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Neuroradiologic study of hamartomas of the tuber cinereum and hypothalamus
Insights
Hamartoma of the tuber cinereum and hypothalamus in children can present with precocious puberty or seizures. These rare tumors require careful diagnosis, especially when calcified.
Area of Science:
- Pediatric Neurology
- Neuro-oncology
- Endocrinology
Background:
- Hamartomas of the tuber cinereum and hypothalamus are rare tumors.
- They can present with diverse neurological and endocrine symptoms in children.
Purpose of the Study:
- To report five pediatric cases of hamartoma of the tuber cinereum and hypothalamus.
- To describe their clinical presentations, imaging findings, and diagnostic challenges.
Main Methods:
- Retrospective case series analysis.
- Review of clinical data, neuroimaging, and histopathological findings for five pediatric patients.
Main Results:
- Five cases (four confirmed histologically) aged 2-12 years were identified.
- Three patients presented with precocious puberty, with characteristic basal cistern hamartomas.
- One case involved a hypothalamic hamartoma presenting with hyponatremia and seizures; another had a calcified suprasellar mass mimicking craniopharyngioma.
Conclusions:
- Hamartomas of the tuber cinereum and hypothalamus are important differential diagnoses in children with precocious puberty or hypothalamic dysfunction.
- Calcification can complicate diagnosis, potentially mimicking other suprasellar tumors.
Abstract:
Five cases, four histologically proven, of hamartoma of the tuber cinereum and hypothalamus in children are reported. The ages of the patients range from 2 to 12 years. Three cases had pubertas praecox, and in all of these the hamartoma was located in the basal cistern between the chiasm and pons and had a collar button shape and size typical of hamartoma of the tuber cinereum. In the third case, a huge calcified mass in the suprasellar region was initially thought to be craniopharyngioma. The fourth case had a hamartoma within the substance of the hypothalamus and presented with hyponatremia and temporal lobe seizures.