Chordoma

Case contributed by Khaloud Alghamdi
Diagnosis certain

Presentation

Progressive visual disturbance, with bitemporal hemianopsia on examination.

Patient Data

Age: 35 years
Gender: Female
ct

A lesion, visible in the sella turcica extending posteriorly, causing destruction of the clivus. Inferiorly. it extends into the sphenoidal sinus, and laterally in both cavernous sinuses. It has a suprasellar extension up to the floor of the third ventricle and displays multiple calcifications. Considering morphology and location, a suspicion of chordoma was raised.

mri

Corroborating the CT findings, the sellar/suprasellar lesion is a destructive process to neighbor structures (cliffs). In addition, it shows heterogeneous enhancement.

The lesion is heterogeneous,  with predominantly high signal intensity in T2 and low signal intensity in T1, with areas of with interspersed hyperintensity in the latter.  Overall, the appearance represents intra-lesional bleeding and areas of cystic degeneration.

There is a significant mass effect in the surrounding area, with a lobulated extension of the tumor towards the prepontine cistern, with indentation of the pons. The superior-anterior extension is also causing displacement of the optic chiasm, tract, and the optic nerve. The lateral aspect is invading cavernous sinuses bilaterally, and displacement of the left cavernous carotid. The tumor is causing mild mass effect on the left mesial temporal structure.

Case Discussion

Chordoma growth causes significant symptoms, necessitating surgical approaches often. The location and morphology of the lesion are key in recognizing the differential diagnosis. The patient underwent partial resection of clival chordoma, followed by radiotherapy. The diagnosis of chordoma was confirmed by histopathologic analysis of the resected specimen.

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