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insuf ciency, or orbital pain and rarely may be the rst indication of a systemic tumor Empty Sella Syndrome More common than the aforementioned conditions is a nontumorous enlargement of the sella ( empty sella ) This results from a defect in the dural diaphragm, which may occur without obvious cause or with states of raised intracranial pressure, such as pseudotumor cerebri (page 538) or hydrocephalus, or may follow surgical excision of a pituitary adenoma or meningioma of the tuberculum sellae or pituitary apoplexy (see below) The arachnoid covering the diaphragm sellae will bulge downward through the dural defect, and the sella then enlarges gradually, due presumably to the pressure and pulsations of the CSF acting on its walls In the process, the pituitary gland becomes attened, sometimes to an extreme degree; however, the functions of the gland are usually unimpaired Downward herniation of the optic chiasm occurs occasionally and may cause visual disturbances simulating those of a pituitary adenoma (Kaufman et al) As mentioned above, a bitemporal hemianopia with a normal-sized sella is usually due to a primary suprasellar lesion (saccular aneurysm of the distal carotid artery, meningioma, or craniopharyngioma) Treatment This varies with the type and size of the tumor, the status of the endocrine and visual systems, and the age and childbearing plans of the patient The administration of the dopamine agonist bromocriptine (which inhibits prolactin) in a beginning dosage of 05 to 125 mg daily (taken with food) may be the only therapy needed for small or even large prolactinomas and is a useful adjunct in the treatment of the amenorrhea-galactorrhea syndrome The dose should be slowly increased by 25 mg or less every several days until a therapeutic response is obtained Under the in uence of bromocriptine, the tumor decreases in size within days, the prolactin level falls, and the visual eld defect improves Some cases of acromegaly also respond to the administration of bromocriptine but even better to octreotide, an analogue of somatostatin The initial dose of octreotide is 200 mg/day, increased in divided doses to 1600 mg by increments of 200 mg weekly In Lamberts series of acromegaly patients, the growth hormone levels returned to normal and tumor size was reduced in 12 of 15 cases Treatment with bromocriptine and octreotide must be continuous to prevent relapse Newer slow-release somatostatin analogues and long-acting dopamine agonists such as cabergoline have been developed for use in patients who do not respond to the conventional agents (Colao and Lombardi) If the patient is intolerant of bromocriptine (or, in the case of acromegaly, to octreotide and the newer drugs mentioned above), the treatment is surgical, using a transsphenoidal microsurgical approach, with an attempt at total removal of the tumor and preservation of normal pituitary function Unfortunately, approximately 15 percent of GH-secreting tumors and prolactinomas will recur at 1 year For this reason, incomplete removal or recurrence of the tumor (or tumors that are unresponsive to hormonal therapy) should be followed by radiation therapy Another alternative treatment for intrasellar tumors is proton beam radiation or some other form of stereotactic radiosurgery provided that vision is not being threatened and there is no other urgent need for surgery These forms of radiation can be focused precisely on the tumor and will destroy it Kjellberg and colleagues and Chapman, using proton beam radiation, treated over 1100 pituitary adenomas without a fatality and with few complications (Kliman et al) A single brief exposure through intact skin and skull was all that was necessary An endocrine de cit will follow in most instances and must be corrected by hormone replacement therapy.

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Proton beam therapy is available in only very few centers worldwide, but equivalent methods ( gamma knife, linear accelerator) are more accessible The advantage of these radiotherapeutic methods is that tumor recurrence is rare A disadvantage is that the radiation effect is obtained only after several months Estrada and colleagues have also reported that external beam radiation therapy may be employed after unsuccessful transsphenoidal surgery for Cushing disease After approximately 35 years, 83 percent of their patients showed no signs of tumor growth There are a few reports, however, of a decline in memory ability after radiation treatment of all types Large extrasellar extensions of a pituitary growth must be removed by craniotomy, usually with a transfrontal approach, followed by radiation therapy Visual eld defects often remain, but some improvement in vision can be anticipated Pituitary Apoplexy This syndrome, described originally by Brougham and Adams, occurs as a result of infarction of an adenoma that has outgrown its blood supply (see also page 486) It is characterized by the acute onset of headache, ophthalmoplegia, bilateral visual loss, and in severe cases, drowsiness or coma, with either subarachnoid hemorrhage or pleocytosis and elevated protein in the CSF The CT scan or MRI shows infarction of tumor, often with hemorrhage, in and above an enlarged sella Pituitary apoplexy may threaten life unless the acute addisonian state is treated by dexamethasone (6 to 12 mg q 6 h) Blindness is the other dreaded complication If there is no improvement after 24 to 48 h or if vision is markedly affected, transnasal decompression of the sella is indicated Factors that may precipitate the necrosis or hemorrhage of a pituitary tumor are anticoagulation, pituitary function testing, radiation, bromocriptine treatment, and head trauma; most cases of necrosis, however, occur spontaneously Some pituitary adenomas have been cured by this accident Ischemic necrosis of the pituitary, followed by hypopituitarism, occurs under a wide variety of clinical circumstances, the most common being in the partum or postpartum period (Sheehan syndrome) Meningioma of the Sphenoid Ridge This tumor is situated over the lesser wing of the sphenoid bone (Fig 31-7B) As it grows, it may expand medially to involve structures in the wall of the cavernous sinus, anteriorly into the orbit, or laterally into the temporal bone Fully 75 percent of such tumors occur in women and the average age at onset is 50 years Most prominent among the symptoms are a slowly developing unilateral exophthalmos, slight bulging of the bone in the temporal region, and radiologic evidence of thickening or erosion of the lesser wing of the sphenoid bone Variants of the clinical syndrome include anosmia; oculomotor palsies; painful ophthalmoplegia (sphenoidal ssure and Tolosa-Hunt syndromes, see Table 47-2); blindness and optic atrophy in one eye, sometimes with papilledema of the other eye (Foster Kennedy syndrome); mental changes; seizures ( uncinate ts ); and increased intracranial pressure Rarely, a skull bruit can be heard over a highly vascular tumor Sarcomas arising from skull bones, metastatic carcinoma, orbitoethmoidal osteoma, benign giant-cell bone cyst, tumors of the optic nerve, and angiomas of the orbit must be considered in the differential diagnosis Scanning by CT and MRI provides the de nitive diagnosis The tumor is resectable without further injury to the optic nerve if the bone has not been invaded Meningioma of the Olfactory Groove This tumor originates in arachnoidal cells along the cribriform plate The diagnosis depends.

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