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Query: UMLS:C0017638 (glioma)
 30,880 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Chordoid glioma of the third ventricle constitutes a rare, very recently recognized histological entity. Most reports of this neoplasm, focused on its distinct histological features, have hypothesized about a probable origin of the lesion at the third ventricle floor and/or the lamina terminalis. We report on a new case, presenting neuroradiological and intraoperative pictorial evidences of the tumoral attachment, limited to the chiasm-lamina terminalis junction. A 53-year-old woman debuted with acute symptoms of obstructive hydrocephalus, visual disturbances and confusion. MRI investigation showed a large solid-cystic third ventricle mass bulging through the lamina terminalis and ventricular floor. After placing a ventriculoperitoneal shunt, the tumor was completely removed through a trans-lamina terminalis approach. A tight tumoral attachment to the junction of the posterior chiasm to the lamina terminalis was identified and dissected. No other adhesions to the third ventricle boundaries were found. A chordoid glioma was diagnosed on histological examination. One year after the surgical procedure the patient does not present new neurological deficits, and there are no signs of tumoral regrowth on the follow-up postoperative MRI. Chordoid glioma should be included in the differential diagnosis of third ventricle tumors. Preoperative neuroradiological suspicion of this lesion should alert the neurosurgeon about the presence of a tight tumoral adherence at the level of the chiasm-lamina terminalis junction. The trans-lamina terminalis approach provides a suitable route for an early control of this attachment under direct vision, allowing a safe dissection of the mass from the third ventricle.
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PMID:Chordoid glioma of the third ventricle attached to the optic chiasm. Successful removal through a trans-lamina terminalis approach. 1859 29

We report two new cases of chordoid glioma of the third ventricle and review all previously published cases of this rare tumor with regard to presentation, optimum management, and outcome. Two new cases are reported with the radiological and histopathologic findings. We searched and cross-referenced PubMed and published reports of this tumor to retrieve an additional 51 cases of this tumor, which were then analyzed according to a proforma. Chordoid glioma has consistent radiological features, although some atypical elements including a cystic component are well described. The insidious presentation and morbidity of attempted surgical resection combine to give high overall morbidity. There is a high incidence of postoperative thromboembolic disturbance. Adjuvant radiosurgery has a promising role. The morbidity of this condition is likely to remain high but may be minimized by a less aggressive surgical approach together with surveillance and early use of radiosurgery.
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PMID:Chordoid glioma: ten years of a low-grade tumor with high morbidity. 2080 39

Chordoid glioma is a rare, slowly growing tumor of the CNS, which is always located in the third ventricle of adults. Chordoid glioma has classic histological features consisting of clusters and cords of epithelioid tumor cells embedded within a mucinous stroma with rich lymphoplasmacytic infiltrate. The important distinctive immunohistochemical feature of this neoplasm is strong and diffuse reactivity for GFAP. Here, we report four cases of chordoid glioma that occupied the anterior portion of the third ventricle or suprasellar region. These four cases were all adult females with almost typical clinical, radiological, histologic and immunohistochemical characteristics of chordoid glioma. However, in one case there was an unusual histologic finding with regard to the papillary region. In this region, elongated tumor cells were observed radiating toward a central vessel to form characteristic papillary structures. Immunohistochemically, three cases showed strong reactivity for GFAP, and one exhibited weak reactivity. All cases were focally positive for epithelial membrane antigen, CD34 and D2-40, but negative for neurofilament protein (NFP). Several ultrastructural investigations have supported the ependymal origin of chordoid glioma. In some cases of immunoreactivity for NFP, some authors have supposed that chordoid glioma originates from a multipotential stem cell with glial and neuronal cell differentiation. With regard to the present four cases with immunoreactivity for D2-40 (an ependymal marker) and CD34 (undifferentiated neural precursors) and based on previously published data, we considered that the majority of chordoid gliomas had an ependymal origin, and that a small minority might have originated from a multipotential stem cell having ependymal and neuronal cell differentiation.
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PMID:Chordoid glioma of the third ventricle: four cases including one case with papillary features. 2271 6

Chordoid glioma of the third ventricle is considered to be a benign glial tumor located exclusively in the mid-anterior portion of the third ventricle near the hypothalamus and optic nerves, with the histological features of a chordoma and immuno-labeling for glial fibrillary acidic protein. Unfortunately, the clinical outcome of chordoid glioma has been poor, even in patients receiving gross total or partial removal with or without radiotherapy. Three cases of chordoid glioma of the third ventricle were treated with less invasive microsurgery for pathological diagnosis or partial removal without neuro-endocrinological complication, followed by gamma knife radiosurgery using a lower marginal dose for the optic nerves and hypothalamus. Gamma knife radiosurgery was performed after open biopsy in two patients, and after partial removal in the third patient using a lower marginal dose of 10.5 to 12 Gy. Serial magnetic resonance imaging revealed gradual decrease or at least no change in the tumor size, without significant complication at follow up 70 and 66 months later in two cases. The third patient accidentally died 13 months after gamma knife treatment. We conclude that low dose gamma knife radiosurgery after less invasive microsurgery is both safe and effective for the control of chordoid glioma of the third ventricle over a very long follow-up period.
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PMID:Therapeutic approach to chordoid glioma of the third ventricle. 2361 18

Chordoid glioma of the third ventricle is a rare neuroepithelial tumor characterized by a unique histomorphology and exclusive association with the suprasellar/third ventricular compartment. Variously interpreted as either astrocytic- or ependymal-like, and speculatively ascribed to the lamina terminalis/subcommissural organ, its histogenesis remains, nevertheless, unsettled. Here, we report on a suprasellar chordoid glioma occurring in a 52-year-old man. Although displaying otherwise typical morphological features, the tumor was notable for expression of thyroid transcription factor 1, a marker of tumors of pituicytic origin in the context of the sellar region. We furthermore found overlapping immunoprofiles of this example of chordoid glioma and pituicytic tumors (pituicytoma and spindle cell oncocytoma), respectively. Specifically, phosphorylated ribosomal protein S6, a marker of mTOR pathway activation, was expressed in both groups. Based on these findings, we suggest that chordoid glioma and pituicytic tumors may form part of a spectrum of lineage-related neoplasms of the basal forebrain.
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PMID:Suprasellar chordoid neoplasm with expression of thyroid transcription factor 1: evidence that chordoid glioma of the third ventricle and pituicytoma may form part of a spectrum of lineage-related tumors of the basal forebrain. 2593 49

Chordoid glioma (CG) is a rare central nervous system neoplasm (WHO grade II) of uncertain origin whose typical localization is in the anterior part of the third ventricle. Its clinical, radiological, and histological features may vary and furthermore mimic other kind of benign lesions usually associated with a better outcome. We report a case of a 43-year-old female who underwent gross total removal of a lesion of the third ventricle causing hydrocephalus. The imaging studies and the intraoperative examination led at first to a hypothesis of meningioma. Early surgical and neurological outcomes were good. The patient underwent multiple complications related to hypothalamic dysfunctions and thrombohemorragic issues and eventually died because of systemic infections. Definitive examination was of chordoid glioma of the third ventricle. Reviewing literature, we evaluated possible pitfalls in radiological and histological diagnosis as well as in surgical and medical treatment of CGs. Despite their benign presentation, a high incidence of multiple possible severe complications is reported. Early alertness and combined treatment strategies could improve overall CGs treatment strategies.
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PMID:Chordoid glioma: a rare radiologically, histologically, and clinically mystifying lesion. 2601 8

Chordoid glioma is a rare tumor that often originate from the third ventricle. We reported two cases of choroid glioma. Although calcification is a rare findings, dot-like calcification was present on one case. On contrast images, one case demonstrated strong, homogeneous enhancement. On MR spectroscopy (MRS) images, the tumor showed high choline (Cho), low N-acetyl aspartate (NAA) and Cho/Cr=2.38, NAA/Cr=1.19, NAA/Cr=1.19. This might mimic a low-grade tumour. MRS offers additional information and facilitate differential diagnosis for chordoid glioma.
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PMID:The radiological findings of chordoid glioma: report of two cases, one case with MR spectroscopy. 2627 47

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.
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PMID:Chordoid Glioma with Intraventricular Dissemination: A Case Report with Perfusion MR Imaging Features. 2679 26

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.
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PMID:Expanded Endoscopic Transnasal Approach to the Chordoid Glioma of the Third Ventricle : The First Case Ever Reported. 2784 80

We report the case of a 63-year-old healthy patient who was admitted for surgery of a suprasellar tumor with extension to the optic chiasm responsible of visual disturbance. Histopathological examination revealed a tumoral proliferation composed of epithelioid cells without atypia arranged in cords in a mucinous matrix surrounded by some lymphocytic inflammatory infiltrates. On immunohistochemistry, the neoplastic cells strongly expressed GFAP and CD34, a weak expression of EMA, an expression of TTF1 without immunoreactivity for brachyury. Ki-67 labeling index was low around 1%. The diagnosis of chordoid glioma was made. Surprisingly, tumor cells expressed IDH1R132H but molecular analysis did not reveal any mutation of IDH1/2 genes. There was no expression of p53 but high overexpression of EGFR. Chordoid glioma is a rare and low-grade entity. The precise histogenesis remains debated. Our case is unusual because of the infiltration of the optic chiasm and because of the immunoexpression of IDH1R132H without underlying mutations of IDH1/2 genes.
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PMID:[A case report of chordoid glioma with unusual features: Immunohistochemical and molecular findings and differential diagnoses]. 2828 12


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