Hypothalamic hamartoma

Q: What is the most likely diagnosis? show answer

A: Hypothalamic hamartoma.

Q: What is the main differential? show answer

A: Hypothalamic-chiasmatic glioma, although enhancement is often present and involvement of the chiasm is common.

Q: How do children with hypothalamic hamartomas present? show answer

A: Gelastic seizures and central precocious puberty are the classical presenting features.

Q: How are these lesions divided? Which one would this be? show answer

A: Sessile or pedunculated. This mass is pedunculated, and it projects (hangs) down into the suprasellar cistern.

Q: What is the typical signal intensity of these lesions? show answer

A: Largely follows grey matter, although they are usually somewhat hyperintense to grey matter on T2 weighted images. They do not enhance.

Q: How are these lesions treated? show answer

A: Children with central precocious puberty can be treated medically. Intractable epilepsy can respond to surgery (resection or disconnection, depending on the morphology of the tumour) and this can be performed via an open craniotomy or more recently via an endoscope.