Presentation
Dilated right pupil for a few months. Problems with blurry vision for 3 weeks. 2 weeks of declined coordination, speech, behavior with emotional lability, insomnia, strange speech and gait difficulties.
Patient Data
Lobulated mass within the suprasellar region centered around the hypothalamus in the floor of the third ventricle, involving the optic chiasm with mass like expansion though sparing the retro-orbital posterior nerves. On the right the mass is intimately related to the anterior cerebral artery with preservation of flow void on susceptibility weighted imaging. Posteriorly the mass abuts the cerebral peduncle without evidence of signal change. This mass avidly enhances, with restricted diffusion. Adjacent edema involving the lateral third ventricular walls, extending anterior to the anterior commissure and laterally and posteriorly within the posterior globus pallidi and abutting the cerebral peduncles. No blooming artefact on susceptibility weighted imaging to suggest presence of calcification or blood products. Loco-regional mass effect with distortion / narrowing of the third ventricle. The pituitary gland appears separate from the lesion.
Separate enhancing mass involve the posterior medial right thalamus and posterior third ventricular wall with some extension to the pineal region and inferiorly into the midbrain tegmentum. Associated with perilesional edema which involves the midbrain tegmentum, greater on the right than left, and the periaqueductal grey matter. Near complete obliteration of the entrance of the cerebral aqueduct which probably accounts for the marginal dilatation of the lateral and third ventricles.
Prominent retrocerebellar CSF appearing space with a normal vermis and fourth ventricle causing anterior displacement of the right cerebellar hemisphere, likely arachnoid cyst.
The remaining grey-white matter differentiation is preserved. Normal expected flow voids of the visualized cerebral vasculature. Mucous retention cyst within the right maxillary sinus. No proptosis.
Case Discussion
Large hypothalamic mass with involvement of the optic chiasm together with a separate lesion of similar characteristics involving the posterior right thalamus with extension into midbrain tegmentum. Both are associated with adjacent edema and locoregional mass effect as described, the second lesion resulting in near complete effacement of the aqueduct and early ventricular dilatation. Overall appearance would be most suggestive of a bifocal germ cell tumor (germinoma) - histologically confirmed in this case.
Spinal imaging should be performed to assess for drop metastases.
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