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 Extra-axial Glioblastoma: Case Report and Literature Review

Glioblastoma multiforme (GBM) is the most frequent and most aggressive primary brain tumor in adults, mainly located in the cerebral hemispheres. In the literature, few cases of primary GBM have been reported to have radiographic and intraoperative features of extra-axial lesions, leading to a diagnostic dilemma. Despite the advances in imaging modalities, the diagnosis of GBM can be challenging, and it is mainly based on the histopathologic confirmation of the excised tumor. We describe the case of a 76-year-old previously healthy female patient who presented to our hospital due to speech disturbances and cognitive impairment. The diagnosis of the tumor type on magnetic resonance imaging (MRI) was difficult, as the findings were suggestive of a malignant meningioma due to the heterogeneous enhancement of a dural-based mass with a dural tail sign. Moreover, the intraoperative findings revealed an extra-axial mass attached to the dura. A histological examination confirmed the diagnosis of glioblastoma with arachnoid infiltration. The patient underwent adjuvant radiotherapy and concomitant temozolomide treatment, she had clinical improvement postoperatively, and was stable during the six months of follow-up. Glioblastoma should be considered in the differential diagnosis of primary extra-axial mass with atypical and malignant features, especially in elderly patients.

IgG4-Related Disease With Tuberculosis: A Case Report and Retrospective Review of Patients in a Single Center

BackgroundIgG4-related disease (IgG4-RD) is a recently recognized systemic fibro-inflammatory disease of unknown cause involving many organs including pancreas, salivary glands, and lymph nodes. Chronic tuberculosis (TB) infection has been reported in IgG4-RD, but the prevalence of TB infection has not been evaluated in IgG4-RD.MethodsCharacterization of a patient with IgG4-RD by physical examination, laboratory tests, magnetic resonance imaging (MRI) and histological examination. TB infection was evaluated by medical history, radiological examinations, sputum examinations, tubercular skin test (TST) and interferon gamma (IFN-γ) release assay test (IGRA). Medical records of IgG4-RD patients were reviewed in our institute from February 2015 to September 2020 to explore the prevalence of TB infection in IgG4-RD.ResultsWe described a 40-year-old Chinese man presented with headache and diplopia. Physical examination revealed bitemporal hemianopsia and limited abduction of both eyes. MRI revealed uniformly enhancing mass overlying clivus with dural tail sign. Laboratory data revealed elevation of IgG4 (1.9g/L), and TB-IGRA demonstrated significantly elevated IFN-γ (414.21 pg/ml). The clivus lesion was subtotally removed and IgG4 was strongly positive on immunohistochemical staining. The diagnosis of IgG4-RD was established, and the patient received treatment of corticosteroids, methotrexate, and cyclophosphamide with isoniazid prophylaxis. Consequently, the mass shrank remarkably within 3 months. A similar concurrence of TB disease or latent TB infection (LTBI) and IgG4-RD was present in 17/47 (36.2%) patients in our institute.ConclusionHigh frequency of TB/LTBI presented in patients with IgG4-RD. Patients with IgG4-RD and LTBI should be closely monitored for resurgence of TB. Whether TB represents a risk for IgG4-RD should be further investigated in prospective cohort.

Posterior fossa extra-axial variations of medulloblastoma: a pictorial review as a primer for radiologists

Manifestations of an atypical variant of medulloblastoma of the posterior fossa in extra-axial locations have been reported, and key questions concerning its interpretation have been raised previously. This review illustrated the clinico-radiological and histopathological features of the posterior fossa extra-axial medulloblastoma and described possible management strategies. We thoroughly reviewed all atypical anatomical locations of medulloblastoma reported within the posterior fossa and extra-axial spaces. The main characteristics of diagnostic imaging and histopathological results, primarily the distinctive radiopathological characteristics, were summarized to distinguish between intra- and extra-axial medulloblastoma, or pathologies mimicking this tumor. Most cases of posterior fossa extra-axial medulloblastoma have been reported in the cerebellopontine angle, followed by the tentorial and lateral cerebellar locations. The dural tail sign, which is commonly observed in meningioma, is rarely seen in intra- or extra-axial medulloblastoma and might be associated with other benign or malignant lesions. In addition to magnetic resonance imaging, the proposed new imaging techniques, including advances in modern neuroimaging modalities, were discussed, as potentially efficient modalities for characterizing extra-axial medulloblastoma. Radionuclide imaging and magnetic resonance perfusion imaging are practical alternatives to limit the number of differential diagnoses. We believe that medulloblastoma cases are likely under-reported because of publication bias and frequent tumors in unusual locations. Addressing these issues would help establish a more accurate understanding of this entity.

Radiological features of the intracranial extra-skeletal mesenchymal chondrosarcoma

Background: Mesenchymal chondrosarcomas are the most malignant form of chondrosarcomas. They have mostly affected bones. Rarely, these tumors can be intracranial extraskeletal (IEMC) that originates from the meninges or parenchyma. Methods and Materials: We presented two IEMC patients who were treated at our institutions and followed up for the long-term. To understand the radiological features of IEMC, we conducted a systematic literature review for previously reported series and cases of IEMCs. Results: We surgically treated two young males with IEMC initially diagnosed at their age of 18 and 20 years. The patients initially treated with gross total resection (GTR) and GTR followed by radiotherapy, and followed-up for 218 and 73 months, respectively. With both patients, we obtained 83 reported IEMC patients from the literature. Among them, only 30 cases were reported with their radiological MRI details. The mean age of the reported cases was 24.5±16.0 years (2 months–71 years). Female predominance was 54.2%. The mean progression-free and overall survivals were 27.9 and 39.0 months, respectively. Most IEMCs showed a partially calcified mass on roentgenography and a highly vascular mass on angiography. On T1WIs, IEMCs almost show hypo- to isointensity and intense heterogeneous enhancement after administering a contrast substance. On T2WIs, IEMCs show iso- to hyperintensity. Conclusions: IEMCs usually show dural attachment without a net dural tail sign and a well-identified brimmed vascular nodule on TOF-MRA. This nodule appears as a prominent blooming on SWI. TOF-MRA and SWI images can help in the radiological diagnosis of IEMCs.

Radiological Features of the Intracranial Extraskeletal Mesenchymal Chondrosarcoma: A Report of Two Cases Insight Into the Literature Review

Background: Mesenchymal chondrosarcomas are the most malignant form of chondrosarcomas. They have mostly affected bones. Rarely, these tumors can be intracranial extraskeletal (IEMC) that originates from the meninges or parenchyma. Methods and Materials: We presented two IEMC patients who were treated at our institutions and followed up for the long-term. To understand the radiological features of IEMC, we conducted a systematic literature review for previously reported series and cases of IEMCs. Results: We surgically treated two young males with IEMC initially diagnosed at their age of 18 and 20 years. The patients initially treated with gross total resection (GTR) and GTR followed by radiotherapy, and followed-up for 218 and 73 months, respectively. With both patients, we obtained 83 reported IEMC patients from the literature. Among them, only 30 cases were reported with their radiological MRI details. The mean age of the reported cases was 24.5±16.0 years (2 months–71 years). Female predominance was 54.2%. The mean progression-free and overall survivals were 27.9 and 39.0 months, respectively. Most IEMCs showed a partially calcified mass on roentgenography and a highly vascular mass on angiography. On T1WIs, IEMCs almost show hypo- to isointensity and intense heterogeneous enhancement after administering a contrast substance. On T2WIs, IEMCs show iso- to hyperintensity.Conclusions: IEMCs usually show dural attachment without a net dural tail sign and a well-identified brimmed vascular nodule on TOF-MRA. This nodule appears as a prominent blooming on SWI. TOF-MRA and SWI images can help in the radiological diagnosis of IEMCs.

Clinical and Radiologic Characteristics, Surgical Outcomes, and Its Possible Origins of Chondroma of the Dural Convexity

Chondroma of the dural convexity (CDC) is a benign and extremely rare type of intracranial chondroma. In this study, we reported five CDCs in a single center and reviewed the available literature to determine the clinical characteristics and surgical outcomes and possible origins of the disease. The clinical data of five patients (4 females) who confirmed to be CDC between 2000 and 2019 in our single center was collected together with 22 cases from literatures. The clinical characteristics and surgical outcomes were reviewed and analyzed. Among all the available CDC cases, the mean age was 31 ± 13.7 years ; the mean tumor volume was 42.3 ± 40.9 c m 3 , showing a female predominance (63% vs. 37%). The tumors showed calcification in 88.2% cases (15/17) on CT scans and hypointense on T1WI (15/19, 78.9%), mixed intense on T2WI (10/18, 55.6%), and inhomogeneous enhancement without dural tail sign after administration of gadolinium (20/21, 95.2%). Almost all the tumors were misdiagnosed as meningiomas preoperatively. In addition, almost all image available CDC lesions (24/25, 96%) located across the cranial sutures indicating that the tumor originated from ectopic chondrocytes from adjacent skull sutures. No tumors recurred after total resection in follow-up. CDCs are characterized with female predominance and may originate from ectopic chondrocytes from adjacent skull sutures. The lesion with inhomogeneous contrast enhancement without dural tail sign and avascular in cerebral angiography are key points to be differentiated from meningioma. The most effective treatment is total resection.