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The long-term outcome after surgical treatment of dorsally exophytic brain-stem gliomas.

Dorsally exophytic brain-stem gliomas represent a distinctive subgroup of pediatric brain-stem neoplasms that are amenable to radical excision because of their benign histology and growth characteristics. However, their attachment to the floor of the fourth ventricle invariably precludes complete tumor excision. The long-term behavior of the residual tumor remains a subject of concern. To address this issue, the authors reviewed their experience with 18 dorsally exophytic brain-stem gliomas treated between 1974 and 1990. At operation, the tumors filled the fourth ventricle, fungating out of a broad-based area of the dorsal brain stem. The exophytic tumor was resected, but no attempt was made to remove tumor from the brain stem. Histological examination showed that 16 of the tumors were grade I or II astrocytomas, one was a ganglioglioma, and one was an otherwise benign-appearing glioma with several foci of anaplasia that was classified as a grade III astrocytoma. The latter patient was one of only two in the series to receive postoperative radiation therapy; both cases so treated have no evidence of disease on follow-up imaging studies 61 and 135 months postoperatively. One other child who had stable disease postoperatively died of shunt malfunction 18 months after tumor excision. Serial radiographic studies in the other 15 patients have shown no evidence of disease in three, stable residual disease in eight, and tumor enlargement 12, 28, 40, and 84 months postoperatively in four (median follow-up period 113 months). Each of the four patients with tumor regrowth underwent repeat tumor excision. Two of these children received perioperative radiation therapy at the time of disease progression and both showed reduction in tumor volume 28 and 65 months after their second operation. In contrast, both patients who did not receive radiotherapy at the time of disease progression had further tumor enlargement 48 and 84 months after their second operation and underwent a third tumor resection; one received postoperative radiation therapy and has no evidence of disease 58 months after his third operation and the other child has stable disease 27 months postoperatively. Histological examination of tumor specimens obtained at second and third operations showed no change from the appearance of the tumor on the initial resection. The authors conclude that the majority of dorsally exophytic brain-stem gliomas can be managed successfully with subtotal excision and, if necessary, cerebrospinal fluid diversion. The small percentage of tumors in this series that showed recurrent growth remained benign histologically.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗

[Neoplasms of the brain stem: clinico-pathologic correlations].

We report here the clinico-pathological data about 4 patients with brainstem tumors. Clinical symptoms were variable but allowed a correct topical diagnosis. On the other hand, all the neuroradiological examinations, especially CT scan of the brain, resulted always negative or without diagnostic value. Good results for the diagnosis were given by pneumoencephalography and ventriculography. The neuropathological examination revealed one spongioblastoma, two malignant astrocytomas and one pontine metastasis of carcinoma of esophagus. Diagnostic troubles, even with modern and sophisticated techniques are stressed here. The clinician should not overweight negative results obtained with these techniques and every time include in the differential diagnosis brainstem tumors.

Adult↗

[Alteration of the early R1-component of the blink reflex in coma: topographical and prognostic value (author's transl)].

Alteration of the early component of the blink reflex (delayed latency or abolished reflex) is considered to be relatively specific for pontine lesions (multiple sclerosis, vacular lesions of the brain-stem, neoplasms, syringes); provided, the trigeminal and facial nerve are unaffected. In comatose patients the electrically, by stimulation of the supraorbital nerve elicited blink reflex was super-imposed (50 or 100 responses) with the LAB 8/e-computer (off-line). It was found that the R1 component was abolished even in cases with supratentorial lesions and secondary brain-stem alterations, while no specific pontine lesions were demonstrated by neuropathological examination. Therefore it is suggested that delayed or abolished blink reflexes in coma due to supratentorial mass lesions are not concluslive for pontine lesions: they rather may reflect an unspecific secondary brain-stem alteration. Recording of the EEG reveals that in some cases the extinction of the early blink reflex precedes corresponding EEG alterations; this may be a matter of prognostic value.

Adolescent↗

REM sleep behavior disorder.

REM sleep is the stage associated with vivid dream mentation, desynchronous cortical EEG, and atonia of antigravitary muscles. REM sleep behavior disorder (RBD) is characterized by the intermittent loss of REM sleep atonia and by the appearance of elaborate motor activity associated with dream mentation. The animal model of REM sleep without atonia indicates that lesions to the perilocus coeruleus disrupt the excitatory connection to the nucleus reticularis magnocellularis in the descending medullary reticular formation and disable the hyperpolarization of the alpha spinal motoneurons. Extensive neurologic evaluations in humans suffering from both idiopathic and symptomatic forms have not identified specific lesions; however, findings in some patients suggest that diffuse lesions of the hemispheres, bilateral thalamic abnormalities, or primary brain-stem lesions may result in the RBD. Symptomatic RBD cases are associated with several neurologic disorders such as dementia, cerebrovascular diseases, multiple sclerosis, brain-stem neoplasm. RBD has been often documented to precede or to co-occur with neurodegenerative disorders, such as dementia, Parkinson's disease and multiple system atrophy. Most importantly, RBD is readily diagnosable and treatable. Patients and their bed partners usually report immediate improvement in sleep-related motor behavior with small doses of clonazepam.

Brain↗

Evaluation of pediatric CNS malignancies with (99m)Tc-methoxyisobutylisonitrile SPECT.

UNLABELLED: SPECT has the potential to add valuable information to the diagnosis and management of central nervous system (CNS) malignancy. Radioactive tracers including (99m)Tc-methoxyisobutylisonitrile (MIBI), or sestamibi, have been shown to be sensitive markers for brain tumors; however, their role in imaging children is poorly defined. METHODS: We undertook a pilot study of 29 pairs of (99m)Tc-MIBI and MRI images from 20 children to explore the clinical usefulness of this tracer in CNS malignancy. RESULTS: Tumor types that took up (99m)Tc-MIBI included brain stem glioma, fibrillary astrocytoma, other low-grade astrocytomas, and glioblastoma multiforme. Most tumors positive for (99m)Tc-MIBI uptake were astrocytomas, including those in the brain stem, cerebellum, and cortex. This method of nuclear imaging not only was able to identify the presence of a tumor but also could identify changes in the same tumor over time. Some correlation between histologic grade and (99m)Tc-MIBI uptake was observed. Several tumors, including craniopharyngioma, medulloblastoma, and optic glioma, were evident on MRI but not on (99m)Tc-MIBI SPECT. CONCLUSION: The results suggest that this modality is a potentially useful tool in the diagnosis and management of CNS malignancies, particularly higher-grade astrocytomas, in children.

Adolescent↗

[Eye findings in paediatric brain tumour - data basis for a follow-up proposal].

BACKGROUND: Morphologic alterations and functional disturbances of the visual system are frequently observed in children with brain tumours. At present we are not aware of published data giving recommendations for ophthalmic follow-up in these patients. PATIENTS: A retrospective analysis of 125 children with a brain tumour was done to perform a risk analysis. METHOD: According to a sequential test strategy risk factors for various eye findings have been evaluated. RESULTS: Tumour location is the major risk factor for ophthalmological findings. Eye motility pathology was mainly associated with tumour invasion of the brain stem, grade of malignancy, and mass effects and occurred mainly in those patients with the presence of visual system symptoms as the leading symptom at the time of diagnosis. VEP alterations were a frequent sign in any group of brain tumours. CONCLUSIONS: As a follow-up for children with brain tumour, we recommend to perform an ophthalmologic examination at the time of diagnosis, before and after any neurosurgery, radiotherapy and chemotherapy respectively. During chemotherapy controls should be performed every 3 months. After the end of treatment the patients should be seen by an ophthalmologist at least once a year lifelong. For some well defined risk populations, we recommend more frequent controls. Visual Evoked Potentials and perimetry ought to be performed in any child at diagnosis, and more often in defined risk patients.

Adolescent↗

[Anaesthesia for radiation therapy of brain tumours in children. A multidisciplinary challenge].

Radiation therapy of childhood intracranial malignancies is always a challenge for radiation oncologists, anaesthetists and paediatric oncologists. Detailed knowledge of the course of the disease prior to radiation therapy and a critical evaluation of the child's actual physical status are mandatory in each case. Furthermore the anaesthetist should be informed about the child's individual preferences and aversions. The optimum prearrangement of the radiation therapy is of paramount importance. Interdisciplinary communication structures which must always involve the child's parents have to be established. Perfect adjustment of the mask that fixes the head during each radiation procedure is necessary to give the child the possibility to breathe spontaneously without an endotracheal tube or a laryngeal mask. Two case reports highlight these aspects of the complex procedure of paediatric radiation therapy which are relevant for the anaesthetist.

Anesthesia↗

MR spectroscopy in the evaluation of recurrent contrast-enhancing lesions in the posterior fossa after tumor treatment.

Recurrent contrast-enhancing lesions arising within foci of prior brain neoplasms treated with chemotherapy and/or radiation therapy pose a significant diagnostic dilemma, as they may represent recurrent or residual tumor, treatment-related changes, or a combination of both. Those lesions specifically in the posterior fossa are even more difficult to assess, given the technical limitations of 2D CSI in the infratentorial compartment. We explored the feasibility of 2D-CSI MR spectroscopy in the evaluation of recurrent contrast-enhancing lesions in eight consecutive patients who had undergone treatment for posterior fossa or brainstem tumors. Mean Cho/Cr (choline/creatine) ratios obtained by 2D-CSI in recurrent tumor, treatment-related changes, and normal white matter were 2.93, 1.62, and 0.97, respectively, mean Cho/NAA (choline/N-Acetyl aspartate) ratios were 4.34, 1.74, and 0.93, and mean NAA/Cr (N-acetyl aspartate/creatine) ratios were 0.74, 0.92, and 1.26, respectively. In conclusion, also in the posterior fossa, MR spectroscopy is likely to be useful as an adjunct to conventional imaging characteristics in distinguishing recurrent tumor from treatment-related changes, irrespectively of the MRS technique used. In most cases spectra of diagnostic quality can be obtained using 2D-CSI to include coverage of both the lesion and its vicinity.

Adult↗