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Hypothalamic hamartoma. Report of two cases
1Department of Neurosurgery, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
Insights
This study presents two cases of hypothalamic hamartoma, a rare brain tumor, in young boys. Surgical removal impacted hormonal balance and seizure control, highlighting the lesion
Area of Science:
- Neuro-oncology
- Pediatric Neurology
Background:
- Hypothalamic hamartomas are rare congenital tumors.
- They can present with diverse neurological and endocrine symptoms.
Observation:
- Two pediatric cases of hypothalamic hamartoma are detailed.
- Symptoms of precocious puberty and epileptic seizures began at age two.
- Imaging revealed suprasellar mass lesions connected to the hypothalamus.
Findings:
- Surgical intervention influenced endocrinological status and seizure control.
- Pathology confirmed well-differentiated neuronal and glial cells.
- Immunohistochemistry showed neuropeptides in one case, but not the other.
Implications:
- Surgical management of hypothalamic hamartoma requires careful consideration of potential sequelae.
- Understanding the diverse biological characteristics is crucial for treatment planning.
- Further research into the role of neuropeptides in hamartoma pathogenesis is warranted.
Abstract:
Two cases of hypothalamic hamartoma are presented. The first patient was a 4-year-old boy with precocious puberty, and the second was a 6-year-old boy with epileptic seizures. In both patients, clinical symptoms and signs appeared at the age of 2 years and progressed thereafter. Computerized tomography and magnetic resonance imaging in both cases disclosed a suprasellar mass lesion in continuity with the hypothalamus. Removal of the lesions affected the endocrinological status and/or seizure control. Pathological examination revealed the lesions to be composed of well-differentiated neuronal and glial cells. Immunohistochemical study demonstrated the presence of beta-endorphin, corticotropin-releasing factor, oxytocin, and neurofilament protein (210 kD) in the neuronal cells of the first patient, but no neuropeptides were detected in the second. Electron microscopic examination on the second patient disclosed the presence of many nonmyelinated and some myelinated neuronal processes containing dense-core and clear vesicles. The morphological characteristics and the role of surgery for this lesion are discussed.