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CLINICAL AND PATHOLOGIC CHARACTERISTICS OF BRAIN TUMORS
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It should be stated at the outset that tumors of the brain may exist with hardly any symptoms Often a slight bewilderment, slowness in comprehension, or loss of capacity for sustained mental activity is the only deviation from normal, and signs of focal cerebral disease are wholly lacking In some patients, on the other hand, there is early indication of cerebral disease in the form of a progressive hemiparesis, a seizure occurring in a previously well person, or some other dramatic symptom, but until imaging studies are performed, the evidence may not be clear enough to warrant the diagnosis of a cerebral tumor In a third group, the existence of a brain tumor can be assumed because of the presence of increased intracranial pressure with or without localizing signs of the tumor In yet another group, the symptoms are so de nite as to make it likely that not only is there an intracranial neoplasm but that it is
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INTRACRANIAL NEOPLASMS AND PARANEOPLASTIC DISORDERS
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mechanisms underlying attention and alertness, and the long association ber systems of the cerebral white matter (affecting frontal and temporal areas and the corpus callosum) Probably, these features re ect dysfunction of the frontal lobes, although as often as not, the tumor is in another part of the brain Headaches (See also page 158) These are an early symptom in fewer than one-quarter of patients with brain tumor and are variable in nature In some the pain is slight, dull in character, and episodic; in others it is severe and either dull or sharp but also intermittent If there are any characteristic features of the headache, they would be its nocturnal occurrence or presence on rst awakening and perhaps its deep, nonpulsatile quality However, these are not speci c attributes, since migraine and hypertensive vascular headaches may also begin in the early morning hours or on awakening But if vomiting occurs at the peak of the head pain, tumor is highly likely as noted below Occipito-nuchal headache with vomiting is indicative of a tumor in or near the cerebellum and foramen magnum Tumor patients do not always complain of the pain even when it is present but may betray its existence by placing their hands to their heads and looking distressed When headache appears in the course of the psychomotor asthenia syndrome, it serves to clarify the diagnosis, but not nearly as much as does the occurrence of a seizure The mechanism of the headache is not fully understood In the majority of instances, the CSF pressure is normal during the rst weeks when the headache is present, and one can attribute it only to local swelling of tissues and to distortion of blood vessels in or around the tumor Later the headache appears to be related to increases in intracranial pressure, thus the early morning occurrence after recumbency and vomiting, as discussed in Chap 10 Tumors above the tentorium cause headache on the side of the tumor and in its vicinity, in the orbitofrontal, temporal, or parietal region; tumors in the posterior fossa usually cause ipsilateral retroauricular or occipital headache With elevated intracranial pressure, bifrontal or bioccipital headache is the rule regardless of the location of the tumor Vomiting and Dizziness Vomiting appears in a relatively small number of patients with a tumor syndrome and usually accompanies the headache when the latter is severe It is more frequent with tumors of the posterior fossa The most persistent vomiting (lasting several weeks) that we have observed has been in patients with low brainstem gliomas, fourth ventricular ependymomas, and subtentorial meningiomas Some patients may vomit unexpectedly and forcibly, without preceding nausea (projectile vomiting), but others suffer both nausea and severe discomfort Usually the vomiting is not related to the ingestion of food; often it occurs before breakfast No less frequent is the complaint of dizziness As a rule it is not described with accuracy and consists of an unnatural sensation in the head, coupled with feelings of strangeness and insecurity when the position of the head is altered Frank positional vertigo may be a symptom of a tumor in the posterior fossa (see Chap 15) Seizures The occurrence of focal or generalized seizures is the other major manifestation besides slowing of mental functions and signs of focal brain damage Convulsions have been observed, in various series, in 20 to 50 percent of all patients with cerebral tumors A rst seizure during adulthood is always suggestive of brain tumor and, in the authors experience, has been the most
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common initial manifestation The localizing signi cance of seizure patterns has been discussed on pages 275 to 278 Seizures due to brain tumor most often have a focal onset, ie, are partial and then become generalized There may be one seizure or many, and they may follow the other symptoms or precede them by weeks or months or exceptionally, in patients with low-grade astrocytoma, oligodendroglioma, or meningioma by several years Status epilepticus as an early event is rare but has occurred in a few of our patients As a rule the seizures respond to standard anticonvulsant medications and may improve after surgery for tumor removal Regional or Localizing Symptoms and Signs Sooner or later, in patients with psychomotor asthenia, headaches, and seizures, focal cerebral signs will be discovered; some patients may present with such signs Nearly always, however, the focal signs are at rst slight and subtle Frequently, CT scanning or MRI will have disclosed the presence of a tumor before either focal cerebral signs or the signs of increased intracranial pressure have become evident The cerebral tumors that are most likely to produce the syndromes described above are glioblastoma multiforme, astrocytoma, oligodendroglioma, ependymoma, metastatic carcinoma, meningioma, and primary lymphoma of the brain The clinical aspects of these diseases, which happen to be the most common brain tumors in adults, are discussed in the sections below Glioblastoma Multiforme and Anaplastic Astrocytoma (HighGrade Gliomas) These tumors, which constitute the high-grade gliomas, account for about 20 percent of all intracranial tumors, or about 55 percent of all tumors of the glioma group, and for more than 80 percent of gliomas of the cerebral hemispheres in adults Although predominantly cerebral in location, they may also arise in the brainstem, cerebellum, or spinal cord The peak incidence is in middle adult life (mean age for the occurrence of glioblastoma is 56 to 60 years and 46 years for anaplastic astrocytoma), but no age group is exempt The incidence is higher in men (ratio of approximately 16:1) Almost all of the high-grade gliomas occur sporadically, without a familial predilection The glioblastoma, known since the time of Virchow, was de nitively recognized as a glioma by Bailey and Cushing and given a place in their histogenetic classi cation Most arise in the deep white matter and quickly in ltrate the brain extensively, sometimes attaining enormous size before attracting medical attention The tumor may extend to the meningeal surface or the ventricular wall, which probably accounts for the increase in CSF protein (more than 100 mg/dL in many cases) as well as for an occasional pleocytosis of 10 to 100 cells or more, mostly lymphocytes The CSF may be normal, however Malignant cells, carried in the CSF, may form distant foci on spinal roots or cause a widespread meningeal gliomatosis Extraneural metastases, involving bone and lymph nodes, are very rare; usually they occur only after a craniotomy has been performed About 50 percent of glioblastomas occupy more than one lobe of a hemisphere or are bilateral; between 3 and 6 percent show multicentric foci of growth and thereby simulate metastatic cancer The tumor has a variegated appearance, being a mottled gray, red, orange, or brown, depending on the degree of necrosis and presence of hemorrhage, recent or old The imaging appearance is usually that of a nonhomogeneous mass, often with a center that is hypointense in comparison to adjacent brain and demonstrating an irregular thick or thin ring of enhancement, surrounded by edema
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