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Hypothalamic hamartoma: a report of 2 cases
R S Markin1, L G Leibrock, C A Huseman
1Department of Pathology, University of Nebraska Medical Center, Omaha.
Pediatric Neuroscience
|January 1, 1987
Summary
Hypothalamic hamartomas in two patients caused precocious puberty. Surgical removal partially reduced luteinizing hormone (LH) response, indicating a link between these tumors and early puberty onset.
Area of Science:
- Neuroendocrinology
- Pediatric Endocrinology
- Pediatric Neurosurgery
Background:
- Hypothalamic hamartomas are rare congenital tumors.
- Isosexual precocious puberty involves early onset of secondary sexual characteristics.
- The exact mechanism linking hypothalamic hamartomas to precocious puberty is not fully understood.
Observation:
- Two patients with hypothalamic hamartoma presented with isosexual precocious puberty.
- Luteinizing hormone-releasing hormone (LHRH) challenge revealed pubertal LH responses in both.
- Computed tomography (CT) showed retrosellar mass lesions; electron microscopy ruled out neurosecretory granules.
Findings:
- Subtotal removal of hamartomas led to decreased LH responsiveness to LHRH in both patients.
- Postoperative FSH response to LHRH varied, with one case showing exaggerated prepubertal levels.
- Histopathology confirmed mature neurons and neuroglial tissue without dense core granules.
Implications:
- This suggests a potential role for hypothalamic hamartomas in the pathogenesis of central precocious puberty.
- Surgical intervention may modulate the neuroendocrine axis controlling puberty.
- Further research is needed to elucidate the precise mechanisms involved.