Craniopharyngiomas are tumours of the squamous epithelium, which arise in the sellar and/or suprasellar regions from embryonic squamous cell rests of the involuted hypophyseal-pharyngeal duct. They account for approximately 3% of intracranial neoplasms, present with symptoms relating to pressure effects on adjacent organs and often have an unpredictable behaviour. Corticotroph adenomas, usually measuring a few millimeters in diameter, are found in 10-12% of surgically removed pituitary adenomas. We describe a case of Cushing' s disease associated with a previously unsuspected craniopharyngioma.
A 54-year old male presented in March 2001 with a 3-year history suggestive of Cushing' s syndrome. Subsequent biochemical studies confirmed pituitary-dependent hypercortisolaemia. He had no visual field defects and neurological examination, apart from a mild proximal myopathy, was normal. The brain MRI revealed a 12 millimeter poorly enhancing nodular mass lesion of the pituitary extending into the suprasellar cistern and in contact with the optic nerves. In September 2001 he underwent transsphenoidal removal of the tumour. The histology showed an ACTH-containing pituitary adenoma and a craniopharyngioma cyst. Post-operative evaluation disclosed cure of the Cushing' s. In May 2002 the MRI showed no residual tumour and possibly a small amount of residual craniopharyngioma around the chiasm. Currently, the patient is on hydrocortisone replacement therapy with no evidence of recurrence of the Cushing' s disease or of the craniopharyngioma.
No other case of a histologically proven coexistent corticotroph tumour and a craniopharyngioma has been reported. Although this may be an incidental finding, common, but as yet unclarified, pathogenetic mechanisms cannot be excluded.
22 - 24 Mar 2004
British Endocrine Societies