Hypothalamic hamartoma

Case contributed by Dr Aruna Pallewatte

Presentation

14 month old male child presented with precocious puberty, generalized hyperpigmentation and accelerated growth. Bone age was advanced. There was no history of epilepsy.

Patient Data

Age: 15 months
Gender: Male
MRI

A well defined midline lesion measuring 2.3cm x1.7cmx1.9cm seen in the suprasellar region projecting into the suprasellar cistern.It shows iso signal intensity in T1 W images, slightly heterogeneously high signal in T2W and FLAIR images. No significant post contrast enhancement. Gray white differentiation of the cerebral hemisphere is normal.No grey matter heterotopia or microgyria. Corpus callosum and pituitary gland are normal.
 

Case Discussion

Hypothalamic harmatoma 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  tubercinerum 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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Case information

rID: 28460
Case created: 26th Mar 2014
Last edited: 19th Jun 2017
Inclusion in quiz mode: Included

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