A 29‐Year‐Old Man with Progressive Short Term Memory Loss

Abstract

A 29-year-old man presented with a 1 year history of memory impairment. He had become somnolent and lethargic for 8 months before seeking medical attention. He needed to sleep over 20 hours per day. The physical examination and neurological examination were unremarkable. RADIOLOGYBrain MRI revealed a 5.7x4.3x4.2 cm in the third ventricle with isosignal intensity on T1W, heterogeneous signal on T2W and FLAIR (not shown). The mass showed intense heterogeneous enhancement on T1W after gadolinium injection. Extension to the suprasellar cistern was demonstrated on sagittal T1W after gadolinium injection ( Figure 1a). There was also thickening and enhancement of the pituitary stalk, suggestive of involvement or origin of hypothalamus. The coronal T1W after gadolinium injection showed separation of the mass from lateral ventricle ( Figure 1b). The inferior wall of the lateral ventricle was displaced upward as well as the septum pellucidum. SURGICAL TREATMENT AND POST OPERATIVE OUTCOMEThe patient underwent biopsy and later bifrontal craniotomy for tumor removal. Intraoperatively, the tumor appeared white and had rubbery to hard consistency. It was located in the third ventricle adhering to lamina terminalis and both thalami. There was low tumor vascularization. The tumor was totally resected. Postoperatively, the patient developed transient diabetes insipidus. There was no evidence of tumor recurrence on follow up MRI after 9 months of surgery. MICROSCOPIC PATHOLOGYIntraoperative squash preparation showed hypercellular smear with epithelioid tumor nests, non-cohesive tumor cells, and 3D papillary-like fragments (Figure 2a). The epithelioid tumor cells contained moderate amount of eosinophilic cytoplasm. They were relatively uniform and possessed round to oval, central or eccentric nuclei, with open chromatin and small nucleoli (Figure 2b). The non-cohesive tumor cells had delicate cytoplasmic process and show similar nuclear features (Figure 2c). Permanent section revealed a well demarcated mass composed of clusters and cords of epithelioid cells in a myxoid stroma with scattered lymphoplasmacytic infiltration (Figure 2d, Figure 2e). Rosenthal fibers are observed in adjacent brain parenchyma. Mitotic figure, necrosis, and vascular proliferation were not present. Higher magnification showed uniform to mildly variate (Figure 2f) epithelioid tumor cells with moderate to ample amount of eosinophilic cytoplasm in a myxoid stroma. Findings on nuclear detail were similar to those present in squash cytology. Immunohistochemically, the tumor cells were diffusely positive to GFAP (Figure 3a), and vimentin (not shown). Focal positivity to CD34 was noted (Figure 3b). Staining for S-100 protein and EMA were negative (not shown). Ultrastructural study on specimen retrieved from paraffinembedded tissue revealed microvilli (Figure 3c) at the luminal surface, and intermediate junctions (not shown). The cytoplasm contained mitochondria, ribosome, lysosome, and dilated rER. Intermediate filaments (Figure 3d) are noted in the cytoplasm of m...

Prognostic factors for recurrence and complications in the surgical management of primary chordoid gliomas: A systematic review of literature

“…Mixed signal intensity was noted in 3 patients. [17, 29, 30] None of the tumors were hypointense. Edema on MRI was noted in 3 patients.…”

“…[13, 19, 26] Of the remaining cases, extent of infiltration was highly variable, ranging from occasional [27, 41] to prominent infiltration. [4, 10, 42, 43] Distribution of tumor infiltrating lymphocytes was also highly variable, with peripheral [21], scattered [30], and focal appearance. [44] Mitoses were consistently low [7, 10, 24, 33, 45–48] or absent [35] when noted.…”

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

“…The tumor tends to occur in adult women (a female to male ratio of 2 to 1) at mean age of 45 years (range, 5-72 years) ( 3 ). Clinical symptoms including headache, nausea, vomiting, visual disturbances, confusion, lethargy, urinary incontinence, ataxia, hypothyroidism, diabetes insipidus, weight loss or weight gain, and behavioral disturbances due to intracranial hypertension, hydrocephalus, compression of the hypophysis, optic chiasm, and hypothalamus by the tumor ( 1 2 3 ).…”

“…Chordoid glioma is classified as grade II tumor according to 2007 World Health Organization (WHO) classification of brain tumors ( 1 ). Approximately 85 cases of chordoid glioma have been reported in English literatures since its first report in 1998 ( 2 ). Chordoid glioma is typically located in the third ventricle or suprasellar region.…”

“…Chordoid glioma is typically located in the third ventricle or suprasellar region. It often extends into the hypothalamus ( 1 2 ). Although chordoid glioma is located within the ventricular system, intraventricular seeding or metachronous tumors along the ventricular wall has not been reported.…”