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19.qxd 3/10/08 9:53 AM Page 599<br />

In turn, upward extension of a macroadenoma may impinge<br />

on the optic chiasm, with a bitemporal hemianopia; on the<br />

hypothalamus, with diabetes insipidus; or, in extreme cases,<br />

on the frontal lobe, with cognitive changes and seizures.<br />

Endocrinologic changes seen with secretory tumors most<br />

commonly involve hyperprolactinemia, with amenorrhea in<br />

females and gynecomastia and erectile dysfunction in males.<br />

Excess growth hormone and adrenocorticotropic hormone<br />

(ACTH) secretion are the next most common changes and<br />

may cause acromegaly or Cushing’s disease respectively.<br />

Gonadotropin and thyroid-stimulating hormone (TSH)<br />

excess are rare. Importantly, in about 10 percent of secreting<br />

adenomas, two or more hormones may be excessively produced.<br />

In addition, in some cases of macroadenomas there<br />

may be ‘kinking’ of the pituitary stalk, and in such cases<br />

dopamine delivery to the posterior pituitary may be<br />

impaired with a resultant secondary hyperprolactinemia.<br />

Magnetic resonance scanning is typically positive with<br />

macroadenomas; however, microadenomas of less than<br />

3 mm in diameter may escape detection. In rare instances,<br />

pituitary adenomas may undergo hemorrhage or infarction,<br />

producing the syndrome of ‘pituitary apoplexy’ with severe<br />

headache, diplopia, blindness, and, critically, an acute case of<br />

adrenocortical insufficiency, which may be fatal.<br />

Craniopharyngiomas are lobulated, calcified tumors<br />

that, although most commonly occurring in children, may<br />

occur in patients of any age. They typically arise from the<br />

junction of the infundibulum and pituitary gland, and may<br />

produce a variety of symptoms, depending on which direction<br />

they grow in. With upward extension and impingement<br />

of the hypothalamus, there may be diabetes insipidus<br />

and obesity, and with compression of the third ventricle,<br />

hydrocephalus may occur. Compression of the optic chiasm<br />

may cause a bitemporal hemianopia, and with downward<br />

extension various forms of pituitary failure may appear.<br />

Pineal tumors include not only pinealomas but also<br />

germ cell tumors, gliomas, and teratomas. These tumors<br />

cause symptoms primarily by compression of the adjacent<br />

quadrigeminal plate and the underlying aqueduct of Sylvius,<br />

with a Parinaud syndrome (limitation of upgaze and an<br />

Argyll Robertson pupil) and obstructive hydrocephalus<br />

respectively.<br />

Hemangioblastomas (Boughey et al. 1990) are cystic<br />

masses found in the cerebellum, which typically contain an<br />

enhancing nodule on MR scanning. They are found in children<br />

or young adults, usually present with cerebellar symptoms,<br />

and may occur as part of von Hippel–Lindau disease.<br />

Colloid cysts of the third ventricle are rare cystic masses that<br />

may cause dementia (Kelly 1951), either by compression of<br />

the surrounding thalamus (Faris and Terrence 1989; Lobosky<br />

et al. 1984) or as a result of obstructive hydrocephalus, as illustrated<br />

in Figure 19.2. Interestingly, by virtue of a ‘ball-valve’<br />

effect, in which the foramen of Monro is intermittently<br />

occluded, these tumors may also cause intermittent elevations<br />

of intraventricular pressure. Thus, patients may experience<br />

intermittent headache, unsteadiness, and confusion, and<br />

report that these symptoms are posture-dependent.<br />

METASTATIC TUMORS<br />

19.1 Brain tumors 599<br />

Figure 19.2 This unenhanced computed tomography scan<br />

demonstrates a colloid cyst, which, by obstructing the foramen of<br />

Monro, has caused obstructive hydrocephalus with dilation of the<br />

lateral ventricles. (Reproduced from Gillespie and Jackson 2000.)<br />

Metastases may arise from various different primary<br />

tumors and almost always reach the brain via hematogenous<br />

spread. By far the most common source is lung cancer,<br />

primarily the non-small-cell type, followed by breast<br />

cancer and melanoma and then by various other tumors,<br />

including those of the colon and rectum, kidney, gallbladder,<br />

liver, thyroid, testicle, prostate, uterus or ovary, and<br />

pancreas; both systemic lymphoma (primarily of the non-<br />

Hodgkin’s type) and leukemia may also metastasize to the<br />

brain. Of all these primaries, melanoma and testicular cancer,<br />

although not common, exhibit the greatest propensity<br />

for metastasis to the brain.<br />

Two-thirds of metastases eventually settle in the brain<br />

parenchyma, whereas the remainder lodge either in the<br />

leptomeninges (where they create a condition known as<br />

leptomeningeal carcinomatosis) or in the dura. Parenchymal<br />

metastases may be found throughout the cerebrum (Delattre<br />

et al. 1988a); they are found in the hemispheres in about<br />

three-quarters of cases, in the cerebellum in about oneeighth,<br />

and in the brainstem or thalamus in the remainder;<br />

of those lodging in the hemispheres, all lobes may be<br />

involved, including, in descending order, the frontal, parietal,<br />

occipital, and temporal lobes.<br />

Metastases to the parenchyma generally appear as<br />

homogenously enhancing masses, often with considerable<br />

peri-tumoral edema; in high-grade malignancies, however,<br />

central necrosis may lead to a ring-enhancing pattern.<br />

Although solitary lesions may occur, multiple tumors, as<br />

noted earlier, are more common, and indeed at autopsy<br />

one may find widespread, microscopic foci.

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