Extradural meningioma en-plaque of the cervical cord

A rare case of cervical extradural en-plaque meningothelial meningioma is reported. The magnetic resonance imaging revealed an extradural sheet of tumor encasing the cervical cord from anterior, posterior and right lateral aspects and emerging from the right C3-4 intervertebral foramina. Though a differential diagnosis of lymphoma and tubercular granulation tissue were considered, its isointense intensity patterns on T1 and T2 weighted images and the intratumoral calcification on intrathecal contrast computed tomographic scan suggested a meningioma. At surgery, the lesion was fibrous, avascular and densely adherent to the dura. The radiological features and management options of the lesion are discussed.     

A case of metaplastic meningioma with extensive xanthomatous change

We report a case of metaplastic meningioma with extensive xanthomatous change occurring in a 61‐year‐old woman. Magnetic resonance imaging demonstrated a well‐demarcated, dura‐based mass measuring 7 cm in maximal diameter in the right occipital to parietal area. Under a clinical diagnosis of meningioma, right parietal craniotomy was performed. Histologically, the tumor showed extensive xanthomatous change together with the common features of meningothelial meningioma. Tumor cells showed diffuse xanthomatous change in various quantities, and xanthomatous tumor cells frequently showed a gradual transition to the non‐xanthomatous counterpart. The xanthomatous tumor cells showed immunopositivity for epithelial membrane antigen (EMA), vimentin, fatty acid synthase and several histiocytic markers (CD68, Ki‐M1p, MAC387, lysozyme, alpha 1‐antitrypsin and alpha 1‐antichymotrypsin). In addition, there was patchy infiltration of foamy macrophages. Since these histiocytes did not show immunopositivity for EMA, these cells were distinguished from the xanthomatous tumor cells. These two types of xanthomatous changes comprised approximately two‐thirds of the whole tumor tissue. Any atypical features such as necrosis, frequent mitotic figures or brain parenchymal invasion were not observed. We diagnosed this case as xanthomatous meningioma, WHO grade I.     

Primary Osteolytic Intraosseous Atypical Meningioma with Soft Tissue and Dural Invasion: Report of a Case and Review of Literatures

Primary intraosseous meningioma is a rare tumor, and atypical pathologic components both osteolytic lesion and dura and soft tissue invasion is extremely rare. A 65-year-old woman presented with a 5-month history of a soft mass on the right frontal area. MR imaging revealed a 4 cm sized, multilobulated, strongly-enhancing lesion on the right frontal bone, and CT showed a destructive skull lesion. The mass was adhered tightly to the scalp and dura mater, and it extended to some part of the outer and inner dural layers without brain invasion. The extradural mass and soft tissue mass were totally removed simultaneously and we reconstructed the calvarial defect with artificial bone material. The pathological study revealed an atypical meningioma as World Health Organization grade II. Six months after the operation, brain MR imaging showed that not found recurrence in both cranial and spinal lesion. Here, we report a case of primary osteolytic intraosseous atypical meningioma with soft tissue and dural invasion.     

Concurrent spinal nerve root schwannoma and meningioma mimicking single‐component schwannoma

We present a first case of concurrent tumors consisting of schwannoma and meningioma arising at the same spinal level in a patient without neurofibromatosis. A 49‐year‐old man without clinical evidence of neurofibromatosis presented with a 5‐month history of right neck pain. MRI demonstrated an extradural tumor involving the right‐sided C2 nerve root with a small intradural component. T1‐ and T2‐weighted and contrast‐enhanced MRI could not differentiate the intradural tumor as different from the extradural tumor. Total removal of the tumors was performed. No contiguity of the extradural tumor with the intradural tumor was seen. The intradural tumor attached strongly to the dura mater around the C2 nerve root exits. Intraoperative pathological diagnosis confirmed the extradural tumor as schwannoma and the intradural tumor as meningioma. We therefore thoroughly coagulated the dura mater adjacent to the intradural tumor and resected the dura mater around the nerve root exits together with the tumor. Pathological examination revealed that the resection edge of the extradural component consisted of a spinal nerve with thickened epineurium and was free of neoplastic cells. No schwannoma component was evident in the intradural tumor. No obvious transition thus existed between the extra‐ and intradural tumors. Distinguishing these tumors prior to surgery is critical for determining an optimal surgical plan, as schwannoma and meningioma require different surgical procedures. We therefore recommend a careful review of preoperative imaging with the possibility of concurrent tumors in mind.     

Idiopathic Hypertrophic Spinal Pachymeningitis with an Osteolytic Lesion

Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a chronic, progressive, inflammatory disorder characterized by marked fibrosis of the spinal dura mater with unknown etiology. According to the location of the lesion, it might induce neurologic deficits by compression of spinal cord and nerve root. A 58-year old female with a 3-year history of progressive weakness in both lower extremities was referred to our institute. Spinal computed tomography (CT) scan showed an osteolytic lesion involving base of the C6 spinous process with adjacent epidural mass. Magnetic resonance imaging (MRI) revealed an epidural mass involving dorsal aspect of cervical spinal canal from C5 to C7 level, with low signal intensity on T1 and T2 weighted images and non-enhancement on T1 weighted-enhanced images. We decided to undertake surgical exploration. At the operation field, there was yellow colored, thickened fibrous tissue over the dura mater. The lesion was removed totally, and decompression of spinal cord was achieved. Symptoms improved partially after the operation. Histopathologically, fibrotic pachymeninges with scanty inflammatory cells was revealed, which was compatible with diagnosis of idiopathic hypertrophic pachymeningitis. Six months after operation, motor power grade of both lower extremities was normal on physical examination. However, the patient still complained of mild weakness in the right lower extremity. Although the nature of IHSP is generally indolent, decompressive surgery should be considered for the patient with definite or progressive neurologic symptoms in order to prevent further deterioration. In addition, IHSP can present as an osteolytic lesion. Differential diagnosis with neoplastic disease, including giant cell tumor, is important.     

Secretory meningioma of the middle ear: A light microscopic,immunohistochemical and ultrastructural study of one case

A 66‐year‐old woman was referred with left hearing loss. A probable diagnosis of left secretory otitis media with effusion was formulated. A left myringotomy was performed to remove hyperplastic hard tissue from the tympanic cavity. A high resolution CT scan of the temporal bone disclosed a soft‐tissue mass completely involving the mastoid and tympanic cavity, surrounding the ossicular chain which appeared spared with no signs of infiltration. The histopathologic, immunohistochemical and ultrastructural response was secretory meningioma, a rare variant of conventional meningothelial meningioma in atypical sites.     

Malignant meningioma with adenocarcinoma‐like metaplasia: Demonstration of intestinal phenotype

Meningiomas show a diverse histopathologic appearance, often referred to as metaplastic changes; however, adenocarcinoma‐like metaplasia is an extremely rare condition. Here, we present a novel case. A dura‐based bulky mass located in the right frontotemporal region was identified radiologically in an 83‐year‐old woman. The tumor, yellow to ash‐gray in color, was subtotally removed. Histopathological examination revealed robust adenocarcinoma‐like structures within a conventional meningothelial neoplasm. Meningioma elements showed a WHO grade I to III histology. Morphological and immunophenotypic transition between meningothelial and columnar epithelial cells was confirmed on detailed observation. It was of note that the adenocarcinomatous components shared an immunophenotype with intestinal epithelium, expressing CDX2, MUC2 and cytokeratin 20. The present case could be differentiated from secretory meningioma based on distinct cellular atypia, lack of intracytoplasmic lumina and pseudosammoma bodies, and the intact status of the KLF4 gene. In addition, the morphological and immunophenotypic transition excluded the possibility of metastatic carcinoma within meningioma. This is the first reported case of meningioma with adenocarcinoma‐like metaplasia harboring an intestinal immunophenotype.     

Lymphoplasmacyte-rich meningioma mimicking idiopathic hypertrophic pachymeningitis.

A 24-year-old man presented with long-term headache and progressive visual loss. Neuro-ophthalmic manifestations included finger counting acuity in both eyes, weakly reactive pupils, pale optic discs, and increased deep tendon reflexes. Brain MRI showed meningeal thickening that involved the optic nerves and chiasm and enveloped and displaced the brainstem as far caudally as the foramen magnum. The diffuse extensive nature of the lesion suggested an inflammatory process such as idiopathic hypertrophic pachymeningitis (IHP), but anterior temporal brain biopsy disclosed a relatively high proportion of meningothelial cells with islands of polyclonal inflammatory reaction consistent with a diagnosis of lymphoplasmacyte-rich meningioma (LRM), a rare variant. Among the 19 reported cases of LRM, none has shown as extensive a mass as seen in our patient. Distinguishing between LRM and IHP is important because these entities are treated differently.     

Coexistence of amyotrophic lateral sclerosis with neuro‐Behçet's disease presenting as a longitudinally extensive spinal cord lesion: clinicopathologic features of an autopsied patient

We report the clinical and autopsy features of a 65‐year‐old Japanese man who clinically exhibited overlap of both neuro‐Behçet's disease (NBD) and amyotrophic lateral sclerosis (ALS). The patient had a HLA‐B51 serotype, a recent history of uveitis and had suffered paraparesis, sensory and autonomic disturbance, frontal signs and tremor. A brain and spine MRI study revealed a longitudinally extensive thoracic cord (Th) lesion, but no apparent intracranial abnormalities. The lesion extended ventrally from Th4 to Th9, exhibiting low intensity on T1‐weighted images, high intensity on T2‐weighted and fluid‐attenuated inversion recovery images and gadolinium enhancement. The patient's upper and lower motor neuron signs and sensory disturbance worsened and he died 16 months after admission. At autopsy, the spinal cord and brain exhibited characteristic histopathological features of both NBD and ALS, including chronic destruction of the ventral thoracic white and gray matter, perivascular lymphocytic infiltration, binucleated neurons, lower and upper motor neuron degeneration, Bunina bodies and skein‐like inclusions. Although incidental coexistence of these rare disorders could occur in an individual, this case raises the possibility of a pathomechanistic association between NBD and ALS.     

The origin of intracranial fibrosarcoma

Summary To elucidate the cellular derivation of intracranial fibrosarcoma, specimens from two cases of primary sarcoma and two cases of secondary, radiation-induced sarcoma in the cranium were examined by light and electron microscopy and histochemical techniques. The findings were carefully compared with those in four cases of benign fibroblastic meningioma. All the intracranial sarcomas examined in this study were localized on the cerebral surface and invaded the dura mater and brain.The histology of these tumors closely resembled that of fibrous fibrosarcoma described by Christensen and Lara [2]. The microscopic features of primary sarcomas were characterized by a spoke wheel arrangement of tumor cells, intercellular deposits of variable amounts of collagen fibers and the presence of many xanthoma cells. Secondary sarcomas presented tumor cells arranged in parallel interlacing bundles. An island of tumor tissue with the characteristics of benign meningioma was disclosed in a primary sarcoma. In meningioma was disclosed in a primary sarcoma. In electron micrographs, sarcoma cells showed ultrafine structures characteristic of meningothelial cells, including interdigitated cytoplasmic processes, desmosomes and/or half desmosomes, and abundant intracytoplasmic filaments. Basement membrane or an analogous substance, which is very unusual in the meningioma, were identified in some specimens of our primary sarcomas and fibroblastic meningioma. In the histochemical study, the activities of acid phosphatase and non-specific esterase showed no significant differences between the sarcoma cells and the meningioma cells.On the basis of morphological and histochemical findings, it is concluded in this study that fibrous fibrosarcomas in the cranium are of meningothelial derivation and therefore should be included in the category of malignant meningioma.     

Pineal chordoid meningioma complicated by repetitive hemorrhage during pregnancy: Case report and literature review

Chordoid meningioma is an uncommon variant of meningioma, and is very rarely found in the pineal region. We report a case of pineal region chordoid meningioma occurring in a young woman complicated by repetitive hemorrhages in the setting of pregnancy. A 23‐year‐old woman, 28 weeks pregnant, was transferred to our hospital for further management of a multi‐septated, hemorrhagic pineal region mass and hydrocephalus. MRI revealed a heterogeneous T2‐hyperintense lesion measuring 1.7 × 1.7 cm in the pineal gland. Resection of the tumor through an occipital transtentorial approach was performed. Histopathologic examination of the lesion confirmed the diagnosis of chordoid meningioma demonstrating cords and clusters of eosinophilic cells with rare cytoplasmic vacuolation arranged in a mucinous stroma. Additionally, there was abundant lymphoplasmacytic infiltration within the tumor. The details of this case are presented with a review of the literature.     

A case of hypertrophic cranial pachcymeningitis in a patient with lymphocytic hypophysitis]

We report a 67-year-old man of lymphocytic hypophysitis complicated by hypertrophic cranial pachcymeningitis. The hypophysitis presenting with central diabetes insipidus caused isolated adrenocorticotropic hormone (ACTH) deficiency after three years and two months since the onset of his illness. Moreover he suffered from left cavernous sinus syndrome after four years. Brain gadolinium (Gd) enhanced MRI, which was obtained in 2001, showed only enhancement of the pituitary stalk. However, brain Gd enhanced MRI, performed four years later, showed not only a remarkable thickening and enhancement of the pituitary stalk but also abnormal enhancement of the lesion in the left cavernous sinus. In addition, it showed diffuse thickening and enhancement of the dura mater. This clinical course suggested that chronic inflammation of the pituitary had spread to the dura mater, a clinical condition differing from parasellar chronic inflammatory disease (PSCID). In addition, because diffuse thickening and enhancement of the dura mater was present, it was likely that lymphocytic hypophysitis was complicated by hypertrophic cranial pachcymeningitis due to autoimmune reactions. We should carefully observe cases of lymphocytic hypophysitis and assess change over time in the dura mater of the whole brain by Gd enhanced MRI.     

Dura mater graft‐associated Creutzfeldt‐Jakob disease with 30‐year incubation period

Over 60% of all patients with dura mater graft‐associated Creutzfeldt‐Jakob disease (dCJD) have been diagnosed in Japan. The incubation period has ranged from 1 to 30 years and the age at onset from 15 to 80 years. Here, we report a 77‐year‐old male Japanese autopsied dCJD case with the longest incubation period so far in Japan. He received a cadaveric dural graft at the right cranial convexity following a craniotomy for meningioma at the age of 46. At 30 years post‐dural graft placement, disorientation was observed as an initial symptom of dCJD. He rapidly began to present with inconsistent speech, cognitive impairment and tremor of the left upper extremity. Occasional myoclonic jerks were predominantly observed on the left side. Brain MRI presented hyperintense signals on diffusion‐weighted and T2‐weighted images, at the right cerebral cortex. The most hyperintense lesion was located at the right parietal lobe, where the dura mater graft had been transplanted. Single‐photon emission CT scan showed markedly decreased cerebral blood flow at the right parietal lobe. EEG revealed diffuse and slow activities with periodic sharp‐wave complex discharges seen in the right parietal, temporal and occipital lobes. He died of pneumonia 9 months after onset. Brain pathology revealed non‐plaque‐type dCJD. Laterality of neuropathological changes, including spongiform change, neuronal loss, gliosis or PrP deposits, was not evident. Western blot analysis showed type 1 PrP^CJD. Alzheimer‐type pathology and PSP‐like pathology were also observed.     

Meningioma with multiple rheumatoid nodules

Summary A case of a left tentorial meningioma in a 42-year-old man with a 12-year history of widespread manifestations of active rheumatoid arthritis, is reported. The patient had numerous subcutaneous rheumatoid nodules involving among other areas, the nuchal scalp. The surgically removed meningioma contained many classical rheumatoid nodules with gradual transitions seen from tumor cells to the pallisading peripheral cell layer of the nodules. Although intracranial rheumatoid nodules of the dura mater, leptomeninges, and of the choroid plexus have been described earlier, this appears to be the first reported case of rheumatold nodules found within the substance of a meningioma. The participation of the tumor cells in the formation of rheumatoid nodules attests to the potential of neoplastic meningothelial cells to behave as mesenchymal elements under certain circumstances.     

特发性肥厚性硬脑膜炎临床、实验室检查及影像学特点分析

目的分析6例特发性肥厚性硬脑膜炎(IHP)患者的临床特点及辅助检查特征。以提高对特发性肥厚性硬脑膜炎的认识。方法回顾性分析并总结2014年1月至2017年11月在河南省人民医院神经内科就诊的6例临床诊断为特发性肥厚性硬脑膜炎(IHP)患者的临床、实验室检查结果及影像学特点。结果 6例患者主要临床表现为头痛、颅神经受累、癫痫、精神行为异常和共济失调。实验室检查血沉(ESR)、C反应蛋白(CRP)及脑脊液细胞和蛋白等炎性指标增高。磁共振影像表现为对称或不对称的硬脑膜T1加权等和(或)低信号,T2加权低信号,增强有强化。所有患者均给予激素冲击治疗,预后良好。结论 IHP以头痛及颅神经受累为主要表现,实验室检查炎性指标的升高及影像学硬脑膜增厚强化均有助于临床医师诊断及识别本病。     

Intracranial subdural osteoma: A rare benign tumor that can be differentiated from other calcified intracranial lesions utilizing MR imaging

We report the magnetic resonance (MR) imaging characteristics of subdural osteoma and other benign calcified intracranial lesions to highlight imaging features that differentiate between these disease entities. A 63-year-old woman presented with progressively altered mental status. Non-contrast CT demonstrated a densely calcified right middle cranial fossa extra-axial mass. MR imaging of the lesion demonstrated T1 and T2 hypointensity without evidence of contrast enhancement, parenchymal abnormality, or connection to adjacent venous structures. Diffusion weighted imaging demonstrated markedly decreased signal intensity and artificially reduced diffusion on apparent diffusion coefficient map. Histologically, the tumor was predominantly composed of lamellar bone and small fragments of residual dura consistent with subdural osteoma. This case demonstrates that radiological examination can provide additional insight into the origin of intracranial osteomas (extradural versus subdural versus sinonasal) and help distinguish from other diagnostic considerations including benign meningeal ossification and calcified meningioma prior to surgical resection.     

Lateral ventricular meningioma encapsulated by the dura‐like membrane

The first case of a ventricular meningioma that was encapsulated by the dura‐like membrane is reported. Magnetic resonance imaging (MRI) showed a heterogeneous mass with a low intensity rim in the trigone of the right lateral ventricle of a 63‐year‐old male. Histological examination revealed that the tumor was a transitional meningioma encapsulated by a thick dura‐like membrane. Moreover, abundant clusters of the dura‐like connective tissue existed in the tumor, indicating that both the dura‐like capsule and the dura‐like clusters in the tumor were created by the tumor cells.     

IgG4-Related Intracranial Hypertrophic Pachymeningitis : A Case Report and Review of the Literature

Hypertrophic pachymeningitis is an uncommon disorder that causes a localized or diffuse thickening of the dura mater. Recently, the possibility that IgG4-related sclerosing disease may underlie some cases of intracranial hypertrophic pachymeningitis has been suggested. We herein report the tenth case of IgG4-related intracranial hypertrophic pachymeningitis and review the previous literature. A 45-year-old male presented with left-sided focal seizures with generalization. Magnetic resonance imaging (MRI) revealed a diffuse thickening and enhancement of the right convexity dura matter and falx with focal nodularity. The surgically resected specimens exhibited the proliferation of fibroblast-like spindle cells and an infiltration of mononuclear cells, including predominantly plasma cells. The ratio of IgG4-positive plasma cells to the overall IgG-positive cells was 45% in the area containing the highest infiltration of plasma cells. On the basis of the above findings, IgG4-related sclerosing disease arising from the dura mater was suspected. IgG4-related sclerosing disease should be added to the pachymeningitis spectrum.     

Multifocal dural enhancement associated with temporal arteritis

OBJECTIVE: To report the association of temporal arteritis and enhancement of the dura mater and temporalis muscle. DESIGN AND METHODS: A single patient with a complaint of headache and diplopia was studied. SETTING: Academic medical center. PATIENT: A 69-year-old man presented with lateral rectus weakness, temporal artery tenderness, and an erythrocyte sedimentation rate of 65 mm/h. INTERVENTION: Biopsy of temporal artery and dura mater. MAIN OUTCOME MEASURES: Brain magnetic resonance imaging and pathological findings. RESULTS: Magnetic resonance imaging of the brain showed multifocal dural enhancement and enhancement of the temporalis muscles. The temporal artery showed a necrotizing vasculitis and the dura showed perivascular inflammatory cells. CONCLUSION: It is proposed that the temporal arteritis caused the multifocal dural enhancement and temporalis muscle enhancement on magnetic resonance imaging.     

Primary rhabdoid tumor with low grade glioma component of the central nervous system in a young adult

In the CNS, primary tumors with rhabdoid components are classified as atypical teratoid/rhabdoid tumor, rhabdoid meningioma or rhabdoid glioblastoma. The authors present a young adult patient with supratentorial rhabdoid tumor incidentally found after head trauma as a small pre‐existing lesion in the parahippocampal gyrus. MRI demonstrated an area of hypointensity on T1‐weighted images and hyperintensity on T2‐weighted and fluid attenuated inversion recovery images. A serial MR scan revealed no change 3 months after the initial examination but drastic changes at 6 months. As the tumor and accompanying intratumoral hemorrhage enlarged rapidly, resection of the tumor was performed. Histopathology revealed that the main component of the tumor was typical rhabdoid cells with some necrotic areas. There were also pathological features consistent with oligoastrocytoma. The specimen had neither vascular proliferation usually seen in high‐grade glioma nor the meningothelial pattern that suggests meningioma. Immunohistochemical findings revealed that cells were strongly positive for vimentin, epithelial membrane antigen and INI‐1 antibody throughout the specimen. Further, monosomy 22 was detected by fluorescence in situ hybridization. The tumor was finally thought to be an unclassifiable primitive rhabdoid tumor with oligoastrocytoma that arose in the CNS. The patient died within 5 months of detection of the tumor, regardless of surgical resection, radiotherapy and chemotherapy.