Stereotactic radiosurgery as the initial management option for small-volume hypothalamic hamartomas with intractable epilepsy: a 35-year institutional experience and systematic review

Zhishuo WeiSchool of Medicine, University of Pittsburgh Medical Center;
Center for Image-Guided Neurosurgery, University of Pittsburgh Medical Center; and

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Lena VodovotzSchool of Medicine, University of Pittsburgh Medical Center;
Center for Image-Guided Neurosurgery, University of Pittsburgh Medical Center; and

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Diego D. LuySchool of Medicine, University of Pittsburgh Medical Center;
Center for Image-Guided Neurosurgery, University of Pittsburgh Medical Center; and

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Hansen DengCenter for Image-Guided Neurosurgery, University of Pittsburgh Medical Center; and
Radiation Oncology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania

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Ajay NiranjanCenter for Image-Guided Neurosurgery, University of Pittsburgh Medical Center; and
Radiation Oncology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania

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L. Dade LunsfordCenter for Image-Guided Neurosurgery, University of Pittsburgh Medical Center; and
Radiation Oncology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania

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OBJECTIVE

Young patients with hypothalamic hamartomas (HHs) often present with intractable epilepsy. Currently there are no established management guidelines for HH. The authors retrospectively reviewed their single-institution experience to delineate the role of stereotactic radiosurgery (SRS).

METHODS

Seven patients with HHs (4 females; median age 13.7 years, range 2.5–25 years) with no prior resection underwent SRS between 1987 and 2022. The clinical history, epilepsy profile, radiographic findings, and neurological outcomes were characterized. HH topographical types were classified according to the Régis classification. Outcome measures included Engel seizure classification, HH response, and the need for additional surgical interventions.

RESULTS

All patients had Engel class IV epilepsy. A Leksell Gamma Knife was used to deliver a median margin dose of 18 Gy (range 16–20 Gy) to a median hamartoma volume of 0.37 cm3 (range 0.20–0.89 cm3). Seizure reduction was confirmed in 6 patients, and 2 patients had regression of their hamartoma. Two patients underwent resection and/or laser interstitial thermal therapy after SRS. At follow-up, 1 patient was seizure free, 4 patients achieved Engel class II, 1 patient had Engel class III, and 1 patient had Engel class IV seizure outcomes.

CONCLUSIONS

SRS as the initial management option for HH was associated with a low risk of adverse effects. In this institutional series reviewing small-volume HHs treated with SRS, no adverse radiation effect was detected, and the majority of patients experienced seizure reduction. SRS should be considered as the first-line treatment for seizure control in patients with small-volume HHs.

ABBREVIATIONS

AED = antiepileptic drug; CPP = central precocious puberty; HH = hypothalamic hamartoma; LITT = laser interstitial thermal therapy; SRS = stereotactic radiosurgery.
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Figure from Candela-Cantó et al. (pp 61–70).

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