristics of primary ectopic tympanic meningioma with intact tympanic membrane, and emphasizes the diagnosis and treatment strategy of the middle ear mass.

An ectopic meningioma, such as a primary pulmonary meningioma (PPM), is a rare type of tumor that primarily originates outside of the central nervous system. A 65-year-old female patient underwent a thoracoscopic lung wedge resection of the right lower lobe for a micro solid nodule detected via computed tomography. The histologic result revealed a PPM. PPMs manifested with micro solid nodules are a very rare occurrence in clinical practice. Increased awareness of the clinical and pathological characteristics of this rare disease can assist thoracic surgical teams to apply adequate management.

An 80-year-old male with a previous history of thymoma and kidney cancer underwent a 18F fluorodeoxyglucose (FDG) positron emission tomography (PET)/contrast-enhanced computed tomography (CT) scan: two pulmonary nodules were discovered, both characterized by an increased glucose uptake. Of them, only one showed mild contrast enhancement. Both nodules were surgically resected: the first nodule was a benign meningioma and the second one was a malignant meningioma. This case study shows that malignancy of meningioma in the lung is not correlated with 18F FDG uptake and the contrast enhancement, thus suggesting that PET/CT may represent a suboptimal imaging modality for the evaluation of these lesions.

Meningiomas originate in the central nervous system and are the most common intracranial benign tumor. However, although rarely, they can develop extracranially. Primary extracranial meningiomas are frequently misdiagnosed, resulting in inappropriate clinical management. The most common sites of extracranial meningiomas include the skull, scalp, orbit, nose, paranasal sinuses, middle ear, neck, and skin. A 77-year-old woman presented with a mass on her left eyebrow. Computed tomography revealed an enhancing soft tissue mass in the left frontal area. The differential diagnoses included benign and malignant tumors. The patient underwent surgical excision by a direct approach, with dissection through the galea plane. Histological examination showed tumor cells arranged in sheets or whorls, with occasional psammoma bodies. The margins were free of tumor. The mass measured 2.1 × 1.1 × 2 cm, and was diagnosed as an extracranial meningioma. The patient had no recurrence 1 year later. Extracranial meningiomas are rare; nonetheless, ectopic meningioma should be considered in the differential diagnosis of any mass lesion in the eyebrow region.

Primary intraorbital ectopic meningiomas are rare and their existence remains controversial. We present a 30-year-old female with painless, non-axial proptosis and a palpable superomedial mass. The MRI demonstrated that the mass had no optic nerve sheath or sphenoid wing involvement and was initially reported to have no intracranial extension. The patient was initially thought to have an ectopic orbital meningioma. Subsequent multidisciplinary team (MDT) consultation and further specialist review of the MRI revealed a subtle dural tail connecting to an enhancing mass in the olfactory groove. Biopsy revealed a WHO Grade 1 transitional meningioma with an infiltrative pattern. We argue that some previously reported cases of ectopic meningioma may lack the requisite imaging to discover the primary disease. Our report highlights the importance of MRI in this group of patients and the role of a skull-base MDT with specialist neuroradiology input to determine the true origin and extent of these extradural orbital meningiomas.

Rationale: Primary aldosteronism due to aldosteronoma is the most common form of secondary hypertension, with an estimated prevalence of 4% of hypertensive patients in primary care and around 10% of referred patients. Diagnosis is a clinical challenge with simultaneous occurrence of primary ectopic meningioma in the adrenal gland. To our knowledge this is the first reported case of simultaneous occurrence of aldosteronomas and ectopic meningioma in the adrenal gland based on literatures. Patient concerns: A 30-year-old man presented with resistant hypertension for one year. The computed tomographic scans were suggestive of left adrenal gland hyperplasia. Intervention: The patient underwent partial unilateral laparoscopic adrenalectomy. Diagnosis: The histopathological examination of the resected sample confirmed primary ectopic meningioma in adrenal gland and aldosterone producing adenoma (APA). The saline load test, captopril test, and plasma aldosterone/renin ratio were indicative of primary aldosteronism (PA). Outcomes: The patient had controlled blood pressure postoperatively. Lessons: The patient was diagnosed with PA due to APA and nonfunctional primary ectopic meningioma in the adrenal gland which is very rare and dealt with unilateral laparoscopic adrenalectomy.

Ectopic meningioma is a generally rare type of benign tumor that very rarely occurs in the lung. Here, we report the cytological findings of a primary pulmonary meningioma with a particular focus on immunohistochemical (IHC) assessment. A healthy 60‐year‐old woman visited our hospital with an asymptomatic nodule in the right lower lung lobe. She had no particular past‐history and no other tumors in the central nervous system or elsewhere according to an imaging examination. Transbronchial fine‐needle aspiration cytology revealed clusters of spindle cells in a whorled formation and psammoma bodies. The tumor cells exhibited spindle‐shaped cytoplasm, small fusiform or round nuclei and numerous intranuclear cytoplasmic inclusions. IHC staining of the cytological specimen revealed that the tumor cells were positive for epithelial membrane antigen, negative for thyroid transcription factor‐1 and p40, and equivocal for claudin‐1. Progesterone receptor immunoreactivity of cytology specimen resulted negative at first by manual method but retrieved positive by an autostainer. Following segmentectomy, the pathological diagnosis was a meningothelial meningioma. The patient has remained well without recurrence for 36 months postoperatively. Because the cytological preparation exhibited characteristic findings of meningioma, a correct diagnosis based on pre‐operative cytological findings with appropriate IHC would be possible. Here, we report the cytological and IHC features of this case and highlight the importance of IHC‐quality assurance.

In August 2016, an 11-year-old boy presented to the authors’ institution with a right orbital tumor that was located superotemporally (superolaterally) and adherent to the sclera. The patient’s past medical history revealed that he had undergone 2 previous craniotomies elsewhere in June 2008 and July 2010 for a superomedially located orbital lesion that had been histopathologically diagnosed as a neurothekeoma. After the second craniotomy, the patient underwent adjuvant intensity modulated radiotherapy (IMRT) to the right medial orbit. At the authors’ institution, total excision of the orbital tumor was performed via an anterior conjunctival orbitotomy. Histopathological examination revealed a rhabdoid meningioma. Review of the histopathology obtained at the time of previous tumor excisions showed that the lesion was misdiagnosed as neurothekeoma and instead represented a meningioma from the beginning. The patient was started on a regimen of oral sunitinib and remained free of recurrence at 1.5 years of follow-up. Ectopic meningioma of the orbit is a rare entity. Rhabdoid meningioma is a rarely seen subtype of meningioma, accounting for 1%–3% of all intracranial meningiomas. To the best of the authors’ knowledge, this is the first case of an ectopic orbital rhabdoid meningioma reported in the literature. They suspect that tumor seeding during the previous surgeries might have played a role in the occurrence of the tumor in an orbital location not targeted by IMRT.

Introduction: Ectopic meningiomas are rare tumors which can be encountered by all surgical specialties. Patients and methods: We report on two different cases, a contemporary one and a historical one, highlighting the diversity of clinical presentations and prognoses of these lesions. Furthermore epidemiological aspects, clinical features, and diagnostic and therapeutic work-up in patients with an ectopic meningioma are reviewed. Results: Typically, ectopic meningiomas present as gradually expanding lesions, causing a variety of symptoms by their mass effect. Diagnosis is based on histological characteristics, which are similar to those of intracranial meningiomas. Treatment is primarily surgical. Conclusions: The cases we report are at different ends of the clinical and prognostic spectrum. Therapeutic options for different clinical scenarios are discussed.

Ectopic meningioma occurring in the region of parapharyngeal space is rare in clinical practice and brings great challenge in its diagnosis. This report details such a case in a 14-year-old girl with neurofibromatosis Type 2, which is a highly infrequent association. The clinical manifestations, imaging findings, and pathological manifestations are described, and the relevant literature is reviewed to highlight characteristic imaging findings of ectopic meningiomas.

Summary Primary extradural meningioma, also known as ectopic meningioma, is uncommon and represents ∼2% of all meningiomas. Malignant primary extradural meningioma is even more rare. The rarity of cases means the condition is not readily suspected or diagnosed. In this report, we present one case of primary extradural meningioma in elderly patients who presented with a scalp nodule and another highly suspected case. Both patients underwent surgical resection of the tumors. Histological study and immunostaining confirmed the diagnosis. We also conducted a literature review regarding similar cases. Surgical resection with a wide margin remains the first choice of treatment.

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.

Ectopic meningiomas are a very rare tumor entity. We present a case of a meningioma arising in the mandible of a young woman and initially supposed to be a radicular cyst. Histopathological and immunhistochemical evaluation showed typical cell characteristics of a meningioma. Only six cases of ectopic meningiomas in the mandible have been described in the literature until now, mainly in women at an advanced age and with surgical removal of all tumors. For the first time, no surgical excision has been performed in this case and follow-up control after 12 months showed no significant progression or increasing clinical complaints. Hence, surgical removal seems non-urgent. In conclusion, unclear lesions of the jaws, even if they seem to be clear following diagnostics, should be evaluated by incisional biopsy and histopathological evaluation.

Meningioma originates from arachnoid cap cells and is usually dura-based and slow growing. They account for 15% to 20% of primary brain tumors. Primary intraosseous meningioma (PIM) is a type of ectopic meningioma, and has been rarely reported. PIMs have been reported to constitute less than 2% of meningiomas, and are usual reported in the frontotemporal regions of the calvarium [1], orbits, and anterior cranial fossa. Frequently, patients with PIM of the orbit complain of aesthetic problems. We report a case of PIM of the orbit. A 54-year-old female had presented with gradually progressive exophthalmos of 5 months duration (Fig. 1). The patient had no pain or visual symptom, and there was no previous ocular trauma history. Ophthalmologic and neurologic examinations were normal finding except for 5 mm of exophthalmos. Facial computed tomography (CT) scan revealed thickening of cortical bone of the orbital wall at the right sphenoid wing (Fig. 2). The radiologic diagnosis of preoperative facial CT was fibrous dysplasia of the right lateral orbital wall and right skull base. Fig. 1 Preoperative view. Fig. 2 Preoperative facial computed tomography. Lateral eyebrow and subciliary incision were followed by parallel upper and lower ostectomies at the lateral orbital rim. The osteomised bone segment was excised to create enough space to approach the main mass at the right sphenoidal bone and part of the orbit lateral wall. The bony mass was removed with a high-speed drill and oscillating saw. The osteomised orbital rim segment was repositioned using fixation devices. The main mass consisted of fragile abnormal bone, resembling that of fibrous dysplasia. Histopathologic examination confirmed the diagnosis of meningioma. In microscopic views, oval and spindle shaped cells formed lobules between woven bones. Nuclei frequently exhibited central clearing and whorls were viewed. Cytoplasm and surrounding collagen were immunohistochemically positive for epithelial membrane antigen, and negative for glial fibrillary acidic protein (GFAP), cytokeratin (AE1/AE3), and Ki-67 (Fig. 3). At 3 months after surgery, exophthalmos was reduced to 2 mm, that is, into the relatively normal range (Fig. 4). On postoperative CT, most of the bone mass was removed but minimal amount of mass remained (Fig. 5). Although secondary surgery for complete resection of the mass was planned at 12 months after surgery, the patient was satisfied with the aesthetic results and was not willing to undergo additional surgery. Fig. 3 Epithelial membrane antigen positive finding (magnification, ×100, epithelial membrane antigen immunostain). Fig. 4 Postoperative view. Fig. 5 Postoperative facial computed tomography. Extradural meningiomas are uncommon, and more than 90% of extradural meningiomas occur in the head and neck [2]. Nevertheless, many other locations have been reported, such as, scalp, calvaria, orbit, paranasal sinus, neck, lung, mediastinum, skin, and finger. The differentiation of PIM and intradural meningioma depends on the presence of dural invasion. Hoye et al. [3] defined PIM as not involving the dura, but Lang et al. [2] did not treat dural invasion as an exclusive criteria and regarded main mass location, growth direction, and dural displacement pattern to be of greater importance. The majority of PIMs of the skull base and orbital walls present with pain, exophthalmos, and deformity. PIMs are generally benign and slow growing, but a higher proportion are associated with malignant change than standard meningioma [2]. These tumors are characterized by an intraosseous mass growth leading to significant hyperostosis involving the sphenoid wing, orbital roof, lateral orbital wall, and the middle fossa cranial base and a thin, usually soft-tissue growth at the dura. Several hypotheses have been proposed for the origin of PIM. Azar-Kia et al. [4] suggested that PIM arises from arachnoid cap cells trapped in the cranial suture during birth. In the reports issued by Lang et al. [2] and Crawford et al. [5] 8% and 64%, respectively, were suture related masses. It has also been proposed that PIM arises from dura and arachnoid entrapment caused by previous trauma. The third hypothesis is extradural meningioma arise from multipotent mesenchymal cells, which could explain masses located far from the head and neck. PIM is the condition most commonly confused with fibrous dysplasia. Both PIM and fibrous dysplasia expand bone and have a "ground glass" appearance. However, in intraosseous meningioma, there is irregularity of the cortical bone. In fibrous dysplasia, on the other hand, the bone is enlarged but follows the normal position and intact cortical bone. Surgical resection is required for symptomatic PIM. Excision of the tumor with reconstruction of bone as required is the choice of operation. Operations must be designed to achieve symmetry of both eyes, as proper facial symmetry and tumor resection are important for achieving successful aesthetic outcomes. In conclusion, we reported a rare case of PIM of the orbit that present with progressive exophthalmos. Surgical resection was performed to lessen avoidable morbidity. The patient was satisfied with the aesthetic results achieved.

This chapter covers some common, benign sinonasal entities (Schneiderian papillomas) and uncommon, emerging entities (respiratory epithelial adenomatoid hamartoma and sinonasal serous hamartoma). The latter likely represent benign adenosis-like tumor processes rather than hamartomas. Benign peripheral nerve sheath tumors and ectopic central nervous system entities are also discussed.

Aim Meningioma is a benign intracranial tumor. Occurrence of ectopic meningioma in maxillary alveolar ridge is very rare. Our aim is to add another case of ectopic meningioma in the maxillary alveolar ridge to the literature. Background Meningioma is a benign tumor that originates from meningocytes found in arachnoid villous structures of the meninges. The tumor is rarely seen in extracranial locations from the oral cavity. Case description We report on a 51-year-old edentulous woman with an ectopic meningioma on the right maxillary alveolar ridge. The patient had complained of dull pain and discharge from the right maxillary alveolar region. On clinical examination, we observed a lobulated lesion 2 × 2 cm in size. Radiographic examination showed ill-defined radiolucency with irregular radiopaque areas. We also noted oroantral communication from the root of the lesion into to the maxillary sinus with bony destruction. We closed the oroantral communication by a buccal advancement flap. The histopathological diagnosis of the specimen was transitional type grade I meningioma. The patient's 5-year follow-up was uneventful. Conclusion The histological subtype of the meningioma dictates the mode of surgery. Hence, resection is not considered the treatment of choice for grade I lesions. It is important to keep in mind that meningioma is the differential diagnosis of the intraosseous mandible tumors. Clinical Significance Ectopic meningioma is very rare. Meningioma should be considered as a differential diagnosis in clinical lesion. In the literature review, this is the fifth case report and review of the literature. How to cite this article Simsek HO, Komerik N. Ectopic Meningioma in the Maxillary Alveolar Ridge: Report of a Case with a Review of the Literature. Int J Experiment Dent Sci 2012;1(2):98-101.

Extranodal follicular dendritic cell sarcoma (FDCS) of the pharyngeal region is a rare malignant tumor recognized in recent years, with approximately 37 cases so far reported in the literature. It is often not considered at the initial evaluation and may be misdiagnosed in a small biopsy specimen. We report 4 cases of extranodal FDCS, 2 cases in the nasopharynx that were diagnosed as undifferentiated carcinomas because they were characterized by syncytial epithelial cells with sheet or nest-like distribution and 2 cases in the tonsil and soft palate that were characterized by vaguely concentric whorls consisting of spindle to ovoid cells. The latter case was diagnosed as ectopic meningioma. The analysis of all cases from the literature and ours shows that 58% (21/36) of the cases are misdiagnosed initially, often as undifferentiated carcinoma or meningioma, which the differential diagnoses should be mostly focused on. With a median follow-up of 27 months, the recurrence, metastasis, and mortality rates are 23%, 21%, and 3%, respectively, suggesting that extranodal FDCS of the pharyngeal region remains a low-grade sarcoma. Radical surgery is recommended, whereas there is no evidence to support adjuvant therapy.

Metastasis formation after resection of meningiomas is a rare event, predominantly occurring with malignant phenotypes. As far as we know, the presented case is the first report in the literature of iatrogenic seeding of a benign meningioma to the scalp following surgery. A 37-year-old woman was admitted because of a relapsing meningioma in the frontal lobe. In 1997, she had undergone complete excision of an atypical meningioma in same location. At follow-up, three new masses were found: a bifrontal meningioma on the edge of the falx, a smaller one in the falx just under the saggital sinus and a small mass, believed to be ectopic, in the periosteum at the site of the previous craniotomy. Surgical therapy was indicated. Histologically, the ectopic tumor was an atypical meningioma, similar to the one excised 10 years previously, with no relation to the other two intracranial masses. Because of the histological similarity and the location in the old craniotomy, the ectopic tumor was believed to have developed from an implantation metastasis as a consequence of the first surgery. The authors suggest that strict adherence to oncological principles should be applied in the case of benign neoplasms in order to prevent contamination of wounds with tumor cells and potential recurrence.

The authors describe a rare case of a primary extradural ectopic meningioma occurring in a 9-year-old female. A review of the literature with respect to clinical presentation, radiographic findings, management, and outcome among similar cases is discussed. Common features that may assist with diagnosing this unusual tumor include absence of bone or optic nerve sheath involvement, presentation at a young age, occurrence in the medial orbit, and sinus asymmetry on radiographic imaging. Preferred method of treatment is complete surgical excision.

Primary ectopic meningiomas occurring in the mediastinal region are extremely rare. So far, only five cases of primary mediastinal meningioma have been reported in the literatures. The imaging characteristics and the clinicopathological significance of mediastinal psammomatous meningioma have not been detailed. Here, we report the case of a 42-year-old male with primary posterior mediastinal psammomatous meningioma. The clinical features, imaging, and pathological findings are carefully analyzed, and the relevant literatures were reviewed.

Objective Primary ectopic meningioma is a rare tumor and is usually limited to the paravertebral soft tissues. The aim of this study was to assist in the diagnosis and treatment of the tumor. Methods A 16-year-old boy presented with a nasal floor mass. The computed tomographic scan suggested a neoplastic mass lesion in the nasal floor region, and primary diagnosis is sebaceous cyst. The patient received a surgical resection, and the histologic examinations showed that the lesion was characterized with spindle cells. Immunohistochemical staining showed that the tumor cells presented intense reactivity for epithelial membrane antigen and vimentin. Histologic examination and immunohistochemical staining confirmed the diagnosis of extracranial ectopic meningioma. Results The patient received a radical surgical and immediate restoration of adjacent flap, and the patient fully recovered 1 week later. Conclusions The case highlights the fact that despite its rarity, ectopic meningioma should be considered as a differential diagnosis of any mass lesion in the head and neck region, especially when spindle cells are histologically observed.

Object The authors retrospectively analyzed a consecutive series of patients with cranial vault primary extradural meningioma (PEM), with particular regard to the tumor’s dural involvement. The pertinent literature was reviewed. Methods Clinical data were retrospectively obtained in a consecutive series of 16 patients treated for a PEM at two institutions between 1992 and 2004. The authors created a classification system based on dural involvement of the tumors. Nine women and six men (mean age 55 years) presented with a painless, slowly progressive swelling. Preoperative magnetic resonance (MR) imaging revealed dural enhancement at the site of tumor in 11 patients. On surgical inspection, the tumor infiltrated the dura in all but three patients. Histological examination of tissue samples demonstrated tumor infiltration of the dura in all 14 patients in whom the dura had been resected. Three recurrent tumors were observed on follow-up examination during a mean period of 5.8 years (range 1.5–13 years) and required extirpation. In addition to one patient in whom there was histological evidence of malignancy, the other two cases involved two patients in whom no apparent dural involvement was observed during the first surgery. In a review of the literature, the authors found that histological examination showed dural involvement in 22%; the dura was not histologically evaluated in the remaining patients (78%). Postoperative follow-up data exceeding 2 years were only provided in two of the reported cases. Conclusions Tumor infiltration of the dura should be assumed in PEMs of the cranial vault, and resection of the dura at the site of craniotomy is recommended to prevent tumor recurrence.

Meningioma rarely gives an extra cranial extension. The authors report a case of meningioma of the jugular foramen reaching the left side of the neck. Epidemiological and clinical aspects of meningiomas of the neck are presented. The pathogenesis of this ectopic meningioma is discussed. We stress the contribution of imaging to evoke the diagnosis, determine the exact extent of the tumor, guide biopsies and allow post-therapeutic surveillance. Histological diagnosis of meningiomas of the neck is difficult since it can mimic more commonly encountered neck tumours. Difficulties of surgical techniques used to manage the tumor are also reported.

Ectopic (extradural) meningiomas that do not originate from either the optic fascicle or the intracranial meninges are exceedingly rare. A patient with an ectopic meningioma in the external auditory canal and mastoid was treated in our unit. The world literature of the last 20 years was reviewed.

Meningiomas are benign tumors derived from arachnoid cells. Most commonly an intracranial lesion, meningiomas may be found extracranially in various anatomic sites. A 23-yr-old white female presented with left-sided palpable mass located submucosally in the floor of the mouth. CT scan revealed no evidence of mass elsewhere in the head and neck region. Fine-needle aspiration cytology (FNAC) showed loose and cohesive cellular fragments with lobular growth pattern and uniform round or ovoid cells. The diagnosis of low-grade salivary gland neoplasm, not further classified, was made. The tumor was locally excised. The differential diagnoses of an extracranial meningioma and pleomorphic adenoma were discussed at the frozen section. Based on light microscopic, immunohistochemical, and electron microscopic (EM) findings, the final diagnosis of an ectopic meningioma was rendered. Ectopic meningiomas may pose a diagnostic challenge to clinicians and cytopathologists. It is easily forgotten in the list of differential diagnosis at an ectopic site. Primary ectopic meningioma in a region containing salivary gland(s) may mimic benign and low-grade malignant salivary gland tumors in FNAC.

Rationale: Intradiploic meningiomas are a subset of extradural meningiomas that arise in the skull. They are mostly localized in the frontoparietal and orbital regions, and they are often mistaken for primary bone tumors. Patient concerns: The patient was a 48-year-old man with headache and a 12-year history of frontal cranium occupation, which was first discovered in 2005 and enlarged in 2009. The patient had a history of fracture in his frontal cranium 12 years ago. The computed tomography and magnetic resonance imaging revealed an occupation and intracranial invasion in frontal cranium. And the angiography showed an occlusion at the anterior part of sagittal sinus. Interventions: Bilateral frontal craniotomy, intracranial tumor resection, and cranioplasty were performed. Diagnosis: Histologic examination confirmed an intradiploic ectopic meningioma (World Health Organization Grade I). Outcomes: He was discharged with no neurological deficits 3 days after surgery. At the 6-month clinical follow-up, there was no tumor recurrence or other complaints. Lessons: In this study, we present the case of a frontal intradiploic meningioma with progressive intracranial invasion and review the radiographic and clinical findings of patients with primary intraosseous meningioma.

A 1.7-year-old boy was admitted to our hospital with the first impression of ossified fibroma in the right ethmoid sinus. However, histopathological examination of the surgical specimen turned out to be meningioma. The mass was completely removed endoscopically assisted by image-guided navigation system. This is the youngest ectopic meningioma case reported. The clinic figure and treatment of primary paranasal sinus meningioma is briefly discussed. Considering the young age and small blood volume of this patient, careful preoperative preparation and the cooperation of multi disciplines were of vital importance.

Meningiomas arise from arachnoid cells. They can occur at intracranial or extracranial sites. Extracranial meningiomas are very rare tumors. They may arise from nasal cavity, paranasal sinuses, middle ear, and subcutaneous tissues. The ectopic meningiomas of paranasal sinuses do not have any communication with intracranial meninges. They usually occurs in young males. Imaging studies shows no bone erosion or intracranial extension. Primary surgical excision is the treatment of choice. The present case is reported due to its rarity and presentation at an unusual age.

We present a rare case of primary extracranial meningioma in a 36-year-old man, who had a solitary multinodular mass located in the plantar muscle of the foot. The histology of specimens from simple excision was typical of meningioma, showing bland spindle cell proliferation with a whorl pattern. Immunohistochemical analysis demonstrated that the tumor cells showed diffuse and strong positivity for epithelial membrane antigen as well as moderate reactivity for cytokeratin and vimentin. Ultrastructurally, the tumor cells were characterized by thin bipolar cytoplasmic processes and joined by multiple small desmosomes. There were frequent pinocytotic vesicles and a distinct external lamina on the cell surface. These findings suggest that this primary ectopic meningioma, arising in the soft tissue, may have been derived from perineurial cells of the peripheral nerve, but was morphologically distinguishable from perineurioma. Primary extracranial meningioma should be included in the differential diagnosis of soft-tissue spindle cell tumors, especially those of peripheral nerve origin.

Extradural ectopic meningioma is a rare tumor. We report on an example of microcystic meningioma arising in the skull of an elderly woman. Radiological examination revealed a localized osteolytic lesion in the left parietal bone. At surgery, it was discovered that the tumor was located within the skull without any evidence of extraosseous extension. The light microscopic, immunohistochemical and ultrastructural features were consistent with a microcystic variant of meningioma. To our knowledge, this is the first case of an intraosseous microcystic meningioma, and we believe that this type of meningioma should be considered in the differential diagnoses of myxoid bone tumors of the calvarium.

Intradiploic meningioma, which may be classified as a subgroup of intraosseous meningioma, is a rarely encountered disorder. To date, less than 10 cases have been reported. Here, we report a case of fronto-orbito-nasal intradiploic meningioma, A 12-year-old female with exophthalmos and diplopia was operated on for a cranial intradiploic mass lesion. Histopathological evaluation of the specimen confirmed the diagnosis of intradiploic psammomatous meningioma. Her exophthalmos did not change, but the diplopia disappeared. This case is unique in that it is an extensive case of intradiploic meningioma of the orbital roof and frontal base in a child. Intradiploic meningiomas generally are of psammomatous type. Especially tumors adjacent to the orbita cause exophthalmos; cases located on the other side of the calvarium may not cause any symptom or sign other than headache or sometimes a mass on the scalp. Treat ment, as with meningiomas located in the intracranial cavity, is total resection of the lesion. Copyright (C) 2000 S. Karger AG. Basel.

A 66-year-old man reported a slowly growing tumor on the lateral edge of his left upper eyelid. This lesion was hard but movable on palpation. A neoplasm of the lacrimal gland was suspected. CT showed a highly calcified lesion at the left upper eyelid. Resection of the tumor was performed, which was located just behind the orbital septum and in front of the lacrimal gland. Anatomopathologic investigation of the excised specimen with immunohistochemistry revealed a benign meningioma of a meningotheliomatous type, containing multiple bone elements. An ectopic orbital meningioma is rare, and this is the first case of a unique lateral localization of this lesion. Therefore, it should be included in the differential diagnosis of a lacrimal gland tumor.

Primary extra cranial and extra spinal meningioma is rare and is usually limited to the head and neck region or to the paravertebral soft tissues. Histologic diagnosis (in particular for fresh specimens) is often difficult. A 16-year-old girl with a cervical mass is presented. It was a relatively large mass with an approximate diameter of 5 cm. It was diagnosed in fine needle aspiration as a pleomorphic adenoma. Intra-operative diagnosis was paraganglioma and frozen section suggested an infiltrative tumor. Histologically, the lesion showed uniform spindle cell proliferation separated by hyalinized collagen bundles. The cells were often arranged in sweeping fascicles and concentrically wrapped in tight whorls. Immunohistochemically, tumor cells were positive for epithelial membrane antigen and vimentin, while showing no reaction for cytokeratin, chromogranin, neuron-specific enolase and thyroglobulin. A diagnosis of ectopic meningioma was established based on microscopic appearance and immunohistochemical profile of the tumor. Despite its rarity, ectopic meningioma should be considered in differential diagnosis of any mass lesion in head and neck regions, containing spindle cells.

A 29-year-old female with primary ectopic meningioma in the right ethmoid sinus was reported. She was treated by a right lateral rhinotomy with total removal of the tumor and the subsequent clinical course was good. Contrast enhanced magnetic resonance image (MRI) was most informative to define the tumor and decide the surgical procedure, in which it was demonstrated as well circumscribed mass lesion with considerable homogeneous contrast enhancement.

Intraorbital ectopic meningiomas, which in most cases occur along the orbital wall, are rare. The authors report a 27-year-old woman who suffered from an intraorbital meningioma lying apart from the optic nerve and having no connection with the periorbita or any small nerves.

Ectopic orbital meningioma is a rare tumor usually affecting the medial orbit. We present two cases that occurred in a 56-year-old woman and a 28-year-old man. The tumors in both patients were subtotally excised via orbitotomy surgery and were located in the superior quadrant in one of our patients and in the temporal quadrant in the other. Following histopathologic diagnosis, external beam radiotherapy (EBRT) was administered to one patient and intensity modulated radiotherapy to the other. We identified 12 other well-documented cases of ectopic orbital meningioma previously reported. Ectopic meningioma should be considered in the differential diagnosis of medial as well as lateral and superior orbital tumors. The tumor is usually well circumscribed but can be ill defined in imaging studies. There are intralesional calcifications and sclerosis of adjacent bone in some cases. Ectopic orbital meningioma can recur after incomplete excision. Based on the efficacy of EBRT in optic nerve sheath meningioma, we used this treatment to decrease the risk of recurrence in our two patients and found no tumor recurrence at follow-ups of 24 and 74 months, but one patient had severe vision loss from radiation retinopathy.

Background Over 98% of all meningiomas originate in the central nervous system including the optic fascicle. Spread outside this area occurs in 20% of cases. Ectopic (extradural) meningiomas that do not originate from either the optic fascicle or the intracranial meninges are exceedingly rare. We treated a patient with an ectopic meningioma of the orbit and reviewed the literature concerning ectopic orbital meningiomas. History and signs A 30-year-old man presented with a painless tumor in the left medial canthal area. This tumor had developed over a period of two years. The patient complained of intermittent epiphora. Ophthalmologic examination revealed a slight lateral displacement of the left eye. There was a palpable medial orbital mass with an otherwise normal ophthalmological examination. Computed tomography and magnetic resonance imaging showed a homogeneous, well-marginated soft tissue mass. Therapy and outcome The lesion which had neither a contact to the dura of the anterior cranial fossa nor to the optic fascicle was excised and histologic examination showed a fibroblastic meningioma. There was no recurrent tumor after a follow-up of 18 months. Conclusion Less than 50 ectopic (extradural) meningiomas of the orbit have been described in the literature. The most common histologic type is the meningotheliomatous (syncytial or transitional) meningioma. The mean age at diagnosis of these tumors is 31 years. After complete resection the prognosis is good.

Ectopic meningiomas within the orbit are very rare. Most of the previously reported cases were located along the medial wall. Here we report on three cases of ectopic meningiomas presenting as superomedial orbital masses along with their radiological and histopathological features. All three patients underwent surgical excision of the tumor via anterior orbitotomy.

Meningioma is a well-recognized tumour of the central nervous system. However, extracranial non-dural or ectopic meningiomas are rare and in most of the reported cases, meningiomas are diagnosed only after histopathologic examination. Over the last 5 years, the authors have seen three cases. In these case reports, they discuss the salient features of these lesions, which can aid the preoperative diagnosis and management of these patients. One of the cases is a rarity, owing to the extreme extracranial position which allowed the pressure of the tumour abrade the adjacent structures. In one case, reconstruction of the skull bone using polymeric materials (Acrylic matter) was necessary. In the other two cases, titanium plates and screws were used to reconstruct the jaw.

Introduction Extra-cranial meningioma or ectopic meningioma is a rare tumor. This tumor has been reported in various anatomic sites in the head and neck, mediastinum, skin and soft tissues. We report a rare case of ectopic meningioma in the submandibular region detected by using fine-needle aspiration cytology, histopathology and immunohistochemistry. This case represents another unusual site for extra-cranial meningioma, which prompted us to report it. Case presentation An 18-year-old Dravidian woman presented with swelling in the right submandibular region. The computed tomographic scan findings were suggestive of a neoplastic mass lesion in the right submandibular region. Fine-needle aspiration cytology led to the differential diagnosis of a monomorphic adenoma of a salivary gland or an ectopic meningioma. The patient underwent excision of the submandibular gland and tumor. The histological examination and immunohistochemistry studies confirmed that the lesion was an extra-cranial meningioma. At her two-year follow-up examination, there was no recurrence of the tumor. Conclusion Our experience with this case indicates that, although rare, meningioma should be entertained in the differential diagnosis of a mass lesion in the head and neck region.

We have examined the clinical characteristics and treatment of ectopic meningiomas (EMs). Samples from 17 patients with EMs were analyzed, and their clinical characteristics, mechanism, and treatment were studied in combination with the literature. The main clinical manifestations of EMs included increased intracranial pressure, epilepsy, local mass, and local occupying effects, but diagnosis of EMs depended on the pathology. Surgical removal can achieve the double objectives of confirmed diagnosis and treatment of tumors. The clinical characteristics of EMs vary with the sites of tumors. Operation is the treatment of first choice. Prognosis is better than that of typical meningiomas.

Primary ectopic meningioma is rare and usually limited to the head and neck region. Until now its occurrence in the mediastinum has not been reported in the literature searched on Medline using the keywords meningioma and mediastinum. We report here a case of primary mediastinal malignant meningioma which was treated by surgical resection and additionally followed by radiotherapy. The clinical features, treatment, pathological findings, and prognosis are analyzed and the literature based on ectopic meningioma is reviewed.

Two cases of solitary primary pulmonary tumors showing the immunohistochemical and ultrastructural features of meningothelial meningiomas are presented. The benign clinical and radiologic course, the negative computed tomography scan of the brain (case 1), and negative neuropathologic investigation (case 2) support the diagnosis of a primary pulmonary meningioma rather than a metastazing malignant intracranial meningioma. Negative neuroendocrine markers (neuron-specific enolase, chromogranin, bombesin) and the lack of neurosecretory granules by electron microscopy confirm the diagnosis of this rare pulmonary tumor.