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[Transient cerebellar mutism after removal of a posterior fossa tumor in two cases].

We have reported the cases of two young patients who presented transient mutism in the course of recovery from removal of a cerebellar medulloblastoma. Although cerebellar symptoms were observed immediately after surgery, neither consciousness disturbance nor sensory aphasia was observed when the patients were mutic. From the analysis of serial magnetic resonance imaging (MRI). Gd-enhanced regions were noticed in the dentate nucleus and the cerebellar peduncle when mutism appeared, and they disappeared when mutism was gone. Although the mechanism of this interesting symptom is not clear, these MRI findings may indicate that focal ischemia or edema associated with surgical procedure may play a role in the appearance of this symptom.

Adolescent↗

Excretion of metabolites of biogenic amines in patients with irradiated brain tumours.

The metabolities of biogenic amines were determined in the 24-hour urine samples of patients submitted to surgical removal of a malignant brain tumour and subsequently to telecobalt therapy of the corresponding head region. A significant increase in the excretion of 5-hydroxyindoleacetic acid (5-HIAA), vanillinmandelic acid (VMA) as well as of free 3-methoxy-4-hydroxy-phenylglycol (MHPG) during the period of irradiation was found. This increase is presumably the result of radiation induced release of their parent amines from the brain; in the case of VMA the secondary response of the peripheral sympathetic system might occur.

Adenoma↗

[Use of cyclosporin and verapamil in association with chemotherapy in the treatment of pediatric patients with advanced-stage neoplasms. A pilot study].

Multidrug resistance represents one of the most important factors that may lead to a therapeutic failure in some patients affected by malignancies. One of the best known mechanisms is linked to the genic amplification or the overproduction of a membrane glycoprotein, GP170, that is the product of the gene MDR1. The existence of drugs (calcium blockers, cyclosporine, tamoxifen, reserpine, quinidine) able to bind themselves to gp170 and to paralyze its activity in vitro is well known. We studied 20 pediatric patients (median age 9 years) affected by acute lymphoblastic leukemia (ALL), osteosarcoma, neuroblastoma and medulloblastoma, in advanced stage of disease. We employed in all cases the association of cytostatics with verapamil (50-70 mg/m2 i.v.) and cyclosporine (5-8 mg/kg i.v.) with different infusion schedules. In leukemias we administered vincristine (1.5 mg/m2), and daunomycin (40 mg/m2), in solid tumors VP16 (150 mg/m2) and adriamycin (60 mg/m2). Seventy-two therapeutic courses were performed: 39 in ALL, 16 in osteosarcoma, 16 in neuroblastoma and 1 in medulloblastoma. On the whole 5 complete remissions were achieved in ALL patients and 1 in an osteosarcoma patient. We did not observe a significant myelosuppression during treatment, therefore few infectious complications occurred; furthermore electrocardiographic changes have been mild and promptly resolved after temporary discontinuation of verapamil infusion. Our data suggest a synergy of verapamil and cyclosporine in the inhibition of multidrug resistance induced by gp170, without the occurrence of heavy toxicity. The results obtained in ALL patients are encouraging., especially in view of a possible subsequent bone marrow transplantation, while in solid tumors they are not as satisfying.

Adolescent↗

[Seven cases of subependymoma. Anatomoclinical study and review of the literature].

Subependymomas are uncommon, benign, slowly growing lesions usually located in the fourth ventricle. Their morphology is characteristic, but their histogenesis remains controversial. Seven cases observed in our laboratory over a period of 7 years are reported. Most tumors were located axially in the brain stem, and symptoms were more variable than usually reported. The histological appearance was fairly uniform. Immunohistochemistry showed that the fibrillary component was composed of glial fibers. Ependymary differentiation is inconstant on electron microscopy. The pathogenesis of the lesion (tumor, hamartoma or reactive proliferation) is debated in the literature as is the histogenesis.

Adult↗

Intracranial meningiomas following irradiation therapy for brain tumors.

Two cases of radiation-induced meningiomas following therapeutic irradiation given for primary diagnosed malignant brain tumor are presented. The meningiomas, histologically different from the initial brain tumor, appeared 15 and 21 years after high-dose brain irradiation. The possible risk of carcinogenesis within the CNS resulted from therapeutic cranial irradiation is stressed.

Brain↗

Linear contrast enhancement at the operative site on early post-operative CT after removal of brain tumors.

It is unclear whether a linear contrast enhancement at the edges of the operative site on early CT scan indicates a residual tumor or rather reflects postoperative changes. We have studied 15 patients treated with surgical removal of malignant brain tumor, submitted to periodic CT scan. The enhancement of linear contrast was due to a residual tumor, when seen on very early CT scan (within 24 hours after surgery). If occurring later, this enhancement was either generated by a residual tumor or by post-operative changes.

Adolescent↗

[Clinical aspects of cerebral metastases (author's transl)].

Cerebral metastases are the most common intracranial tumors next to the malignant glioblastoma multiforme. The clinical aspects are described in summary with reference to 191 cases. At least 1/3 of the cerebral metastases are already multiple on diagnosis. The daughter tumors most frequently colonize in the cerebrum - in the centroparietal region in particular - and much more seldom in the cerebellum. Bronchial and mammary carcinomata are the most important primary tumors; melanomas and hypernephromas come next in frequency. The primary tumor escapes clinical detection relatively frequently. The first symptoms are most frequently signs of cerebral pressure. Of the local symptoms of cerebral metastases, the hemi-syndrome occupies first place.

Age Factors↗

Management of cranial and spinal cavernous angiomas (honored guest lecture).

The management of patients with cavernous angioma continues to evolve. Our current recommendations for management are as follows. 1. Patients who are asymptomatic are observed. 2. Patients with acute severe or progressive neurological deficits are operated upon. 3. Patients presenting with a seizure are usually operated upon but some are observed, depending on the factors discussed. 4. Patients with a single hemorrhage in the cerebrum, cerebellum, or spinal cord are usually operated upon. When the hemorrhage is in the brainstem, thalamus, or basal ganglia, they are observed. 5. Patients with a recurrent hemorrhage are usually operated upon but there are exceptions when the lesion is in a deep area with high surgical risk.

Adolescent↗

[Malignant cerebral tumors in children].

Malignant brain tumours (mainly medulloblastoma, glioma, and ependymoma) are the first cause of solid tumours in children, and a major cause of mortality from cancer in paediatrics. The most frequent circumstance of discovery is intracranial hypertension. Early and atypical signs should give the alarm and call for emergency neuroradiological explorations. Major therapeutic advances have been made in the last ten years in the fields of neurosurgery, radiotherapy and chemotherapy of brain tumours in children. Multidisciplinary care is now indispensable usually as part of multicentre cooperative clinical trials. Therapeutic approaches are guided by a concern not only for effectiveness, but also for low toxicity, in order to reduce the long-term sequelae often caused by irradiation of the developing central nervous system.

Brain Neoplasms↗

Rehabilitation outcome in children after treatment of primary brain tumor.

Functional outcome studies after treatment of primary brain tumors in children are lacking. This study of 30 children, ages 3 to 20 years (x 10.8 yrs) was aimed at documenting the effects of rehabilitation on functional outcome after treatment of primary brain tumors. Functional measurements were documented by a modified functional independence measure for children (WeeFIM). The paired t-test, Wilcoxon test, chi 2 analysis, and Friedman test were used to assess significance of data. Statistically significant improvements were documented from admission to discharge, and discharge to follow-up in total WeeFIM scores (p = 0.001 and p = 0.0001) and specifically in the subgroups of self care, mobility, and locomotion. Though no significant improvement was noted in sphincter control from admission to discharge (p = 0.15), significant gains were seen at follow-up (p = 0.006). Borderline improvements at discharge in communication (p = 0.054) and social cognition (p = 0.051) became significant at follow-up (p = 0.01 and p = 0.004). At admission, 7 patients (23%) were independent in self care, 5 (17%) in mobility and 1 (3%) in locomotion compared with 18 (60%), 20 (67%), and 15 (50%) respectively at discharge. At follow-up there was further improvement with 95% independent in self care, 100% in mobility and 70% in locomotion. This study affirms the beneficial effect of comprehensive rehabilitation on functional outcome in children with residual disabilities after treatment of their primary brain tumors.

Adolescent↗

Aggressive fibromatosis (fibrosarcoma) of the facial nerve.

Aggressive fibromatosis of the facial nerve is a very rare tumor. Three cases have been previously reported. The tumor is locally recurrent and often has a fatal outcome. This report is of a ten-year-old boy whose tumor originally developed in the parotid area with subsequent spread to the base of the skull, the neck and the cerebellopontine angle. Treatment was by wide surgical excision, radiation therapy and chemotherapy. Despite treatment, the patient died. The clinical features of this case will be described. Only wide surgical excision early in the course of the disease may offer a chance for cure.

Brain↗

[Cerebral tumors and pregnancy. Apropos of 8 cases].

Pregnancy is an aggravating factor for brain tumours on which it acts by three mechanism: acceleration of tumour growth, increase of peritumoral oedema and the immunotolerance to foreign tissue antigens that is proper to pregnancy. Histologically, the brain tumour most frequently encountered is glioma, usually revealed during the third trimester. Brain tumours is pregnant women have no special clinical features, and their diagnosis rests on computerized tomography or nuclear magnetic resonance completed, if required, by stereotactic biopsy. Following a review of the literature, the authors present an updated description of the neurological and obstetrical actions to be taken, illustrated by a report of eight personal cases. The indications for surgery depend on the site and histological nature of the tumour. As regards obstetrical measures, induced therapeutic abortion and caesarean section, no longer routinely performed, are now being replaced by vaginal delivery with systematic instrumental extraction. In both mother and foetus the prognosis has improved over the last ten year, but it remains very sombre.

Adult↗

[Prognosis in primary tumors of the central nervous system. A patient material from the Norwegian Radium Hospital 1960-94].

We present the results of a retrospective survey of 1,218 patients treated at the Norwegian Radium Hospital during the years 1980-94 for primary tumours of the central nervous system. Median survival for patients with glioblastoma (n = 492) was 12 months, for patients with anaplastic astrocytoma (n = 83) 25 months, astrocytoma (n = 260) 95 months, oligodendroglioma (n = 85) 74 months, mixed glioma (n = 68) 65 months, and medulloblastoma (n = 53) 109 months. Median survival for patients with brain stem tumours (n = 37) was nine months, while 74% of patients with tumours in the pineal region (n = 38) survived for five years. The histology and localisation of the tumour, as well as age and functional status, are important prognostic factors for survival in patients with primary CNS tumours.

Adolescent↗

Up-regulation of specific NF 1 gene transcripts in sporadic pilocytic astrocytomas.

Pilocytic astrocytomas of the optic nerve (optic nerve gliomas) are closely associated with neurofibromatosis 1 (NF1), and allelic losses of the NF1 gene region on chromosome 17q occur in sporadic pilocytic astrocytomas. We therefore hypothesized that the NF1 gene acts as a tumor suppressor gene in pilocytic astrocytomas, and that NF1 gene expression would be reduced or absent in these tumors. To evaluate this possibility, we examined quantitative and qualitative aspects of NF1 gene expression in six sporadic pilocytic astrocytomas. Surprisingly, the NF1 gene was overexpressed up to fourfold in these tumors when compared with normal brain. This up-regulation was accompanied by immunohistochemical positivity using a carboxyl-terminal antibody and by the absence of mutations in one kilobase of the NF1 coding sequence, consistent with the expressed transcript and protein being full length and probably wild type. Pilocytic astrocytomas showed a marked predominance of transcripts containing exon 23a and lacking exon 9br, the same isoforms that are expressed by normal and reactive astrocytes and malignant astrocytomas. These data illustrate that pilocytic astrocytomas overexpress specific NF1 gene transcripts, perhaps as a regulatory response to growth stimuli. The role of the NF1 gene as a tumor suppressor in pilocytic astrocytomas, however, remains to be proven.

Adolescent↗