Abstract
Hypothalamic hamartoma (HH) is assumed to he made of normal neuronal tissue ectopically located, and is often associated with true precocious puberty (PP). Its recognition is of great importance as this tumor does not require irradiation and is generally not invasive. Diagnosis is hased on the following criteria : 1) the localisation to the floor of 3rd ventricle; 2) the CT scan appearance as a mass isodense to gray matter before and after injection of contrast media; 3) the absence of elevated circulating levels of hCG and α-fceto-protein; 4) the lack of extension on follow-up. The contribution of MR was investigated in 7 cases (6 with PP and 1 with gelastic epilepsy). In 6 out of 7 cases the tumor signal was compared to that of gray matter : 1) it was identical on T1 weighted images; 2) but it was superior on T2 indicating some structural difference. A hamartoma like structure was found at hiopsy in one of these cases. The last case showed a signal intensity identical to normal cerebral tissues.
In conclusion, these MR data provide a new diagnostic tool in addition to the other biological, clinical and CT scan data. They also suggest that HH might have a structure different than normal brain tissues.
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Brauner, R., Barral, V., Rappaport, R. et al. MAGNETIC RESONANCE (MR) AS AN ADDITIONAL DIAGNOSTIC TOOL FOR HYPOTHALAMIC HAMARTOMA. Pediatr Res 20, 1188 (1986). https://doi.org/10.1203/00006450-198611000-00088
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DOI: https://doi.org/10.1203/00006450-198611000-00088