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MAJOR CATEGORIES OF NEUROLOGIC DISEASE
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Figure 31-12 Ependymoma of the fourth ventricle A Ependymoma growing out of the fourth ventricle B Contrast-enhanced CT scan in a 4-year-old girl who presented with signs of increased intracranial pressure Note hydrocephalus and end of shunt tube in right lateral ventricle
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tion are disinclined to sit and have vertical downbeating nystagmus Surgical removal offers the only hope of survival The addition of radiation therapy and sometimes ventriculoperitoneal shunting of CSF may prolong life Myxopapillary ependymomas of the spinal cord and lum are discussed with the spinal cord tumors (page 1080) Papillomas of the choroid plexus are about one- fth as frequent as ependymomas They arise mainly in the lateral and fourth ventricles, occasionally in the third Two authoritative studies (Laurence et al; Matson and Crofton) give the ratios of lateral/third/ fourth ventricular locations as 50:10:40 The tumor, which takes the form of a giant choroid plexus, has as its cellular element the cuboidal epithelium of the plexus, which is closely related embryologically to the ependyma An oncogene T (tumor) antigen of the SV40 virus is possibly involved in tumor induction (see Schmidek) Essentially these are tumors of childhood Fully 50 percent cause symptoms in the rst year of life and 75 percent in the rst decade In the younger patients, hydrocephalus is usually the presenting syndrome, sometimes aggravated acutely by hemorrhage; there may be papilledema, an unusual nding in a hydrocephalic infant with enlarging head Headaches, lethargy, stupor, spastic weakness of the legs, unsteadiness of gait, and diplopia are more frequent in the older child Sometimes patients present with a syndrome of the cerebellopontine angle (see further on), where the tumor arises from choroid plexus and projects into the lateral recess of the fourth ventricle One consequence of the tumor is thought to be increased CSF formation, which contributes to the hydrocephalus Some of the tumors acquire more malignant attributes (mitoses, atypia of nuclei) and invade surrounding brain They have the appearance of a carcinoma and may be mistaken for an epithelial metastasis from an extracranial site Treatment by surgical excision is usually curative, but palliative ventricular shunting may be needed rst if the patient s con-
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dition does not permit surgery The prognosis of the rare choroid plexus carcinomas is poor Primitive Neuroectodermal Tumors (PNETs) This term was introduced by Hart and Earle in 1973 to describe tumors that have the histologic features of medulloblastoma but occur supratentorially Various poorly differentiated or embryonal tumors of infancy and childhood were included in this group: medulloblastoma, neuroblastoma, retinoblastoma, ependymoblastoma, and pineoblastoma (described further on) Subsequent authors have broadened the category of PNETs to include all CNS neoplasms of neuroectodermal origin With the advent of immunohistochemical techniques, many of these poorly differentiated neoplasms of infancy came to be recognized as small-cell gliomas (Friede et al); others, after ultrastructural study, could be classi ed as other types of primitive neoplasms To some pathologists, the term primitive neuroectodermal tumors has a certain appeal but has added little to our understanding of their undifferentiated embryonal origin In practical terms, the prognosis and treatment of all these tumors is much the same, regardless of what they are called (see Duffner and colleagues) As described earlier, certain patterns of gene expression are now used to distinguish this group of tumors from histologically similar medulloblastomas Hemangioblastoma of the Cerebellum This tumor is referred to most often in connection with von Hippel-Lindau disease (page 873) Dizziness, ataxia of gait or of the limbs on one side, symptoms and signs of increased intracranial pressure from compression of the fourth ventricle, and in some instances an associated retinal angioma or hepatic and pancreatic cysts (disclosed by CT or MRI) constitute the syndrome There is a tendency later for the development of malignant renal or adrenal tumors Many patients have polycythemia due to elaboration of an erythropoietic factor by the tumor
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