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MAJOR CATEGORIES OF NEUROLOGIC DISEASE
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pineocytomatous rosettes Pineocytes may be impregnated by silver carbonate methods, and some contain the retinal S antigen of photoreceptor cells Pineoblastomas are highly cellular and composed of small, undifferentiated cells bearing some resemblance to medulloblasts The teratoma and dermoid and epidermoid cysts of the pineal have no special features some are quite benign The gliomas have the usual morphologic characteristics of an astrocytoma of varying degrees of malignancy In some cases, the clinical syndrome of the several types of pineal tumors consists solely of symptoms and signs of increased intracranial pressure Beyond this, the most characteristic localizing signs are an inability to look upward and slightly dilated pupils that react on accommodation but not to light (Parinaud syndrome) Sometimes ataxia of the limbs, choreic movements, or spastic weakness appears in the later stages of the illness It is uncertain whether the ocular and motor signs are due to neoplastic compression of the brachia conjunctivae and other tegmental structures of the upper midbrain or to hydrocephalus (dilation of the posterior part of the third ventricle) Probably both mechanisms are operative Precocious puberty occurs in males who harbor a germinoma Although the pineal gland is the source of melatonin, sleep is not affected to an important degree in patients with these tumors, as discussed on page 482 Measurement of CSF or serum melatonin is useful mainly in the detection of tumor recurrence after surgical extirpation The diagnosis is made by CT scanning and MRI (Fig 31-14) The CSF may contain tumor cells and lymphocytes but may also be entirely normal Treatment These lesions were formerly judged to be inoperable However, the use of the operating microscope now makes it possible to excise them by a supracerebellar or transtentorial approach Operation for purposes of excision and histologic diagnosis is advised, because each type of pineal tumor must be managed differently Moreover, one may occasionally nd an arachnoidal cyst that needs only excision The germinomas should be removed insofar as possible and the whole neuraxis irradiated The use of chemotherapy in addition to or instead of cranial irradiation is still being evaluated (Allen) Several of our patients have survived more than 5 years after the removal of a pineal glioma Other Germinomas, Gangliocytomas, and Mixed NeuronalGlial Tumors Malignant germ-cell tumors occurring in locations other than the pineal body are usually found in the suprasellar space and rarely in the roof of the third ventricle Germinoma is the most common of this rare group of neoplasms, which also includes choriocarcinoma, embryonal cell carcinoma, endodermal sinus tumors, and malignant teratomas Certain biochemical markers of these tumors are of interest and of clinical utility, since they may be detected in samples of the blood and CSF The beta subunit of human chorionic gonadotropin (hCG) is elaborated by choriocarcinoma, and alpha fetoprotein by choriocarcinoma and immature teratomas Typical germinomas have shown little elevation of either Most often these markers indicate the presence of complex mixed germcell tumors Gangliogliomas and mixed neuronal-glial tumors are special tumor types, more frequent in the young and of variable but usually low-grade malignancy They are composed both of differentiated glial cells, usually astrocytes, and of neurons in various degrees of differentiation The latter, which may resemble glial cells, can be identi ed by Nissl stains, silver stains, and immunochemical reactions for cytoskeletal proteins Some of these developmental tumors are dif cult to separate from hamartomas or the ventricular tubers of tuberous sclerosis
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Figure 31-14 Pinealoma MRI in the sagittal plane (above) demonstrates the tumor (large arrow), which compresses the superior cerebellum (smaller arrow) and the aqueduct (long-stemmed arrow) An axial cut (below) shows the tumor (straight arrow) and evidence of hydrocephalus (curved arrow), the result of aqueductal compression (Reproduced by permission from Bisese JH: Cranial MRI New York, McGraw-Hill, 1991)
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The best-known type in this group is called the gangliocytoma, one form of which is the dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) This is a slowly evolving lesion that forms a mass in the cerebellum; it is composed of granule, Purkinje, and glial cells Reproduced therein, in a disorganized fashion, is the architecture of the cerebellum The importance of distinguishing this disease from other cerebellar tumors is its lack of growth potential and favorable prognosis It should be excised if symptomatic Other forms of gangliogliomas include the desmophilic infantile ganglioglioma, some of the xanthoastrocytomas, and the dysembrioplastic neuroepitheliomas Often they lie near the ventricles, and they may induce hydrocephalus Many of these tumors are rare and affect children mostly; therefore they are not discussed further here Good descriptions are to be found in the monographs of Rus-
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