how to print barcode in c# INTRACRANIAL NEOPLASMS AND PARANEOPLASTIC DISORDERS in Microsoft Office


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signs Extraneural metastases (cervical lymph nodes, lung, liver, bone) may occur, but usually only after craniotomy, which allows tumor cells to reach scalp lymphatics In rare instances the tumor cells may be spontaneously blood-borne and become metastatic to lung or liver Decerebrate attacks ( cerebellar ts ) appear in the late stages of the disease The radiologic appearance is also distinctive: high signal intensity on both T1- and T2-weighted MRIs, heterogeneous enhancement but of lesser extent than is typical for gliomas, and, of course, the typical location adjacent to and extending into the fourth ventricle The tumor frequently lls the fourth ventricle and in ltrates its oor (Fig 31-11) Seeding of the tumor may occur on the ependymal and meningeal surfaces of the cisterna magna and around the spinal cord The tumor is solid, gray-pink in color, and fairly well demarcated from the adjacent brain tissue It is very cellular, and the cells are small and closely packed with hyperchromatic nuclei, little cytoplasm, many mitoses, and a tendency to form clusters and pseudorosettes The interstitial tissue is sparse
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Treatment Treatment begins with maximal resection of the tumor The addition of chemotherapy and radiotherapy of the entire neuraxis improves the rate and length of disease-free survival even for those children with the most extensive tumors at the time of diagnosis (Packer) The combination of surgery, radiation of the entire neuraxis, and chemotherapy permits a 5-year survival in more than 80 percent of cases The presence of desmoplastic features (ie, connective tissue formations) is associated with a better prognosis Brainstem invasion, spinal subarachnoid metastases, incomplete removal, and very early age of onset (younger than 3 years) reduce the period of survival Neuroblastoma This, the most common solid tumor of childhood, is a slightly different tumor from medulloblastoma but of nearly identical histologic appearance, arising in the adrenal medulla and sometimes metastasizing widely Usually it remains extradural if it invades the cranial and spinal cavities Polymyoclonus with opsoclonus may occur as a paraneoplastic complication, as discussed further on A rare form of neuroblastic medulloblastoma in adults tends to be more benign (Rubinstein) Retinoblastoma Another closely related tumor is the retinoblastoma This proves to be one of the most frequent extracranial malignant tumors of infancy and childhood Eighty percent develop before the fth year of life It is a small-cell tumor with neuro brils and, like the neuroblastoma, has a tendency to form rosettes, which are diagnostic histologic features The tumor develops within the eye and the blindness that it induces may be overlooked in an infant or small child The tumor is easily seen ophthalmoscopically, since it arises from cells of the developing retina An abnormal protein encoded by a growth-suppressor or antioncogenic gene, mentioned earlier in relation to the genetics of brain tumors, has been identi ed It is postulated that an inherited mutation affects one allele of the normal gene, and only if this is accompanied by a mutation that eliminates the function of the second allele will the tumor develop Early recognition and radiation or surgery effect cure Ependymoma and Papilloma of the Fourth Ventricle Ependymomas, as pointed out earlier in this chapter, arise from the lining cells in the walls of the ventricles About 70 percent of them originate in the fourth ventricle, according to Fokes and Earle (Fig 3112) Postmortem, some of these tumors, if small, are found protruding into the fourth ventricle, never having produced local symptoms Whereas the supratentorial ependymoma occurs at all ages, fourth ventricular ependymomas appear mostly in childhood In the large series of Fokes and Earle (83 cases), 33 developed in the rst decade, 6 in the second, and 44 after the age of 20 years Males have been affected almost twice as often as females Ependymomas usually arise from the oor of the fourth ventricle and extend through the foramina of Luschka and Magendie They may later invade the medulla These tumors produce a clinical syndrome much like that of the medulloblastoma except for their more protracted course and lack of early cerebellar signs The histologic features of this tumor have been described earlier in this chapter The degree of anaplastic change varies and has prognostic signi cance The most anaplastic form is the ependymoblastoma, a highly aggressive tumor that falls within the spectrum of primitive neuroectodermal tumors (see below) Symptoms may be present for 1 or 2 years before diagnosis and operation About two-thirds of the patients come to notice because of increased intracranial pressure; in the remainder, vomiting, dif culty in swallowing, paresthesias of the extremities, abdominal pain, vertigo, and neck exion or head tilt are prominent manifestations Some patients with impending cerebellar-tonsillar hernia-
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Figure 31-11 Medulloblastoma MRI in the sagittal (above) and axial (below) planes, illustrating involvement of the cerebellar vermis and neoplastic obliteration of the fourth ventricle
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