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Robert H. Wilkins and Peter C. Burger

✓ Three patients are presented who had benign intraparenchymal cysts of the brain. The wall of each cyst was biopsied and had no epithelial lining or other microscopic feature to indicate the etiology of the cyst. The authors review published information about benign brain cysts and speculate about the origin of those having a wall composed solely of neuroglial tissue.

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Robert H. Wilkins, Rodney A. Radtke, and Peter C. Burger

✓ The authors report a 36-year-old woman with a 23-year history of simple and complex partial seizures who was treated surgically for an anteroinferior temporal encephalocele, with resolution of the seizure disorder. This patient's presentation, findings, and response to treatment are typical of those associated with anteroinferior temporal encephalocele, and different from the clinical patterns of four other types of spontaneous temporal encephalocele.

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Peter C. Burger, E. Ralph Heinz, Taichiro Shibata, and Paul Kleihues

✓ To provide baseline information for the “local” therapy of the glioblastoma multiforme (GBM), whole-brain histological sections of 15 untreated GBM's were studied to determine the distribution of neoplastic cells. These findings were then compared with the computerized tomography (CT) scans in 11 cases in order to determine the extent to which the peripheral portion of the neoplasm can be estimated by the presence of a low-density area without contrast enhancement. The results of the histological study confirmed the marked heterogeneity of GBM's and disclosed a great variability in the geometry, extent, and character of the peripheral “infiltrating” margin. In spite of the widely held concept that glioblastomas are localized within 2 cm of the contrast-enhanced rim, there were three cases in this two-dimensional study in which this distance was exceeded, and it seems likely that three-dimensional reconstructions would have detected additional cases in which neoplastic cells extended beyond this arbitrary limit. Only three of the 15 GBM's were restricted to the distribution of one internal carotid or one vertebral artery. To the extent that the neoplasms in the present series are representative, this suggests that glioblastomas will be difficult to treat successfully by intra-arterial therapy using existing therapeutic agents. Correlations of histological sections with the CT scans revealed that the vast majority of the neoplastic tissue was contained within the contrast-enhancing and “peritumoral” areas of low density, but that in five cases fingers of neoplasm extended for short distances beyond the outer margin of the latter region. This indicates that the distribution of cells of a GBM cannot be inferred from CT images since the “peritumoral” area of low density can over- or underestimate the extent of the lesion.

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Orest B. Boyko, Peter C. Burger, and E. Ralph Heinz

✓ Although hypertension can be associated with phencyclidine (PCP) use, subarachnoid hemorrhage (SAH) is a rare result. The radiological and pathological findings are reported of a patient with acute SAH who had chromatographic evidence of PCP in his blood. The occurrence of SAH in a patient who uses PCP may be caused by a disrupted arterial vessel wall and/or vasospasm due to the pharmacological action of the drug on the cerebral vasculature.

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Douglas C. Anthony, Susan K. Atwater, Marvin P. Rozear, and Peter C. Burger

✓ Following a 15-foot fall from a roof, a 70-year-old man became comatose and developed signs of pontine dysfunction. There was a severely comminuted fracture of the distal left femur suggesting that he had landed in an upright position. It was clinically unclear whether the fall was secondary to a pontine infarct; however, an autopsy revealed a fracture of the clivus which had entrapped and occluded the basilar artery, causing death. These findings, and those in similar cases, suggest that this entity results from a force transmitted in an axial direction.

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J. Hartley Bowen, Peter C. Burger, Guy L. Odom, Philip J. Dubois, and James M. Blue

✓ Two adults presented with frontal lobe masses. As visualized by computerized tomography, both lesions were large cysts with contrast-enhancing mural nodules and enhancing circumferential rims. En bloc resections of the mural nodules and cyst walls were performed. Pathological evaluation of each nodule disclosed a meningioma, and neoplastic cells were found in the distant cyst walls. Although the walls of large cysts associated with some meningiomas have been composed of reactive glia or collagen, the neoplastic character of the cysts in the present cases underscores the need for resection and careful pathological evaluation of the large cysts associated with meningiomas.

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Albert Moghrabi, Henry S. Friedman, Peter C. Burger, Robert Tien, and W. Jerry Oakes

✓ Six patients with optic pathway gliomas who were previously managed with surgery and/or chemotherapy were treated with carboplatin (560 mg/sq m) after radiographic evidence of disease progression. The median age at diagnosis was 2 years (range 4 months to 7 years), and the interval between diagnosis and carboplatin therapy ranged between 7 months and 6.5 years (median 1.8 years). Treatment was given at 4-week intervals and continued until unacceptable toxicity supervened, the disease progressed, or the disease was stable for 12 months. All patients demonstrated disease stability at the outset of the third cycle and continued to do so at the time of this writing. Two patients are 16 and 32 months from initial carboplatin therapy and have been off treatment for 5 and 14 months, respectively; two patients are still receiving therapy at 7 and 11 months after their initial treatment. During the study, two patients developed hypersensitivity to the drug, requiring its discontinuation. Toxicity was minimal, consisting mainly of thrombocytopenia, requiring a one-dose reduction in four of the six treated patients. No platelet transfusions were needed. These results suggest that carboplatin can arrest growth of progressive optic pathway gliomas in children and can allow delay of radiotherapy. A larger trial will be required to define the optimal use of carboplatin in the treatment of low-grade gliomas in children.

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Darryl C. Longee, Henry S. Friedman, Robert E. Albright Jr., Peter C. Burger, W. Jerry Oakes, Joseph O. Moore, and S. Clifford Schold Jr.

✓ Seventeen patients with recurrent gliomas were treated with the combination of cyclophosphamide and vincristine. All but one had previously received and failed chemotherapy. Cyclophosphamide was administered at doses ranging from 250 to 1000 mg/sq m by intravenous infusion on Days 1 and 2, and vincristine was given at a dose of 1.0 mg/sq m (2 mg maximal dose) intravenously on Day 1; cycles were repeated every 4 weeks. Clinical and radiographic improvement was observed in eight of 16 evaluable patients, and four other patients had stabilization of previously progressive disease. Four patients are alive and off treatment without evidence of recurrence for a median period of 37 months; these included an adult with a cerebral anaplastic astrocytoma now more than 51 months after therapy. Toxicity included moderately severe myelosuppression that required hospitalization in seven patients. These results indicate that the combination of cyclophosphamide and vincristine has activity in the treatment of recurrent gliomas, and warrant the use of these drugs in larger controlled studies, particularly if they can be used in conjunction with hematopoietic growth factors.

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Peter C. Burger, Philip J. Dubois, S. Clifford Schold Jr., Kenneth R. Smith Jr., Guy L. Odom, David C. Crafts, and Felice Giangaspero

✓ Pathological findings in 20 cases of glioblastoma multiforme were correlated with clinical histories and computerized tomographic (CT) scans. This was done to define the neoplasm in three stages: before treatment, during remission, and during recurrence.

The untreated lesions were markedly cellular neoplasms composed predominantly of small anaplastic cells. The radiographic central region of low density was necrosis, the enhancing rim was a cellular zone of viable neoplasm, and the perilesional low-density area was edema with infiltrating tumor. In these 20 cases, all of the identifiable neoplasms lay within the zone of peritumoral edema or contrast enhancement, although small anaplastic cells may have been present in more distant regions. The lesions in remission were remarkable for their minimal mass effect, discrete nature, extensive necrosis, and content of large bizarre glia. The large cells were confined to the original tumor bed and were consistent with neoplastic cells inactivated and immobilized by radio- and chemotherapy. These lesions were accurately localized by CT scanning. The recurrent lesions were heterogeneous, but most were formed of widely disseminated small anaplastic cells. The highly cellular regions of such lesions could be localized by CT scanning, but CT could not detect less cellular foci in the cerebrum, cerebellum, or brain stem. In one patient, the contrast-enhancing lesions of “recurrence,” were foci of radionecrosis, underscoring the difficulty in distinguishing this entity from recurrent neoplasm.

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Thomas B. Crotty, Bernd W. Scheithauer, William F. Young Jr., Dudley H. Davis, Edward G. Shaw, Gary M. Miller, and Peter C. Burger

✓ Two distinct clinicopathological variants of craniopharyngioma exist: the classic adamantinomatous type and a recently described papillary form that predominates in adults and reportedly behaves in a less aggressive manner. The present study describes the clinicopathological features of 48 patients with papillary craniopharyngioma treated at the Mayo Clinic between 1910 and 1994. An additional four tumors were found to have histological features of both adamantinomatous and papillary craniopharyngioma. Whereas adamantinomatous tumors typically occur in adolescent patients, the mean age of the 48 patients (23 males and 25 females) with papillary craniopharyngioma was 44.7 years (range 10 to 74 years). Presenting clinical features included visual impairment (84%), headache (68%), and pituitary insufficiency (anterior 42%; posterior 27%). Preoperative computerized tomography (CT) and magnetic resonance (MR) imaging in 17 patients typically revealed a noncalcified, partially cystic mass that enhanced peripherally and contained mural nodules (67%). Many (41%) of the lesions involved or extended into the third ventricle on imaging. At first surgery, gross total tumor removal was achieved in 17 patients (36%) and subtotal resection in 30 patients (64%) in whom tumor resection was attempted. Tumor recurrence was noted in two patients who underwent gross total removal. Tumor-free survival rates of 100% and 78% were obtained in patients who underwent gross total and subtotal resection at initial surgery, respectively. Postoperative radiation therapy was beneficial to patients having undergone a subtotal resection, with an increase in tumor-free survival from 26% to 86%.

Aside from well-documented morphological distinctions, papillary craniopharyngiomas differ from adamantinomatous tumors in several important respects. These include the almost exclusive occurrence of papillary tumors in adulthood and their more uniform appearance on both CT and MR imaging. However, a preliminary analysis of our data suggests there are no significant differences between the two lesions with respect to resectability, efficacy of radiation therapy, and overall survival.