Large Hypothalamic Hamartoma With Calcification and Cystic Components in an Adult ―Case Report

A 24-year-old female presented with an unusual case of hypothalamic hamartoma manifesting as seizure. Neuroimaging findings were atypical, showing the large tumor (maximum diameter, 50 mm) with a cystic component and calcification. Surgery was performed and histological examination demonstrated hete...

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Veröffentlicht in:Neurologia medico-chirurgica 2010, Vol.50 (6), p.495-498
Hauptverfasser: Shinya YAMAGUCHI, Satoshi O.SUZUKI, Yoshihiro MATSUO, Toshio UESAKA, Koichiro MATSUKADO, Kenta MASUI, Toru IWAKI
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Sprache:jpn
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Zusammenfassung:A 24-year-old female presented with an unusual case of hypothalamic hamartoma manifesting as seizure. Neuroimaging findings were atypical, showing the large tumor (maximum diameter, 50 mm) with a cystic component and calcification. Surgery was performed and histological examination demonstrated heterotopia. Hamartoma should be considered in the differential diagnosis of a suprasellar, non-enhanced mass attached to the hypothalamus. Excessive unnecessary surgery should be avoided, and intraoperative pathological examination may lead to enhanced assessment and better outcomes. [Introduction]Hypothalamic hamartoma is a non-neoplastic, congenital mass consisting of heterotopic neural tissue that commonly manifests as gelastic seizures and/or precocious puberty in infants. Patients typically present with developmental disturbances because of uncontrollable seizures. Computed tomography (CT) and magnetic resonance (MR) imaging usually show hypothalamic hamartomas as small (diameter, 10-30 mm) round masses3) that are connected to the hypothalamus, with almost identical signal intensity to that of brain parenchyma, with clear demarcation, and no contrast enhancement. 2,3,6,7,14,23,25,27,29)
ISSN:0470-8105