INTRACRANIAL AND SPINAL DISSEMINATION OF AN ACTH-SECRETING PITUITARY NEOPLASIA - CASE-REPORT AND REVIEW OF LITERATURE

We report a case of a 52-year-old woman presenting with a recurrence o f a large pituitary adenoma with suprasellar extension and an overt Cu shing's clinical picture, five years after successful transsphenoidal treatment. After transfrontal ablation of the tumour, followed by exte rnal radiotherapy, she was asymptomatic for six years before she exhib ited epileptic seizures. A left frontal intracranial neoplasm was diag nosed and removed, and at histological examination it was found to be constituted by a localization of the pituitary ACTH secreting neoplasi a. One month later she exhibited spinal dissemination of the ACTH secr eting neoplasia which was only partially removed. After four months a Magnetic Resonance Image (MRI) revealed recurrence of the intracranial localization and further spinal dissemination. Because of compressive symptoms, spinal masses with the same histologic features, were parti ally removed again in three successive surgical operations. Several me dical treatments for obtaining the control of corticoid excess, caused by the ACTH overproduction, were tried, but none were satisfactory. F inally a bilateral adrenal venous embolization was performed thus obta ining a critical transient fall of serum cortisol. Five months later t he patient died. At necroscopy bilateral adrenal enlargement was found , spinal disseminations were confirmed, and no metastatic lesions were discovered.