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 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.

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.

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 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