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con rmatory of an endocrine disorder, as described above, and sometimes a ballooned sella turcica in plain lms of the skull are occasionally found Patients who are suspected of harboring a pituitary adenoma but in whom the plain lms are normal should be examined by MRI This procedure will visualize pituitary adenomas as small as 3 mm in diameter and show the relationship of the tumor to the optic chiasm This also provides the means of following the tumor s response to therapy (Fig 31-18) It should be kept in mind that pituitary tissue normally enhances on CT and MRI, and small tumors are therefore revealed as nonenhancing nodules Tumors other than pituitary adenomas may sometimes expand the sella Enlargement may be due to an intrasellar craniopharyngioma, carotid aneurysm, or cyst of the pituitary gland Intrasellar epithelium-lined cysts are rare lesions They originate from the apical extremity of Rathke s pouch, which may persist as a cleft between the anterior and posterior lobes of the hypophysis Rarer still are intrasellar cysts that have no epithelial lining and contain thick, dark brown uid, the product of intermittent hemorrhages Both types of intrasellar cyst may compress the pituitary gland and mimic the endocrine-suppressive effects of pituitary adenomas Neoplasms originating in the nasopharynx or sinuses may invade the sella and pituitary gland, and sarcoid lesions at the base of the brain may do the same Also, the pituitary gland (and the chiasm) may be the site of metastases, most of them from the lung and breast (Morita et al); they give rise to diabetes insipidus, pituitary

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Figure 31-18 Pituitary tumors Upper gure: T1-weighted, enhanced coronal MRI A microadenoma is seen as a nonenhancing nodule (arrow) within the normally enhancing pituitary gland The optic chiasm is seen just above the gland Lower gure: Nonenhanced MRI Macroadenoma (arrow) compressing the optic chiasm

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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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