Synchronous Occurrence of a Hemorrhagic Hypothalamic Hamartoma and a Suprasellar Teratoma

Abstract

Hypothalamic 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.

Hypothalamic hamartoma simulating a suprasellar arachnoid cyst: resolution of precocious puberty following microsurgical lesion resection

“…Sai Kiran et al 47 proposed bleeding as a possible etiology for cyst formation. In a developmental sense, this hypothesis seems to favor the abnormal proliferation theory responsible for the cystic degenerative process involved in giant cystic HHs as reported by Prasad et al 42 The vascular etiology of cystic changes could be related to the anatomical proximity of a perforating hypothalamic artery described by Sherwin et al 48 Dorfer et al proposed that factors determining cystic degeneration include a delicate balance between growth of the mass and the angiogenic potential providing perfusion.…”