Chordoid glioma

Chordoid glioma, also known as chordoid glioma of the third ventricle, is an extremely rare, slow-growing brain tumor classified as a WHO grade 2 neoplasm. It arises almost exclusively in the anterior part of the third ventricle, a fluid-filled cavity deep within the brain. The tumor is believed to originate from specialized glial cells (tanycytes) of the lamina terminalis. Histologically, it is characterized by clusters and cords of epithelioid tumor cells embedded in a mucinous stroma with a prominent lymphoplasmacytic infiltrate, giving it a "chordoid" appearance reminiscent of chordoma. A recurrent PRKCA D463H mutation has been identified as a molecular hallmark of this tumor. Chordoid glioma predominantly affects adults, with a mean age at diagnosis typically in the fourth to fifth decade of life, and shows a slight female predominance. Due to its location in the third ventricle, the tumor frequently causes obstructive hydrocephalus, leading to symptoms such as headaches, nausea, vomiting, visual disturbances, and memory impairment. Patients may also experience endocrine dysfunction including hypothalamic-pituitary axis abnormalities, weight gain, and disturbances in thermoregulation or sleep-wake cycles. Some patients present with psychiatric symptoms or cognitive decline. The primary treatment for chordoid glioma is surgical resection. However, complete removal can be challenging due to the tumor's deep midline location and its tendency to adhere firmly to surrounding hypothalamic structures and blood vessels. Gross total resection, when achievable, is generally considered curative, as recurrence after complete removal is rare. Subtotal resection carries a risk of tumor regrowth. The role of adjuvant radiation therapy remains uncertain and is generally reserved for cases of incomplete resection or recurrence. Overall prognosis is favorable when the tumor can be safely removed, though surgical morbidity related to hypothalamic injury can be significant. Fewer than 100 cases have been reported in the medical literature since the tumor was first described in 1998.

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