Hypothalamic hamartoma (HH) is a dysplastic lesion fused with hypothalamus and followed by epilepsy, precocious puberty and behavioral disorders. Up to 50% of patients become free of seizures after surgery, but various complications occur in 1/4 of cases. Radiofrequency thermocoagulation, laser interstitial thermal therapy and stereotactic radiosurgery (SRS) are alternative treatment options.

OBJECTIVE

To define the indications for SRS in patients with HH and to clarify the irradiation parameters.

MATERIAL AND METHODS

Twenty-two patients with HH and epilepsy underwent SRS at the Moscow Gamma-knife Center. A retrospective analysis included 19 patients with sufficient follow-up data. Median age of patients was 11.5 years (range 1.3–25.8). The diameter of irradiated HHs ranged between 5.5 and 40.9 mm. In 8 (36%) cases, the volume of hamartoma exceeded 3 cm³. Mean prescribed dose was 18±2.0 Gy, mean prescribed isodose – 48±4.2%. Median follow-up period was 14.8 months (range 3.4 - 96.1).

RESULTS

Three (15.8%) patients were free of seizures. One patient (5.3%) improved dramatically after treatment with compete resolution of generalized seizures and experienced only rare emotional seizures (Engel IB). Eleven (57.8%) patients reported lower incidence of seizures. Severity and incidence of seizures were the same in 4 patients (21.1%). The best results were achieved in mean target dose over 20–22 Gy, minimal target dose over 7–10 Gy, covering by the prescribed dose of at least 70-80% of hamartoma volume, as well as in patients with the prescribed dose of 12 Gy delivered to almost entire volume of tumor. None patient had any complications after SRS.

CONCLUSION

SRS is safe regarding neurological, endocrine or visual disturbances. Careful patient selection for SRS makes it an effective option for HH-related epilepsy. The best candidates for SRS are children with seizures aged over 1 year, hamartoma <3 cm³ and area of fusion with hypothalamus <150 mm².