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Primary cerebral neuroblastoma

Long-term follow-up review and therapeutic guidelines

Mitchel S. Berger, Michael S. B. Edwards, William M. Wara, Victor A. Levin and Charles B. Wilson

✓ Primary cerebral neuroblastoma is a distinct pathological and clinical entity that differs from other primitive neuroectodermal tumors. To characterize the clinical course of this lesion, the authors performed a retrospective analysis in 11 patients who ranged in age from 17 months to 26 years. The tumor had no predilection for either sex. Signs and symptoms were mostly those associated with increased intracranial pressure. The lesions commonly involved the parietal and occipital lobes. Computerized tomography scans of nine patients showed five solid and four cystic lesions; calcifications were found more commonly in the solid lesions. Contrast enhancement was seen in all tumors, yet angiograms typically showed an avascular mass. Total removal of tumor was possible in only two patients, both with cystic tumors. The remaining nine underwent subtotal resection of a solid lesion (in five) or a cystic lesion (in four). All 11 patients underwent postoperative irradiation that included the spinal axis in two cases; only one received adjuvant chemotherapy (solid tumor). Four patients, all with solid tumors that initially were subtotally resected, had evidence of tumor recurrence. The only patient with a subtotally resected solid lesion who did not have recurrence received adjuvant chemotherapy. The six patients who had cystic lesions are free of recurrent tumor at 26 to 109 months after surgery. Based on follow-up analysis of the 11 patients, recommendations are proposed for the treatment of primary cerebral neuroblastomas.

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Mitchel S. Berger and Yoshio Hosobuchi

✓ A persistent carotid-basilar anastomosis (primitive trigeminal artery), identified by four-vessel vertebral angiography, was shown to be the cause of a cavernous sinus fistula in a 51-year-old woman. The fistula, but not the primitive artery, was identified on a carotid arteriogram. Because of the flow contribution from the posterior circulation, balloon embolization via the carotid system failed, and the fistula was repaired through a direct surgical approach. The operative technique is described and the hemodynamic aspects of a cavernous sinus fistula that is related to this primitive anastomosis are reviewed.

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Intracranial dissecting aneurysms of the posterior circulation

Report of six cases and review of the literature

Mitchel S. Berger and Charles B. Wilson

✓ Dissecting aneurysms of the intracranial posterior circulation are unusual lesions that affect otherwise healthy young adults, are difficult to diagnose and manage, and carry a high morbidity and mortality rate. Headache in the suboccipital-posterior cervical region is the most common presenting symptom. The dissection usually occurs between the intima or internal elastic lamina and the media; subadventitial dissection does occur and accounts for the infrequent finding of subarachnoid hemorrhage. A deficit in the inner layers of the vessel is the proposed source of dissection. The angiographic features are inconsistent, although an irregularly narrowed arterial segment with proximal and/or distal dilatation are typical findings. Depending on the location of the dissection, the surgical options are: ligation, trapping, or reinforcement of exposed abnormal portions of the vessel. Anticoagulation therapy is not indicated in the management of this lesion.

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Mitchel S. Berger and Charles B. Wilson

✓ Epidermoid cysts originating in the paramedian basal cisterns of the posterior fossa are congenital lesions that grow to a large size through slow accumulation of desquamated epithelium. These lesions grow between and ultimately displace cranial nerves, vascular structures, and the brain stem, causing a long course of progressive neurological deficits. The onset of symptoms usually occurs during the fourth decade of life. Epidermoid cysts are easily diagnosed with computerized tomography scans, which characteristically show a low-density extra-axial pattern. The primary surgical objective is to decompress the mass by evacuating the cyst contents and removing nonadherent portions of the tumor capsule; portions of the capsule adherent to vital structures should be left undisturbed. Aseptic meningitis is the most common cause of postoperative morbidity, and its incidence may be minimized by intraoperative irrigation with steroids followed by systemic therapy with dexamethasone. Symptomatic recurrences that occur many years after surgery should be managed with conservative reoperation.

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Mitchel S. Berger, Lawrence H. Pitts, Mary Lovely, Michael S. B. Edwards and Henry M. Bartkowski

✓ A consecutive series of 37 children (17 years old and under) with severe head injury is presented. The data confirm that morbidity and mortality are lower in children than in adults: 51% of these young patients had a good recovery or moderate disability at 6 months. The mortality rate in this series (33%) is higher than in some reports, but probably more closely approximates the death rate from these injuries in an unselected pediatric population than do statistics from tertiary care hospitals. There was no significant relationship between age and outcome in this age group, but mass lesions and uncontrolled intracranial hypertension adversely affected outcome. Diffuse cerebral swelling was commonly seen on computerized tomography scans, and generally was associated with a satisfactory outcome (75%). Two of 13 deaths were considered preventable, emphasizing the narrow therapeutic safety margin and extreme care required in treating these patients.

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Mitchel S. Berger

✓ A skull-mounted apparatus is described for use with ultrasound probes 16 mm in diameter (5.0-MHz probes for near-field and 7.5-MHz probes for far-field lesions). The system permits ultrasound-guided stereotaxic biopsy of intracranial lesions through a burr hole in awake or anesthetized patients. This apparatus has been used in 19 patients for biopsy of central nervous system lesions 1.5 to 5 cm in diameter and for drainage of abscess cavities and cysts. The time required to obtain a tissue sample after incision of the skin ranged from 25 to 40 minutes. The only complication was a delayed hemorrhage in a patient with acquired immunodeficiency syndrome. The advantages of this method over those guided by computerized tomography (CT) include less time required for the entire procedure, immediate confirmation of the biopsied target by imaging the echogenic needle track, assessment of cyst or abscess drainage, and detection of hemorrhage within minutes after biopsy. The apparatus may be especially useful in pediatric patients because it obviates the need for general anesthesia during transport to and from the CT scanner. This ultrasound-guided system does not require a craniotomy, craniectomy, or two separate burr holes.