Operative or endovascular treatment of ruptured intracranial vertebral artery aneurysms?

Our purpose was to evaluate the surgical and endovascular treatment outcomes of ruptured intracranial vertebral artery aneurysms (RIVAA). The outcomes of 44 patients with RIVAA treated between 1983 and 1998 surgically (26), endovascularly (20) or both (2) were evaluated. The aneurysms were clipped in 24 patients, and clipped and wrapped in two. We treated 20 by the endovascular approach, 12 with Guglielmi detachable coils (GDC), and eight by parent-vessel occlusion using detachable balloons. Three patients had endovascular treatment after a failed or inadequate surgical attempt. Posttreatment follow-up was 17-183 months (mean 101 months) for surgically treated patients. For the GDC-treated group angiographic follow-up was carried at 8-49 months (mean 19 months). The condition of seven (27 %) of the surgically treated patients worsened due to procedure-related complications, compared with 10% in the endovascular treatment group. Of the patients initially presenting with Hunt and Hess grade IV or V, three of five (60%) died who were treated surgically and two of eight (25%) who were treated endovascularly. A good outcome was achieved in 17 surgically treated patients (85% of the survivors) and in 16 of the endovascular group (89% of the survivors). This present "same-site" report on treatment of a specific abnormality, RIVAA, treated surgically or by an endovascular approach indicates that especially in poorer Hunt and Hess grade patients, the latter may offer a clinical outcome as good as that of surgery, although long-term efficacy of GDC treatment is still to be determined.

Surgery, tamoxifen, carboplatin, and radiotherapy in the treatment of newly diagnosed glioblastoma patients.

A historically controlled phase II study was undertaken to investigate the efficacy and toxicity of a postoperative treatment consisting of high-dose continuous tamoxifen, carboplatin and radiotherapy in patients with newly diagnosed glioblastoma. Between 1995 and 1998, 50 patients with newly diagnosed glioblastomas underwent surgery and were subsequently treated with 200 mg day(-1) tamoxifen continuously, 3 cycles of carboplatin (300 mg m(-2)), and radiotherapy. Survival data for a historical control group were calculated from respective prognostic indices and were obtained from studies with comparable patient populations treated with operation and radiotherapy only. In our study, the median time to tumor progression was 30 weeks and the median survival time (MST) 55 weeks (95% confidence interval: 46-63 weeks). The MST of the control group (48 weeks) showed to be within this interval. In addition to already known prognostic factors in malignant gliomas (age, Karnofsky performance score, extent of tumor resection), the gender (females lived longer than males, p = 0.0025) showed to influence survival. Serious side effects (thrombosis, pulmonary embolism) occurred in 6 patients. A high incidence of multifocal tumor recurrences (33%), which might be related to study-treatment, was observed. In conclusion, the combined therapy failed to demonstrate a higher efficacy than standard treatment for glioblastoma patients.

Surgical management of intramedullary hemangioblastoma of the spinal cord.

This is a study on 19 patients, who underwent complete excision of an intramedullary hemangioblastoma of the spinal cord during the period 1984-1997. The study was conducted to evaluate their functional outcome. Some peculiarities of their clinical presentation and surgical treatment are discussed as well. There were 12 males and 7 females whose age ranged from 16 to 69 years. Five of 6 patients were affected by Lindau's disease had multiple intramedullary tumors. The length of their history averaged 22.6 months. While pain was the most common complaint at presentation, 12 out of 19 patients had progressive sensorimotor deficits. A total of 22 operation was performed. One patient underwent resection of a minute tumor residue a few months after the first operation. In 2 patients with multiple tumors a second tumor, which became clinically relevant, was resected 17 and 36 months after the first operation. There was no mortality. One patients developed a wound infection which required secondary closure. The functional status of the patients registered at discharge was worse in 22.7%, unchanged in 59.1%, and improved in 18.2% of the patients. At follow-up (6-142 months), the status of 9.1% of the patients was still worse, in 50% was unchanged and in 40.9% better than the preoperative one. All but one patients had complete postoperative pain relief. The data support the concept that radical excision of intramedullary hemangioblastomas can be achieved at low levels of surgical mortality and morbidity. Symptomatic patients should undergo surgery before they develop extensive sensorimotor deficits. In patients with multiple lesions, tumors distant from the symptomatic one should not be tackled.

[Suprasellar meningioma. A disease still frequently diagnosed too late]. / Das supraselläre Meningeom. Ein immer noch häufig sehr spät erkanntes Krankheitsbild.

BACKGROUND AND OBJECTIVE: Suprasellar meningioma continues to be diagnosed very late after the onset of first eye symptoms. This study was aimed at demonstrating the effect of delay on the long-term visual loss. PATIENTS AND METHODS: In the course of a retrospective study all 53 consecutive patients operated on for suprasellar meningioma from 1982 to 1991 were contacted (47 women, 6 men; average age 49.5 years) 46 of the 49 surviving consented to the follow-up investigation. The extent of preoperative visual loss, tumour size, presence of optic nerve atrophy and duration of visual loss, data that provide an indirect measure of how soon the correct diagnosis was made, were analysed with regard to their effect on long-term ophthalmological results. RESULTS: The mean period elapsing from onset of first visual symptoms to the definitive diagnosis of suprasellar meningioma was 22.3 months. The data showed that the long-term results were the worse the later the diagnosis was made. CONCLUSIONS: The commonly very late diagnosis of suprasellar meningioma as cause of visual loss is an international problem and is presumably due to the low incidence of the tumour (1-2 cases per 1 mill. population per year). If long-term results are to be improved, primary care doctors must be made aware of the differential diagnosis of visual loss caused by pressure from a tumour.

Treatment and long-term outcome of pineal nongerminomatous germ cell tumors.

In 1994 we reported on a small series of 3 children with malignant, marker-positive pineal nongerminomatous germ cell tumors treated with a 'sandwich' protocol. Here, we report on the long-term survival of these children. Preoperative chemotherapy consisted of two courses of bleomycin, etoposide, and cisplatin. En bloc resection of the tumors via the supracerebellar, infratentorial route was performed immediately after decline of tumor marker levels. Postoperatively, two courses of vinblastine, ifosfamide, and cisplatin were applied, followed by craniospinal irradiation. The patients showed no major neurological deficits and no evidence (neuroradiologically or with regard to tumor marker levels) of recurrence of disease after 66, 71, and 78 months, respectively. We propose this regimen for children with tumors of the pineal region in whom the tumor markers are positive. It should be started without histological classification of the tumor to avoid possible spillage of malignant tumor cells to the cerebrospinal fluid.

Tissue culture of human neurocytomas induces the expression of glial fibrilary acidic protein.

Cell cultures were established from three human neurocytoma specimens (primary and recurrent). The phenotypic evolution was analyzed by immunocytology in different culture conditions in the presence and absence of serum including the addition of epidermal growth factor, rat caudate extract, retinoic acid, and N-acetyl cystein. The cells were grown on glass cover slides or an extracellular matrix (ECM) from bovine corneal endothelial cells. Immunostainings were performed after overnight incubation and were repeated after 5 and 10 days of culture. The cultures were compared to an oligoastrocytoma also arising at the foramen of Monro and an ependymoma of the frontal lateral ventricle, two tumors supposedly originating from the same tissue matrix as the neurocytoma. After overnight incubation, 90% of the neurocytoma cells were positive for A2B5 and synaptophysin. GFAP reactivity appeared in the periphery of cell processes in less than 1% of the cells. The staining patterns and morphology were nearly identical under the different culture conditions. After 5 days, almost all cells were strongly positive for GFAP, while the number of cells remaining positive for synaptophysin and A2B5 was unchanged from the earlier time point. Again, there were no fundamental differences between the incubation conditions. At this point, cultures maintained on ECM were compared to their counterparts on untreated glass cover slides with identical staining results, although many fewer cells had attached. An identical immuno-reactive pattern was found on day 10. In contrast to the neurocytoma cultures, there was an immediate strong GFAP signal in both the mixed glioma and the ependymoma. A2B5 was also positive, but synaptophysin was absent. Because the neurocytoma specimens were synaptophysin positive but GFAP negative by immunohistochemistry, it is concluded that neurocytomas may represent a human neuronoglial precursor tumor that switches its phenotype in culture to astroglial differentiation despite very diverse culture conditions.

Preoperative functional magnetic resonance imaging (fMRI) of the motor system in patients with tumours in the parietal lobe.

Intracranial lesions may compromise structures critical for motor performance, and mapping of the cortex, especially of the motor hand area, is important to reduce postoperative morbidity. We investigated nine patients with parietal lobe tumours and used functional MRI sensitized to changes in blood oxygenation to define the different motor areas, especially the primary sensorimotor cortex, in relation to the localization of the tumour. Activation was determined by pixel-by-pixel correlation of the signal intensity time course with a reference waveform equivalent to the stimulus protocol. All subjects showed significant activation of the primary sensorimotor cortex while performing a finger opposition task with the affected and unaffected side. In five patients the finger opposition task additionally activated the ipsilateral sensorimotor cortex and the supplementary motor area (SMA). Extension and flexion of the foot, additionally performed in two patients, also activated the sensorimotor cortex, in one case within the perifocal oedema of the tumour. Tumour localization near the central sulcus induced displacement of the sensorimotor cortex as compared to the unaffected side in all patients with a relevant mass effect. The results of our study demonstrate that functional MRI at 1.5 T with a clinically used tomograph can reproducibly localize critical brain regions in patients with intracranial lesions.

Suprasellar meningiomas--neurological and visual outcome at long-term follow-up in a homogeneous series of patients treated microsurgically.

Most of the previously published surgical series of suprasellar meningiomas have two disadvantages: (1) patients involved were treated within a relatively long time period, making analysis more difficult, (2) radiographic long term follow-up examinations with either CT- or MRI-scans were not performed. Both disadvantages were overcome in our retrospective clinical study, consisting of 50 consecutive patients with suprasellar meningiomas treated between 1982 and 1991. Radiological, ophthalmological, and neurological investigations were performed preoperatively, postoperatively and at long term follow-up (mean: 5.7 years). A radiologically confirmed radical tumour removal could be achieved in 84% of patients. Both, the peri-operative mortality (2%) and serious operative morbidity (6%) were low. However, 12% of patients developed late onset epilepsy. At long term follow-up, visual function was improved in 67%, unchanged in 9% and worsened in 24%. In more than 50% of patients the vision showed recovery over a longer time period than the first 10 days after operation. Radiographic control examinations revealed tumour recurrences in 2 patients (both asymptomatic) and progress of residual tumour in 5 patients (2 symptomatic, 3 asymptomatic). Since introduction of modern neurosurgery, a clear improvement in the surgical treatment of suprasellar meningiomas can be observed. However, the still long delay in diagnosing these tumours correctly prevents a further improvement of the ophthalmological results at long-term follow-up. Due to a relatively high rate of late onset epilepsy, anticonvulsive prophylaxis for 6 months seems to be justified. Regarding present preoperative diagnostic measures, ia-DSA seems only be indicated in patients with CT/MRI-scans, suspicious for tumourous narrowing or invasion of major cerebral arteries. In addition, we recommend radiographic control examinations at regular time intervals to confirm radical tumour removal and to detect the "ideal" point of time for renewed treatment.

[Asymptomatic cerebral aneurysms--surgical and endovascular therapy options]. / Asymptomatische zerebrale Aneurysmen--Chirurgische und endovaskuläre Therapieoptionen.

Aneurysmal subarachnoid hemorrhage carries a risk of 50% mortality. Therefore it is recommended to also treat asymptomatic, previously unruptured aneurysms. Deciding whether to treat or to observe depends on the consideration of risk of hemorrhage and the surgical risk. Such decision could be facilitated if predisposing factors affecting the outcome were known. 15 Patients with 19 unruptured aneurysms managed in a period when both, surgical and endovascular treatment options were available, were selected from a group of 47 asymptomatic patients documented since 1984. The patients were either operated (n = 9) or were treated by endovascular occlusion with GDC coils (n = 6). In most cases, a long history of headaches eventually lead to a CT-scan or MRI in which a suspicious abnormality was found. Upon subsequent angiography, 8 middle cerebral, 6 carotid, 3 basilar and 1 pericallosal, and ophthalmic artery aneurysm(s), respectively, were found. The aneurysms varied from 4 to 30 mm in diameter. The patients were followed between 9 months and 2.5 years. Treatment results in this small series were independent from size, location, age or sex of the patient. The initial, mostly unrelated symptoms persisted after treatment in most cases. The morbidity according to GOS in this small series (3 out of 19 patients deteriorated) is not representative of the complete series where morbidity and mortality was 5.4% and 1.2% respectively). In agreement with the literature reviewed herein, we recommend surgical treatment of previously unruptured aneurysms, especially when the patients are below 60 years of age. In patients with posterior circulation aneurysms, endovascular coil occlusion is a valid alternative, especially in older patients with concurrent medical problems.

Intracranial germ-cell tumours--treatment results and residuals.

UNLABELLED: There is only sparse information about the development of children after successful treatment for intracranial germ-cell tumours. Between January 1981 and June 1992, 26 children with intracranial germ-cell tumours were treated in the University Hospital Hamburg-Eppendorf. We report on treatment results, long standing residuals and the "quality of life" of these patients. The long-term event-free survival was 88% for the germinomas and 43% for the malignant teratomas. Of the patients 58% had no relevant functional neurological deficits and 37% had mild impairment. Only 1 patient with metastatic disease was severely handicapped. Six patients showed neuro-endocrine dysfunction. All of them had suprasellar/hypothalamic lesions and all received successful substitution therapy. As to neuropsychological functions, 53% of the patients had no or only mild disturbances. The most affected function was speed of information processing. Of the children 69% were able to proceed with their education at the same level as before therapy. The overall self-assessment revealed good results in 75% of the patients. CONCLUSION: After surgical removal and radiation therapy long-term survival of intracranial germinomas amounts to 88%. Despite craniospinal axis radiation severe residuals are rare and a good quality of life is common. In malignant teratomas treatment regimens including chemotherapy are much less successful.

Central neurocytoma: clinical, immunohistologic, and biologic findings of a human neuroglial progenitor tumor.

BACKGROUND: Central neurocytomas are rare brain tumors recognized by their typical radiologic and histologic features. In general, a good prognosis is achieved by total removal. The histogenesis is still under debate, but a neuronal origin is widely assumed. METHODS: This study presents the clinical and immunohistologic findings of five patients and the results of cell culture experiments of two patients with central neurocytoma treated surgically between 1983 and 1993. RESULTS: The patient age at diagnosis ranged from 21 to 30 years (mean, 25 years). The male-to-female ration was 1:4. Raised intracranial pressure due to hydrocephalus was the main cause of the clinical manifestations. Total resection was achieved in two cases. Four patients received radiotherapy. One patient suffered a recurrence 1 year after surgery, requiring a second resection and radiotherapy. Follow-up studies took place between 1 and 10.5 years (mean, 7.1 years). To date, all patients are free of their tumors. Two patients suffered from permanent memory disturbances after surgery. Immunohistochemistry confirmed the neuronal nature of the tumors. Cell-culture studies, which have been carried out for the first time, demonstrated concomitant expression of neuronal (synaptophysin) and glial (GFAP) markers. CONCLUSION: Total removal is the therapy of choice. In tumor recurrence or limited surgery (e.g. due to severe affliction of the fornical structures), radiotherapy has shown to be efficacious. The cell-culture experiments give new insight on the histogenesis of central neurocytoma, indicating that the tumor arises from an undifferentiated precursor cell with the capacity of bipotential neuroglial differentiation.

Malignant chiasmal glioma: a rare cause of rapid visual loss.

The clinical, neuroradiological, and histological findings of an adult patient suffering from malignant optic glioma is reported. Rapid visual deterioration was misdiagnosed for several months until biopsy confirmed the tumor. The patient died despite radiation therapy nine months after first symptoms. Our presentation will focus on the problems of diagnosing and establishing therapeutic procedures in this rare malignant neoplasm.

Human neurocytoma cells in culture show characteristics of astroglial differentiation.

Cultured human neurocytoma cells from two neurosurgical patients were analysed for their immunocytochemical staining patterns and growth characteristics. In both cases, the cells stained positive for glial acidic fibrillary protein (GFAP) within one day of tissue culture in medium, with and without fetal calf serum, whereas the histological tumor specimens were negative. Both cases contained cells concomitantly expressing GFAP and synaptophysin (SNP) in the primary cultures. Epidermal growth factor (EGF) was mitogenic for the cultured cells but not platelet derived growth factor alpha (PDGF AA) or nerve growth factor (NGF). It is concluded that the human neurocytomas may represent neoplasms of a pluripotent neuroglial cell which can provide an interesting model to study the determinants for human glial/neuronal differentiation in vitro.

Surgical management of intramedullary spinal cord tumors: functional outcome and sources of morbidity.

We are reporting the functional outcome of 69 of 86 patients affected by intramedullary spinal cord tumors who underwent surgery at our department during the period of 1984 to 1992. The results on 17 patients affected by hemangioblastomas and cavernomas were excluded from this study and will be published separately. Twenty-eight patients had astrocytic processes; 34 had ependymoma; 4 had lipoma; 2 had neurofibroma; and 1 had oligodendroglioma. The overall rate of "radically" resected tumors was 55.1%, as opposed to 17.4% "quasiradically," and 27.5% of "partially" resected processes. There was one postoperative death. Five other patients, affected by anaplastic astrocytomas, died because of tumor progress within 16 months from the operation. A postoperative functional assessment showed that the function of the upper and lower extremities had deteriorated in 65.4 and 55.1% of the patients; a respective functional deterioration by 1 degree of the scale of Cooper and Epstein was registered in 88.8 and 86.8% of the patients. The patients who recovered improved within a period of 6 to 18 months, whereas the function of the dorsal columns was impaired the longest. At follow-up (mean, 54 mo; range, 8-107 mo), the functional recovery (as compared with the preoperative status) was as follows: upper extremity, 17.1% of the patients were improved, 55.5% were unchanged, and 31.5% were worse (89.4% by 1 degree); lower extremity, 22.4% of the patients were improved, 51.5% were unchanged, and 29.4% were worse (most by 1 degree). Surgery on tumors of the cervicothoracic and upper thoracic region carried a relatively higher morbidity in this series.(ABSTRACT TRUNCATED AT 250 WORDS)

Hypothalamic hamartomas: with special reference to gelastic epilepsy and surgery.

This study presents six patients with hypothalamic hamartomas diagnosed on the basis of magnetic resonance imaging. Histological confirmation was performed in three patients who underwent surgery. Immunohistological assays were used to determine the neurosecretory pattern. Four patients presented with epilepsy, including gelastic seizures. Other symptoms included behavioral abnormalities in four patients and precocious puberty and visual impairment in two patients. One patient presented associated developmental defects. Good results without morbidity were achieved with surgical resectioning in two patients with large hamartomas associated with behavioral abnormalities and gelastic epilepsy that was unresponsive to conventional medical treatment and in one patient with visual impairment. We propose a classification of the hypothalamic hamartomas based on topographical and clinical data obtained from 36 selected cases in the literature and six of our own cases. This classification should help to classify the various treatment methods and the surgical risks into four subgroups (Types la, lb, lla, and llb). We conclude that the surgical approach is a realistic alternative in certain cases, including large and broad-based Type llb hamartomas associated with gelastic epilepsy and behavioral disorders.

Treatment of nongerminomatous germ-cell tumors of the pineal region.

Germ-cell tumors can be subdivided into germinoma, embryonal carcinoma, choriocarcinoma, endodermal sinus tumor (yolk-sac tumor), and teratoma. They are also distinguished by their production of secreted markers such as alpha-fetoprotein produced in endodermal sinus tumors and embryonal carcinoma or beta-human chorionic gonadotropin, produced by choriocarcinoma and embryonal carcinoma. Germinoma and teratoma produce none of the markers. Because it has been proposed that teratomas may differentiate from multipotent stem cells contained in embryonal carcinoma and are thus lineage related, the presence of markers indicates the presence of a nongerminomatous germ-cell tumor. Nongerminomatous germ-cell tumors are an invariably fatal subgroup within the pediatric pineal region germ-cell tumors. There is no effective, established therapeutic regimen. We report the treatment regimen for three children diagnosed with this highly aggressive tumor entity. The children were first given a course of chemotherapy with bleomycin, etoposide, and cisplatin. This resulted in the normalization of markers and the shrinkage of tumors. These were then removed by the infratentorial supracerebellar approach. Removal was followed by a second course of chemotherapy with vinblastine, ifosfamide, and cisplatin; after which the children underwent radiotherapy. All three children are well and without evidence of residual or recurrent disease 20, 30, and 32 months after surgery, respectively. We propose this therapy regimen for children in whom the markers are positive.