Presentation
This patient presented with seizures and had history of delayed growth.
Patient Data
IV-enhanced MRI with MR spectroscopy shows:
A rather sizable well defined mass, originating from the tuber cinereum, exhibiting homogenous isointense signal to the grey matter in T1 and T2, and shows no significant enhancement in the post contrast study.
In MR spectroscopy, there is elevation of the Cho levels with reversal of the NAA/Cho ratio.
Those MRI criteria are highly suggestive of tuber cinereum hamartoma.
Case Discussion
Tuber cinereum hamartomas are not true tumors, they are congenital malformations caused by normal neurons and glial cells from the tuber cinereum in an abnormal location.
Clinically, they usually present by early precocious puberty and gelastic seizures; less commonly by developmental delay. There is no correlation between the clinical symptoms and the size of the hamartoma.
On MR studies, the tuber cinereum hamartoma appears as a round suprasellar mass with variable size from a few millimeters to 5 cm in diameter. The signal is similar to that of grey matter on all sequences and no enhancement after gadolinium injection. MR spectroscopy can be normal or shows a slight decrease in N-acetyl aspartate and an increase in choline and myoinositol. Non-enhancing hypothalamic-chiasmatic glioma is the only differential diagnosis of hamartoma.
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