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INTRACRANIAL NEOPLASMS AND PARANEOPLASTIC DISORDERS
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5 months on average (see below) The addition of the chemotherapeutic agent carmustine (BCNU) alone increases survival slightly Although there are no satifsactory randomized trials, it is considered by most neuro-oncologists that somewhat longer survival can be achieved when a combination of drugs procarbazine, lomustine (CCNU), and vincristine (a combination termed PCV) is given (Levin et al) Cisplatin and carboplatin provide similar marginal improvement in survival beyond that obtained by debulking and radiation therapy In keeping with general experience in this eld, the report of the Glioma Meta-analysis Trialists (GMT) Group concluded there was a clear but small bene t of chemotherapy Brachytherapy (implantation of iodine-125 or iridium-193 beads or needles) and high-dose focused radiation (stereotactic radiosurgery) have so far not signi cantly altered survival times The treatment of recurrent tumor after surgery and radiation, an almost inevitable occurrence, is controversial and must be guided by the location and pattern of tumor growth, the patient s age, and relative state of health Almost all glioblastomas recur within 2 cm of their original site and 10 percent develop additional lesions at distant locations Reoperation is sometimes undertaken for local recurrences, as is brachytherapy, both with uncertain results The most aggressive approach, a second surgery and chemotherapy, has been generally utilized in patients under age 40 whose original operation was many months earlier If the PCV regimen discussed above has not already been used, that combination or the newer and better-tolerated alkylating agent temozolomide (which may be used if the PCV regimen was administered previously) are sometimes used in cases of recurrent glioblastoma and anaplastic astrocytoma In general, these chemotherapeutic drugs prolong the symptom-free interval but have little effect on survival With aggressive surgical removal and radiotherapy, as described above, median survival for patients with glioblastoma is 12 months, compared to 7 to 9 months without such treatment The median survival in cases of anaplastic astrocytoma is considerably longer, 2 to 4 years Viewed from another perspective, in a recent large series, the 18-month postoperative survival was 15 percent in patients with glioblastoma and 62 percent in those with anaplastic astrocytoma Astrocytoma (Well-Differentiated Astrocytoma) The welldifferentiated astrocytomas (grades I and II in former classi cations), which constitute between 25 and 30 percent of cerebral gliomas, may occur anywhere in the brain or spinal cord Favored sites are the cerebrum, cerebellum, hypothalamus, optic nerve and chiasm, and pons In general, the location of the tumor appears to be in uenced by the age of the patient Astrocytomas of the cerebral hemispheres arise mainly in adults in their third and fourth decades or earlier; astrocytomas in other parts of the nervous system, particularly the posterior fossa and optic nerves, are more frequent in children and adolescents These tumors are classi ed further according to their histologic characteristics: protoplasmic or brillary; gemistocytic (enlarged cells distended with hyaline and eosinophilic material); pilocytic (elongated, bipolar cells); and mixed astrocytoma-oligodendroglioma types The most common type is composed of well-differentiated brillary astrocytes The tumor cells contain glial brillary acidic protein (GFAP), which is a useful diagnostic marker in biopsy specimens Some cerebral astrocytomas, as already noted, present as mixed astrocytomas and glioblastomas The most common low-grade brillary type corresponds to a WHO grade II and is distinguished from the more benign WHO grade I pilocytic tumor (see page 578) The rarer pleomorphic
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xanthoastrocytoma was also classed as WHO II These distinctions correlate to a large degree with the biologic behavior of the astrocytomas and therefore have prognostic importance Cerebral astrocytoma is a slowly growing tumor of in ltrative character with a tendency in some cases to form large cavities or pseudocysts Other tumors of this category are noncavitating and appear grayish white, rm, and relatively avascular, almost indistinguishable from normal white matter, with which they merge imperceptibly Fine granules of calcium may be deposited in parts of the tumor, but this nding in a slow-growing intracerebral tumor is more characteristic of an oligodendroglioma The CSF is acellular; the only abnormalities in some cases are the increased pressure and protein content The tumor may distort the lateral and third ventricles and displace the anterior and middle cerebral arteries (seen in CT scans, MR arteriograms, and conventional angiograms; see Fig 31-3) In about half of patients with astrocytoma, the opening symptom is a focal or generalized seizure, and between 60 and 75 percent of patients have recurrent seizures in the course of their illness Other subtle cerebral symptoms follow after months, sometimes after years Headaches and signs of increased intracranial pressure are relatively late occurrences MRI may be helpful in distinguishing the more frequent brillary from pilocytic astrocytomas Pilocytic types are sharply demarcated, with smooth borders and little edema On T1-weighted MRI, they are isointense or hypointense; on T2 sequences, hyperintense, and there tends to be marked enhancement after gadolinium infusion Cyst formation and small amounts of calcium are common, especially in cerebellar tumors The brillary tumors have a less stereotyped appearance, generally taking the form of a hypodense mass with less well de ned borders and little or no contrast enhancement (Fig 31-3)
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Figure 31-3 Astrocytoma of the left frontal lobe; the T2-weighted MRI shows an in ltrating tumor with minimal mass effect and slight edema The degree of enhancement varies
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