Circumscribed astrocytic gliomas: Contribution of molecular analyses to histopathology diagnosis in the WHO CNS5 classification

The newest revision of the WHO classification of tumors of the central nervous system, also known as WHO 5th edition, introduces substantial changes, especially within the glial tumor category and separates adult-type and pediatric-type glial tumors into different categories for the first time. In addition, another category of glial tumors, “Circumscribed Astrocytic Gliomas” were also created. This group includes pilocytic astrocytoma, pleomorphic xanthoastrocytoma, subependymal giant cell astrocytoma, chordoid glioma, astroblastoma, and the highly nebulous novel entity high-grade astrocytoma with piloid features. We present a brief and critical review of the pathological and molecular characteristics of these often well-demarcated tumors that can occur in adults as well as in the pediatric population.

Chordoid Meningioma: Literature Review

Chordoid meningiomas (CMs) are a rare subgroup of tumors, accounting for 0.5% of all meningiomas. Chordoid meningioma tumors correspond to World Health Organization (WHO) Grade II lesions and behave aggressively, with an increased likelihood of recurrence. There are few genetic studies about CMs, but we understand that there is deletion at many chromosomal loci. Histologically, CMs are characterized by strands and cords of meningothelial cells arranged in a mucinous stroma. Morphologically, it can mimic other chondroid and myxoid tumors within the brain and its vicinity, thus posing a diagnostic challenge. Chordoid meningiomas have an aggressive clinical course and a propensity to recur compared with classical meningiomas. The goal of the treatment is surgery, with total resection of the tumor; however, due to its high degree of recurrence, radiotherapy is often necessary as an adjuvant treatment.

A Case of Thoracic Extradural Chordoid Meningioma: Focusing on Radiologic Features

Chordoid meningioma, an uncommon subtype of meningioma, occurs rarely in the spine. In this case report, the authors present a case of spinal chordoid meningioma in a young female patient, and include a detailed description of imaging findings and a literature review.

Primary Pulmonary Chordoid Meningioma

Primary pulmonary meningioma is a rare disease, and chordoid meningioma is an uncommon variant of meningioma in the central nervous system (CNS) with a high recurrence rate. We report a case of primary pulmonary chordoid meningioma that presented as a solitary pulmonary nodule (SPN). The SPN was resected by thoracoscopic wedge resection and was revealed to have characteristics of chordoid meningioma. After confirming the absence of a meningioma in the CNS by brain imaging, the nodule was diagnosed as a primary pulmonary chordoid meningioma. The patient remained disease-free after 26 months postoperatively. To our knowledge, this is the third case of primary pulmonary chordoid meningioma to be reported.

Chordoid Glioma of the Third Ventricle, a Rare Tumor with an Unexpected Outcome

Chordoid glioma is a rare tumor of the third ventricle whose imaging features are difficult to distinguish from other more common lesions in this location. There are only 83 cases described so far in the literature. Although gross total resection (GTR) is the treatment of choice, immediate postoperative mortality with this approach can be as high as 29%, and morbidity among survivors can reach 67%. We report a case of a male patient of advanced age, with a third ventricle mass lesion, who presented with a progressive right temporal hemianopia. Imaging was compatible with craniopharyngioma, meningioma or even metastasis. Chordoid glioma was not considered in the differential diagnosis. The patient underwent surgery and GTR was achieved. There were no postoperative complications, and the patient was discharged from the hospital three weeks later. Unexpectedly, two days afterwards, he suffered a major brainstem hemorrhagic stroke and, unfortunately, died.

O glioma cordoide é um tumor raro do terceiro ventrículo, e as suas características imagiológicas são difíceis de distinguir de outras lesões mais comuns nesta localização. Até a data presente, existem apenas 83 casos de gliomas cordoides descritos na literatura. A remoção macroscópica total destes tumores deve ser o tratamento de escolha; no entanto, a mortalidade pós-operatória imediata pode chegar aos 29%, e a morbilidade pode atingir os 67% entre os sobreviventes. Nós descrevemos o caso de um homem idoso com uma lesão tumoral no terceiro ventrículo, que se manifestou com uma hemianopsia temporal direita progressiva. Os exames de imagem eram compatíveis com craniofaringioma, meningioma ou até metástase. O glioma cordoide não foi considerado como uma das hipóteses no diagnóstico diferencial inicial. O paciente foi submetido a cirurgia, tendo-se obtido a remoção macroscópica total. Não houve qualquer complicação no período pós-operatório, e o paciente teve alta hospitalar após três semanas. Inesperadamente, dois dias após a alta clínica, o paciente sofreu um AVC hemorrágico do tronco cerebral, e acabou por falecer.

The value of MRI in diagnosis of chordoid meningioma and differenciation with other meningiomas / 实用放射学杂志

Objective To analyze the MRI features of chordoid meningioma (CM),and to compare with other meningiomas. Methods Clinical and MRI features of 7 patients with CM confirmed by pathology were analyzed retrospectively.Featrues of every MRI sequence were investigated qualitatively.Meanwhile normalized signal ratios of each sequence were analyzed furtherly,including normalized T1 weighted image intensity ratios (NT1 ),normalized T2 weighted image intensity ratios(NT2 ),normalized ADC ratios (NADC)and normalized T1 WI contast enhancement intensity ratios (NCE).Fisher ’s exact test and Dunnett T 3 test were employed to analyze the difference of CM and nonchordoid meningiomas (34 cases of WHO grade Ⅰ meningioma,1 6 cases of nonchordoid WHO grade Ⅱ meningioma,and 5 case of WHO grade Ⅲ meningioma).Results The CM showed hyperintensity on T2 WI and meanwhile other nonchordoid meningiomas demonstrated isointensity,hypointensity or slight hyperintensity on T2 WI.Evaluations of DWI revealed variable signal intensities of CM.But all 7 cases had no obvious restricted diffusion,and increased signal was seen on the ADC map in each patient.The solid parts of CM enhanced markedly after contrast agent injection.Quantitative indicators NT2 , NADC and NCE of CM were higher than other meningiomas(P <0.01),but NT1 showed no statistic difference between CM and other meningiomas (P =0.889).Whether there was cyst,necrosis,orflow void sign on T2 WI,and no matter with or without perifocal edema,dural tail sign and a broader base among meningiomas had no significance in differentiating CM and other meningiomas.Conclusion CM has typical MRI features.The tumors show hyperintensity on T2 WI,especially have no obvious restricted diffusion and enhance markedly after contrast.The measurement of NT2 ,NADC and NCE enables reliable preoperative prediction of the atypical histopathologic diagnosis.

Meningioma cordoide: reporte de dos casos y revisión de la literatura / Cordoid meningioma: report of two cases and review of the literature

El meningioma cordoide es un subtipo infrecuente de meningioma. La mayoría es de gran tamaño y de localización supratentorial. Suelen presentarse clínicamente con síntomas inespecíficos. La Resonancia Magnética de Cerebro muestra una lesión extraaxial compatible con meningioma, siendo la histología y las pruebas de inmunohistoquímica necesarias para realizar el diagnóstico definitivo. La anatomía patológica remeda a la del cordoma ya que se encuentran constituidos por células vacuoladas, epiteloides o fusiformes, dispuestas en cordones o islas dentro de una matriz mixoide. La importancia de conocer esta variante de meningioma radica en su alta tasa de recurrencia, principalmente si la exéresis es incompleta, por lo que son considerados como tumores de mayor agresividad y peor pronóstico. Se presentan dos casos de meningioma cordoide operados en nuestro centro.

Chordoid meningiomas are a rare form of meningioma that, as with most meningiomas, typically present as a bulky, supratentorial tumor. Otherwise, their clinical presentation is non-specific. Brain MRI shows an extra-axial lesion compatible with a meningioma; however, the diagnosis must be confirmed by histopathology and immunohistochemistry. Microscopically, these tumors are composed of spindle or epithelioid, partly-vacuolated cells arranged in nests or cords in a myxoid matrix, resembling a chordoma. Recurrence rates are high following subtotal resection. As such, these tumors are considered a more aggressive variant associated with poorer outcomes. Two patients with a chordoid meningioma treated surgically at our centre, using microsurgical techniques, are presented.

Chordoid Glioma with Intraventricular Dissemination: A Case Report with Perfusion MR Imaging Features

Chordoid glioma is a rare low grade tumor typically located in the third ventricle. Although a chordoid glioma can arise from ventricle with tumor cells having features of ependymal differentiation, intraventricular dissemination has not been reported. Here we report a case of a patient with third ventricular chordoid glioma and intraventricular dissemination in the lateral and fourth ventricles. We described the perfusion MR imaging features of our case different from a previous report.

Expanded Endoscopic Transnasal Approach to the Chordoid Glioma of the Third Ventricle : The First Case Ever Reported

Chordoid glioma of the third ventricle is a rare and challenging tumor to surgery because of its unique anatomical location and its close juxtaposition to the neurovascular structures and hypothalamus. The authors report a case of chordoid glioma of the third ventricle in a 43-year-old woman, who presented with headache and somnolence. The tumor was approached by endoscopic transnasal technique with a favorable result. Histopathologic examination disclosed a neoplastic tissue composed of eosinophilic epithelioid cells, mucinous, periodic acid Schiff-diastase positive, extracellular matrix, and scattered lymphoplasmacytic infiltrates. The best treatment option remains controversial. Customarily, the surgical route to remove chordoid glioma is transcranial; however, the undersurface of the optic chiasm and optic nerves preclude an adequate surgical visualization. In contrast, an expanded endoscopic transnasal approach provides a direct midline corridor to this region without any brain retraction.

Chordoid Glioma Originating in the Intrasellar and Suprasellar Regions: Case Report

Chordoid glioma is a rare, low-grade brain neoplasm typically located in the third ventricle. Herein, we report an unusual case of histologically confirmed chordoid glioma located in the pituitary fossa and suprasellar region, not attached to the third ventricle. A 57-year-old woman presented with a 2-month history of headache and visual disturbance. Magnetic resonance imaging revealed an ovoid mass in the pituitary fossa and suprasellar region, compressing the optic chiasm without involvement of the third ventricle. The tumor showed low signal intensity on T1-weighted images and iso- to high signal intensity on T2-weighted images, with strong and homogenous contrast enhancement. Subtotal resection was performed via the transcranial approach, and the patient subsequently received adjuvant gamma knife radiosurgery. However, the residual mass showed disease progression 5 months after the initial surgery.

Clinical Features and Treatment Outcome of Chordoid Meningiomas in a Single Institute

OBJECTIVE: Meningioma is the second most common primary central nervous system neoplasm. In contrast, chordoid meningioma is rare; due to the paucity of cases, little is known about its clinical features or treatment outcomes. The objectives of this study were to describe the clinical characteristics and outcomes for patients with chordoid meningioma. METHODS: In total, 16 patients, with newly diagnosed chordoid meningioma who underwent surgical excision between 1999 and 2012 were included. We retrospectively evaluated the medical records, radiological findings, and pathological findings. The median follow-up period was 56.5 (range, 3-170) months. The MIB-1 labeling index ranged from 1 to 26.60% (median, 5.04). RESULTS: Simpson grade I, II, and III resections were performed in four, nine, and three patients, respectively. The overall recurrence rate was 37.5%. Overall progression-free survival (PFS) after resection was 94.7 months (95% CI=62.9-126.6). Of the 4 patients with Simpson grade I resection, recurrence occurred in one patient. Among the Simpson grade II and III resection groups, eight patients underwent adjuvant radiation therapy and they showed significantly longer PFS (121 months, 95% CI=82.1-159.9) than the patients who underwent surgery alone (40.5 months, 95% CI=9.6-71.3) by the log-rank test (p<0.05). CONCLUSION: Chordoid meningiomas are difficult to manage and have a high rate of recurrence. Complete resection of the tumor is a key determinant of better outcomes. Adjuvant radiation therapy is recommended, eparticulary when Simpson grade I resection was not achieved.

Glioma cordoide do terceiro ventrículo: descrição de um novo caso / Chordoid glioma of the third ventricle: a new case report

O glioma cordoide é um tumor cerebral raro, recentemente descrito, localizado na região do terceiro ventrículo e com características histológicas, imuno-histoquímicas e ultraestruturais peculiares. Este estudo ilustra um caso de glioma cordoide do terceiro ventrículo em uma paciente de 59 anos de idade.

Chordoid glioma is a recently described and rare brain tumor located in the third ventricular region, with distinctive histological, immunohistochemical and ultrastructural features. The present report describes a case of chordoid glioma of the third ventricle in a 59-year-old female patient.

Chordoid Glioma : A Case Report of Unusual Location and Neuroradiological Characteristics

Since the World Health Organization (WHO) classification for central nervous system neoplasms was declared in 2000, chordoid glioma of the third ventricle has been noted as a newly recognized tumor for central nervous system neoplasms. Although there is not enough universal experience to know the nature of this tumor due to its rarity, the origin of chordoid glioma was guardedly proposed to be the ependymal cells of the third ventricle. Such an idea has been primarily based on the specific location of the tumor, that is, third ventricle, suprasellae, and hypothalamus. However, we report a rare case of histologically confirmed chordoid glioma located in the left thalamus, not attached to any of the midline structures having unusual neuroradiological characteristics.

Chordoid Glioma of the Third Ventricle with Unusual MRI Features

Chordoid glioma is a rare primary brain tumor of the third ventricle, and was recently characterized as a novel tumor entity. Typical radiological features of chordoid gliomas include; a solid, round-to-ovoid, wellcircumscribed, contrast-enhancing mass of the hypothalamus and anterior third ventricle. Despite being classified as WHO grade 2, the third ventricular chordoid glioma has been reported to have a poor clinical outcome because of its anatomical location. The authors report a case of chordoid glioma of the third ventricle in a 48-year-old man with unusual radiological features of cystic component and ill defined circumscription.

Chordoid Glioma: an Uncommon Tumor of the Third Ventricle

Chordoid glioma is an uncommon low-grade tumor of the third ventricle with histologic features of a chordoma and immunolabeling for glial fibrillary acid protein. We present a rare case of a patient with a chordoid glioma of the third ventricle and review the literature regarding this tumor's clinical, radiological and pathologic aspects.

Chordoid Meningioma

A case of chordoid meningioma occurring in a 61-year-old woman who did not have a Castleman's disease is presented. The patient had suffered from headache and motor dysphasia. Laboratory findings are normal. The tumor, located in the left frontal region and associated with peritumoral edema, was totally resected. Surgical specimen revealed a solid mass with irregular surface that measured 3.5 x 4.5 x 4cm. Immunohistochemical staining revealed that the tumor cells expressed epithelial membrane antigen(EMA) focally, but not S-100 protein and glial fibrillary acid protein (GFAP), and the Ki-67 proliferative index of the tumor was 9%. The neoplasm was diagnosed chordoid meningioma of the World Health Organization(WHO) grade II. After total resection, her preoperative headache and dysphasia were disappeared.

Chordoid Glioma in the Third Ventricle: Case Report

Chordoid glioma of the central nervous system is a recently recognized tumor. It is necessary to be differentiated with the craniopharyngioma or other suprasellar masses due to its clinical and radiologic similarities. The authors report a patient of 47-year-old man with a chordoid glioma in the third ventricle who underwent total removal of the tumor. Preoperatively, the patient showed hypersomnia, memory disturbance, bitemporal hemianopsia, drowsy mental status, and striking weight gain(25Kg) during the last 5-6 months. MRI showed huge homogeneous enhancing mass(5x5x6cm), locating in the sellae extending to the third ventricle and corpus callosum. Tumor was removed via interhemispheric transcallosal interforniceal approach. Histopathologic finding was chordoid astrocytoma characteristically showing GFAP(+), EMA(-), Collagen type IV(-), ki-67(-), p53(-) in immunohistochemical stain and epithelioid cells with eosinophilic cytoplasm, mucinous matrix, high cellularity, no mitosis in H & E stain. Postoperatively, a patient recovered normal mental status, sleep pattern, and vision. Postoperative fractionated radiation therapy was done.

Suprasellar Chordoid Glioma Combined with Rathke's Cleft Cyst: Case Report

Chordoid glioma, a recently introduced clinicopathologic entity, is a rare neoplasm occurring mainly in the third ventricle and hypothalamus. The authors had experienced a case of chordoid glioma combined with Rathke's cleft cyst which occurred in the sellar and suparasellar region. Here we report clinical, radiological, and histopathological features of this neoplasm with review of literature

"Chordoid" Meningioma with Systemic Manifestations of Castleman Syndrome: A case report

Chordoid meningioma is a recently established meningeal tumor and is characterized by a chordoma like histologic appearance, peritumoral lymphoplasma cell infiltrates causing systemic manifestations similar to Castleman syndrome and having a good prognosis. We experienced a case of chordoid meningioma in a 25 year-old woman. The patient preoperatively manifested iron-resistant hypochromic microcytic anemia, polyclonal gammopathy with beta-gamma bridging and detected a huge mass in the right temporo-parietal convexity of the brain. Microscopically, the mass was composed of nests and cords of cuboid, partly vacuolated cells in a mucoid matrix, simulating chordoma. The tumor was surrounded by masses of lymphoplasma cells around vessels, many of the plasma cells contained Russell bodies. Ultrastructural findings showed intranuclear cytoplasmic invaginations, microvilli protruding from cytoplasmic surfaces and well formed desmosomes. Some portions of tumor cell surface were covered by stretches of basal lamina.

A Case of Chordoid Meningioma at Right Temporo-Parietal Lobe: Case Report

A large tumor located at right temporoparietal lobe was found in 25-years-old female. The brain CT scan and MR imaging revealed a large multi-lobulated mass which was composed of solid and cystic portions involving right temporoparietal lobe. Preoperative routine laboratory check showed microcytic hypochromic anemia and monoclonal gammopathy with bate-gamma bridging. The mass was removed totally by operation and histologic diagnosis resulted in chordoid meningioma surrounded by massive polyclonal lymphoplasmacellular infiltrates. After the mass was removed, the blood picture of the patient normalized and the patient discharged with neurologically free stage. We report this case with relative literatures.