Abstract
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.
Highlights
IntroductionChronic inflammation processes or oral surgery can trigger the proliferation of these ectopic cells or the stimulation of multipotent mesenchymal cells, forming extracranial meningiomas (EMs) [3]
Meningiomas represent the most common benign histological tumor of the central nervous system; they originate from cellular elements of the meninges, arachnoid cap cells, arachnoid granulations, subarachnoid blood vessels, fibroblasts, and pia mater [1].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 [2].Chronic inflammation processes or oral surgery can trigger the proliferation of these ectopic cells or the stimulation of multipotent mesenchymal cells, forming extracranial meningiomas (EMs) [3]
Demographics areneck reported in Table tumors, or they had no data available
Summary
Chronic inflammation processes or oral surgery can trigger the proliferation of these ectopic cells or the stimulation of multipotent mesenchymal cells, forming extracranial meningiomas (EMs) [3]. Due to their rarity, EMs can be misdiagnosed: their current occurrence is underestimated, affecting their clinical management negatively. Perineuriomas share many features with meningiomas, but the latter show higher morbidity and greater incidence of neighboring structures infiltration. The management of meningiomas includes radiosurgery to treat eventual tumor remnants, and the surgical strategy is more aggressive if compared to perineuriomas [4,5]. We performed a systematic literature review, and we present an emblematic case of primary neck extracranial meningioma, providing further insights into the behavior of these rare tumors, improving their clinical management
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