Hypothalamic hamartoma

Case contributed by Aruna Pallewatte
Diagnosis almost certain

Presentation

Precocious puberty, generalized hyperpigmentation and accelerated growth. Bone age was advanced. There was no history of epilepsy.

Patient Data

Age: 15 months
Gender: Male

Note: This case has been tagged as "legacy" as it no longer meets image preparation and/or other case publication guidelines.

mri

A well-defined midline lesion in the suprasellar region projecting into the suprasellar cistern. It shows iso signal intensity in T1 weighted images and slightly heterogeneously high signal in T2W and FLAIR images. No significant post-contrast enhancement. Grey-white differentiation of the cerebral hemisphere is normal. No grey matter heterotopia or microgyria. The Corpus callosum and pituitary gland are normal.

Case Discussion

Hypothalamic hamartoma is a  relatively rare congenital lesion consisting of non-neoplastic heterotrophic grey and white matter with a varying degree of differentiation. It occurs at the tuber cinereum of the hypothalamus on the floor of the third ventricle.

The patients usually present with precocious puberty, accelerated growth, gelastic seizures, behavioral abnormalities etc. Our patient presented with precocious puberty and increased body height but no seizures. 

Contributed by Dr. Aruna Pallewatte and Dr. Umakanthan Nadaraja

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