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Pediatric neurosurgery · Jan 2011
Case ReportsSynchronous occurrence of a hemorrhagic hypothalamic hamartoma and a suprasellar teratoma.
- Sai Kiran Narayanam Anantha NA Department of Neurosurgery, Sri Sathya Sai Institute of Higher Medical Sciences, Bangalore, India., Nandita Ghosal, Sumit Thakar, and Alangar S Hegde.
- Department of Neurosurgery, Sri Sathya Sai Institute of Higher Medical Sciences, Bangalore, India.
- Pediatr Neurosurg. 2011 Jan 1; 47 (6): 430-5.
AbstractHypothalamic hamartomas have been reported to coexist with lesions like Rathke's cleft cyst and arachnoid cysts in the suprasellar or temporo-sylvian regions. This is the first report in indexed literature describing its association with a suprasellar teratoma. A 7-year-old girl presented with long-standing precocious puberty and generalized tonic-clonic seizures and recent-onset raised intracranial pressure. MRI done prior to the onset of symptomatic raised intracranial pressure revealed 2 distinct lesions in the suprasellar region. One was a midline, pedunculated lesion arising from the hypothalamus, with evidence of an old bleed within it. A separate lesion, with a wide base near the tuberculum sellae and a posteriorly directed conical tip, was noted in an adjacent sagittal cut. CT scan done at the time of admission demonstrated a re-bleed in the suprasellar region with blood in the lateral and third ventricles and gross hydrocephalus. The child was taken up for a ventriculoperitoneal shunt followed by complete excision of the lesions. Histopathologic examination confirmed the pedunculated lesion to be a hypothalamic hamartoma with evidence of hemorrhage, and the other to be a mature teratoma. Postoperative MRI confirmed complete excision of both the lesions. The child reported regression of precocious puberty and remained seizure-free until the last follow-up 6 months after surgery. A hypothesis based on a dysontogenetic mechanism is discussed to explain the unusual occurrence of the dual, seemingly unrelated pathologies. Hemorrhage into the hamartoma was an added oddity in this case.Copyright © 2012 S. Karger AG, Basel.
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