FIBROSING PSEUDOTUMOR OF THE SELLA AND PARASELLAR AREA PRODUCING HYPOPITUITARISM AND MULTIPLE CRANIAL NERVE PALSIES

WE PRESENT AN unusual patient with a medical history of a fibrosing ps eudotumor of the left orbit that had been stable for 8 years who prese nted with acute anterior hypophyseal failure. During the next 10-month period, sequential magnetic resonance scans showed a rapid growth of a plaque-like sellar and parasellar mass extending into the right cave rnous sinus, right Meckel's cave, along the dural surfaces of the cliv us, dens, and body of the second cervical vertebra. A transsphenoidal biopsy revealed sphenoid and intrasellar pseudotumor that invaded the adenohypophysis and had microscopic features identical to those of the previously excised orbital pseudotumor. Rapid growth of the pseudotum or continued despite a course of radiotherapy. Palsies of cranial nerv es V and VI and of the sensory root of the cranial nerve VIII develope d on the right side. Steroid therapy was associated with improvement o f the cranial nerve palsies. This is the first report of a sellar fibr osing pseudotumor producing not only anterior hypophyseal failure, but also cranial nerve dysfunction secondary to plaque-like extension int o the cavernous sinus, Meckel's cave, and cranial base dura. This intr acranial plaque-like extension of a fibrous pseudotumor corresponds to a hypertrophic intracranial pachymeningitis, which is a rare, previou sly described phenomenon associated to the syndrome of multifocal fibr osclerosis.