A 42 year-old man presented in 1994 with a two-week history of headaches, drowsiness and confusion. There had been reduced libido and weight gain for 2 years. There was testicular atrophy and morbid obesity, but no neurological abnormality. CT scan showed a large, partially calcified, suprasellar tumour extending up into the third ventricle. Downward displacement of the posterior clinoid was identified with features of haemorrhage peripherally. MRI scan showed tumour of mixed signal intensity, with some vascular features. He underwent fronto-temporal craniotomy and extensive biopsy. The histological diagnosis was cavernous haemangioma.
His postoperative progress was characterised by hyperphagia with progressive weight gain. There were recurrent admissions with dehydration, which were attributed to a poor thirst drive. Overt diabetes insipidus was excluded. Endocrine assessment indicated secondary hypogonadism, with pituitary function otherwise preserved. Serial MRI scanning over nine years showed no significant change in the size of the suprasellar and hypothalamic cavernous haemangioma.
Cavernous haemangiomas are vascular malformations of the CNS composed of dilated thin-walled capillaries with simple endothelial lining. Residua of previous haemorrhage including haemosiderin-laden macrophages may be seen. To our knowledge, this is the first documented case where hypothalamic syndrome and pituitary dysfunction resulting from cavernous haemangioma have been described.
22 - 24 Mar 2004
British Endocrine Societies