Tuber cinereum hamartoma

Case contributed by Kanwal Laique
Diagnosis almost certain

Presentation

Central precociuous puberty

Patient Data

Age: 2 years
Gender: Male
mri

Solitary well delineated small pedunculated lesion arising from tuber cinereum projecting inferiorly into the suprasellar cistern. It is returning iso-intense signal to the grey matter on T1WI and T2WI without enhancement on post contrast study. 

Case Discussion

Tuber cinereum hamartoma / hypothalamic hamartoma is a non-neoplastic heterotopic overgrowth of normal nervous tissue. It clinically manifests as precocious puberty, gelastic seizures, cognitive malfunction or epilepsy/ neurobehaviour disorder. It has two morphological types: sessile and pedunculated. It is made up of grey matter hence they have a similar imaging appearance of the cortex. A close differential diagnosis is a hypothalamic-chiasmatic glioma, which returns different signal intensity and exhibits post-contrast enhancement. Patients with central precocious puberty are treated medically, however, intractable seizures are subjected to surgery, stereotactic radiofrequency thermoablation or gamma knife radiosurgery.

How to use cases

You can use Radiopaedia cases in a variety of ways to help you learn and teach.

Creating your own cases is easy.