Instrumental activities of daily living in neuro-oncology: International validation of the EORTC IADL-BN32 questionnaire.

BACKGROUND: Neurocognitive impairments are common in patients with a brain tumour, and may negatively impact on functioning in daily life, particularly on instrumental activities of daily living (IADL). The EORTC IADL-BN32 questionnaire was developed to measure IADL in this patient population. METHODS: In this international validation study, we evaluated the EORTC IADL-BN32 questionnaire on several psychometric properties in a large sample of patients with a primary or metastatic brain tumour. We administered the 32-item questionnaire three times: at 'baseline', after 2 weeks and after 3 months. Procedures were in accordance with EORTC Quality of Life Group module development guidelines. RESULTS: In total, 326 patients participated in the study. A bifactor scale structure showed satisfactory model fit measures, with five multi-item scales and two single items, and an IADL sum score. The internal consistency of the multi-item scales ranged from good to excellent (range Cronbach's α: 0.86-0.97). We found significant differences in scale scores between patients with and without neurocognitive impairments or complaints, supporting the construct validity. Initial cross-cultural validity analyses showed indications of item response biases for certain items. Analyses indicated moderate to good test-retest agreement (intraclass correlation coefficient > 0.70) between baseline and the 2-week follow-up assessment for all but one scale. Deterioration of EORTC IADL-BN32 scale scores were consistent with clinically relevant deterioration on other functional measures with small to large effect sizes, however, subgroup sample sizes were small. CONCLUSION: Overall, the EORTC IADL-BN32 questionnaire exhibited adequate to excellent psychometric properties. Cross-cultural validity and responsiveness should be further explored.

Language testing in brain tumor patients.

Neuropsychological testing is not a luxury in the management of patients with brain tumors. Regardless of the therapeutic approach selected in each case (but, especially in the case of awake surgery), it provides information on the status of language and other cognitive functions. This information can be used preoperatively to identify eloquent areas and to provide a baseline against which further examinations can be evaluated, intraoperatively to identify eloquent areas, and post-operatively and at follow-up to ascertain the short-term and long-term consequences of surgery, as well as those of other treatments (chemotherapy and radiotherapy), and to plan and monitor rehabilitative treatments.

Cognitive outcome as part and parcel of clinical outcome in brain tumor surgery.

Although validated tools (neuropsychological tests, patient reported outcomes, mood and psychological profile) were first introduced many years ago in clinical practice, the impact of the tumor itself on patient cognition has not been extensively studied. Furthermore, while outcome research is evolving in an attempt to adapt the use of different tools to the preoperative and postoperative phases, the standard guidelines for evaluating outcome after brain surgery, by neurological examination and complication assessment, are frequently neglected in the current literature. This article attempts to provide an appraisal of the evidence for the impact of surgical treatment on cognitive function of brain tumor patients within the context of general outcome.

Cognitive outcome after awake surgery for tumors in language areas.

In surgery for tumors of the dominant hemisphere, the attention devoted to quality of resection and preservation of language function has not been accompanied by comparable interest in preservation of cognitive abilities which may affect quality of life. We studied 22 patients undergoing awake surgery for glioma removal in the language areas of the brain. Besides monitoring tumor variables (size, location, histology, edema), we used a multifaceted battery of tests to investigate mood, cognition, and language in an attempt to assess the burden of disease and treatment, and the relationships between these three dimensions. Baseline assessment showed that 45% of the patients were depressed and 23% anxious; some cognitive and language impairment was noted for 59 and 50%, respectively. A general decline in postoperative cognitive performance (significant for memory and attention only) and language function (significant for picture naming) was observed, whereas depression was unchanged and anxiety decreased. Tumor histology, but not demographic variables or extent of resection, correlated with postoperative cognitive changes: patients undergoing surgery for high-grade tumors were more likely to improve. No correlation was observed between scores for mood, cognition, and language function. A subset of patients with low-grade glioma was followed up for 3-6 months; although some improvement was observed they did not always regain their preoperative performance. In conclusion, we believe that cognitive assessment performed in conjunction with language testing is a necessary step in the global evaluation of brain tumor patients both before and after surgery.

Trigeminal laser-evoked potentials: a neurophysiological tool to detect post-surgical outcome in trigeminovascular contact neuralgia.

BACKGROUND: The aim of this study was to explore the nociceptive system of patients affected by trigeminal neuralgia (TN) secondary to documented vascular contact who underwent microvascular decompression. For that purpose, we used the classical trigeminal reflexes and the trigeminal laser-evoked potentials (tLEPs) before and after surgery, in order to verify any possible change after decompression and determine if there was any correlation between the neurophysiological parameters and the clinical outcome. METHODS: Eleven patients affected by TN caused by trigeminovascular contact and 10 age-matched controls underwent conventional trigeminal reflexes (bilateral Blink Reflex/BR and Masseter Inhibitory Reflex stimulating infraorbital and mental nerves/MIR V2 and V3) and tLEPs. Patients repeated neurophysiological tests one week after surgery. RESULTS: Short-latency BR and MIR were normal in all patients before surgery and there was no statistical difference before and after surgery. Conversely, in patients before surgery, tLEPs' amplitudes were significantly lower in the affected than in the healthy side (p = 0.017 for V2 and 0.037 for V3 branches). After surgery, on the affected side, tLEP amplitude increased and the pre/post-operative difference was significant (p = 0.017 for V2 and 0.028 for V3 divisions). Nine patients referred satisfactory pain relief and the favourable clinical outcome correlated with the neurophysiological recovery. CONCLUSIONS: This study demonstrates that TN caused by trigeminovascular compression may be related to Aδ fibres impairment, and tLEPs are more sensitive than conventional trigeminal reflexes to reveal small fibre dysfunction and to monitor the post-surgical outcome in these patients.

Microsurgical removal of petroclival meningiomas: a report of 33 patients.

This is a report of 33 consecutive cases of petroclival meningioma treated surgically at our institution over the last 10 years; there were 21 women and 12 men between the ages of 27 and 68 (mean age, 52). All patients were assessed by computed tomographic scans including coronal sections and bone algorithm studies; in most cases, digital subtraction angiography and magnetic resonance imaging were also done. The largest tumor diameter was between 2 and 3.5 cm in 14 cases, 3.5 to 6 cm in 15 cases, and over 6 cm in 4 cases. Dural attachment predominantly involved the clivus and apical petrous bone on one side only; in 14 cases, however, the tumor grew over the clivus midline or crossed the tentorial notch. Cranial nerve deficit was extant in all cases and was commensurate with tumor size. Cerebellar signs and somatic motor deficits were present in 60 and 30% of cases, respectively. The surgical approaches used were the retromastoid-retrosigmoid in 23 cases, subtemporal in 5 cases, and combined retromastoid subtemporal presigmoid in the remaining 5. Total removal was achieved in 26 cases (79%); incomplete removal occurred in 7 cases (21%). The extent of tumor removal and operative morbidity were not significantly related to tumor size. Brain stem indentation, arterial and cranial nerve encasement, and epidural invasion were the main factors that prevented total tumor removal and influenced operative morbidity. There was no intraoperative mortality, but three patients (9%) died perioperatively. In the postoperative period, most patients went through momentary neurological deterioration, chiefly due to new cranial nerve deficits. The average follow-up was 4.3 years in 27 patients; of these 17 were unchanged and 10 were improved. Before surgery, only 13 patients were self-sufficient; at long-term follow-up, another 6 had achieved independence. Our experience suggests that, even though real petroclival meningiomas still represent a formidable surgical challenge, such tumors can in most cases be removed completely with low attendant mortality and acceptable morbidity.

Assessment and surgical management of posterior fossa epidermoid tumors: report of 28 cases.

OBJECTIVE: The management of a series of 28 patients operated on for posterior fossa epidermoids is reviewed, emphasizing the need for long-term follow-up. We discuss the rationale for a comprehensive classification system that may allow the comparison of results from homogeneous series. METHODS: We grouped the tumors to differentiate the surgical management according to various tumor sites and the degree of extension. Twenty patients harbored tumors located in the cerebellopontine angle, five patients harbored tumors in the fourth ventricle, and three patients harbored tumors in the posterior fossa basal. In 17 patients, extensions of tumors outside the posterior fossa included the following regions: the suprasellar/ chiasmatic (n = 5), the parasellar/temporobasal (n = 5), and the mesencephalic/pineal (n = 7). Tumor extension was also defined by the number of regions involved. Pre- and postoperative magnetic resonance imaging and computed tomographic findings collected in 17 and 28 patients, respectively, were carefully evaluated. RESULTS: Clinical features and surgical approaches varied according to location and growth pattern. Fifty-seven percent of the tumors were completely removed. A higher total removal rate was achieved in patients with tumors confined to the primary location. One patient (3%) died in the perioperative period. Approximately half of the patients presented with transient mild focal deficit impairments resulting from the manipulation of the nervous structure over a wide area. There was a higher rate of surgical complications with fourth ventricle and mesencephalic extended cerebellopontine angle tumors. The mean follow-up period was 8.6 years. Thirty percent of the patients with subtotal removal experienced symptomatic recurrences after 8.1 years, whereas all patients with total removal were still asymptomatic. The recurrence-free survival rate was 95% at 13 years for patients with total removal compared with 65% for patients with subtotal removal. Problems of identification of tumor regrowth are discussed. CONCLUSION: By assessing posterior fossa epidermoids, we determined that location and extension play a major role in the prognosis. Our data suggest that more aggressive surgery is called for at first operation, and that a second operation should be planned when regrowth becomes symptomatic and/or tends to extend outside its original site.

Sequential measurements of cerebral blood flow in the acute phase of subarachnoid hemorrhage.

Cerebral blood flow (CBF) measurements were made at predetermined intervals over the first 20 days after a subarachnoid hemorrhage (SAH) by the method of 133Xe inhalation clearance in 39 patients, Hunt and Hess degrees I, II or III. Mean hemispheral and regional blood flow showed a similar pattern of behavior over time: namely an initial hypoperfusion, being more marked in patients with consistent or thick blood deposition at CT scanning, followed by transient improvement and subsequent deterioration in the second week, this being especially conspicuous in patients who developed vasospasm; deterioration, at any rate, was distributed widely regardless of the initial CBF readings or magnitude of bleeding. Patients developing later neurological deficit (23% of the total) were those who showed a statistically significant increase of hemispheric asymmetries and regional hypoperfusion at the time when deterioration occurred. Accordingly, the Author calls attention to the practical value of CBF measurements in SAH patients, in view of the relationships that obtain between certain CBF patterns and the emergence of late neurologic deficits.

Endosellar meningiomas: report of 2 cases and review of the literature.

Two cases of endosellar meningiomas are presented. The clinical and radiological picture is not always sufficiently specific to distinguish meningiomas from other types of sellar lesions, but pre-operative diagnosis is of fundamental importance in choosing the best surgical approach. In both cases, the transsphenoidal approach was used, and due to the dense consistency of the tumors, only biopsies were performed. One of the two patients was reoperated on using the sub-frontal approach for radical removal of the tumor. In the authors' experience, craniotomy proved to be the more favorable approach than the transsphenoidal route for radical excision of endosellar meningiomas. Literature on the classification of sellar meningiomas for radiological diagnosis and surgical strategy of endosellar meningiomas is reviewed.

Intracranial coexistence of neurinoma with epidermoid cyst or cholesterol granuloma. Report of 2 cases.

The authors present 2 cases of a rare association of intracranial tumors of different cell types: neurinoma with epidermoid cyst, and neurinoma with cholesterol granuloma. The presenting symptoms resulted from neurinomas arising from the V and VIII cranial nerves, respectively. The diagnoses were achieved using Magnetic Resonance Images (MRI). The association of these rare lesions is discussed using recent literature pertaining to the coexistence of multiple brain tumors.

Intracranial myxoid chondrosarcoma with early intradural growth.

Chondrosarcomas are extremely rare intracranial cartilaginous tumors of which the myxoid variant is the least reported in the literature. They develop extradurally and generally infiltrate the dura only in advanced stages or at recurrence. We describe the case of a 55-year-old woman with a posterior cranial fossa myxoid chondrosarcoma which had a primarily intradural extension.

Experience in "radical" surgery of supratentorial gliomas in adults.

This study reports the preliminary results obtained in a series of 107 patients with histologically proven malignant gliomas (86 glioblastomas and 21 anaplastic astrocytomas) operated upon between 1986 and 1989, with an aggressive attitude aimed to achieve extensive and possible "radical" excision of the tumor. Gross total removal was achieved in 62% of cases, while in the remaining the postoperative contrast enhanced CT scan showed more than 10-15% of residual tumor mass. There was no operative and postoperative mortality. The one-year survival rate was 60% in patients with total removal and 24% in those with partial resection. Furthermore the Karnofsky rating at discharge was improved in the former group while was unchanged in the latter. Although preliminary, these data seem to confirm the primary positive role of radical surgery in the combined management of malignant gliomas.

Choroid plexus papilloma of the cerebellopontine angle: a twelve patient series.

BACKGROUND: Choroid plexus papillomas (CPPs), of the cerebellopontine angle (CPA), are a rare entity and no surgical series have been reported so far. We reviewed all the pertinent literature of 12 patients operated on in the last 20 years at our institution. METHODS: All the patients were adults, ranging from 19 to 61 years. The group included 6 males and 6 females. Preoperatively, on computerized tomography (CT) (n = 10) or magnetic resonance imaging (MRI) (n = 4), differential diagnosis was difficult to achieve, especially with meningiomas. Hydrocephalus was disclosed in 8 cases. Angiography (n = 11) showed tumor blush with typical vascular supply in almost half the cases. RESULTS: In 6 patients a midline approach via the cerebellomedullary fissure was used; in the remaining 6 patients the retromastoid route was adopted. We found 2 "unconnected" tumors; no hilum was identified at surgery. Total tumor removal was achieved in 6 patients, predominantly in the last cases. Two patients died in the postoperative period, 3 patients had mild additional deficits, whereas 7 patients were stable or improved. All the patients were followed up for a mean period of 8.2 years. Conventional radiotherapy was carried out in 5 patients; 1 of them after tumor recurrence. Stereotactic radiotherapy was performed in 3 patients; 2 of them after recurrences. Two patients showed tumor progression and died during the follow-up. One of them presented a suprasellar metastasis and died much earlier (2 versus 7 years). CONCLUSION: Careful assessment and surgical planning is accomplished with the combined information from CT, MRI, and angiography. Typical characteristics are the following: vascular supply from the choroidal arteries, ventral extension, adhesion to the brainstem, and lower cranial nerves. Nowadays, total removal of CPPs of the CPA can be achieved with acceptable morbidity and mortality. In our experience, conventional radiotherapy did not prove effective.

Radiologic and surgical aspects of pure spinal epidural cavernous angiomas. Report on 5 cases and review of the literature.

BACKGROUND: Cavernous angiomas (CAs) that are localized completely in the spinal epidural space are uncommon vascular malformations. Although they have increasingly been reported in the literature in recent years, diagnostic and surgical features are not clearly defined. METHODS: We report five patients with pure spinal epidural cavernous angiomas (PSECAs) and review the literature, focusing on their radiologic and surgical characteristics. We also compare these tumors with other extra-axial CAs as well as with their intra-axial counterparts. RESULTS: PSECAs, like all other extra-axial CAs, differ from intra-axial ones on MRI: the hemorrhagic variant is less frequent, hemosiderin rim is rare, the signal is different, and contrast enhancement is the rule. They are very similar to spinal meningiomas but they differ in their growth pattern and morphology, since they infiltrate intervertebral foramina and have an oval shape. In PSECA, intraoperative bleeding is rarely profuse, in contrast to other extra-axial CAs, especially those of the cavernous sinus. CONCLUSIONS: On MRI, PSECAs and other extra-axial CAs constitute a homogeneous group since they enhance significantly. At operation, since there is rarely enough bleeding to limit removal, radical excision of PSECAs can be achieved with good results.

Direct surgery for brainstem tumours.

Updating a previous report, the authors offer a review of 45 patients between age 2 and 63 treated by direct surgical excision for brainstem tumours of various description. Since 1986 all candidate patients were examined by NMR imaging in addition to CT scanning, sometimes with the further addition of digital-subtraction vertebral angiography. By Epstein and McLeary's criteria, 24 of the tumours were focal, 12 were cervicomedullary and 9 were diffuse. The most frequent histological diagnosis was glioma (36 cases between low-grade astrocytoma, anaplastic astrocytoma and glioblastoma); the balance was provided by cavernoma (6 cases), haemangioblastoma (2 cases), and lipoma (2 cases). Gross total resection was achieved in 28 patients, namely all those with ependymoma or vascular tumours and 14 of 17 with low-grade astrocytoma. Resection was subtotal in 16 cases and confined to a generous biopsy in one. There was no operative mortality, but 2 deaths occurred in the early postoperative period. At discharge, neurological status was unchanged or improved in 35 cases. At 3-month follow-up examination, 12 patients were improved, 27 were unchanged and 3 were worsened. By January 1990 (6 to 72 months postoperatively) 27 of the first 40 patients treated were alive: 13 had resumed normal life, 6 were self-sufficient and 8 were disabled. The authors conclude that present-day microsurgical resection of intra-axial brainstem tumours is associated with low mortality and morbidity and affords favourable results for which they credit high-quality NMR imaging, efficient microsurgery, adequate anesthesia, and competent postoperative intensive care.

Conservative treatment of a traumatic direct low-flow carotid-cavernous sinus fistula: a case report.

A case of a 17-year-old boy presenting with a traumatic carotid-cavernous sinus fistula (CCSF), associated with an intracavernous pseudo-aneurysm, is reported. On angiography, the CCSF proved to be a direct and low-flow shunt. Conservative management was chosen and definitive closure of the fistula was obtained in two months by daily self-compression of the common carotid artery.

Dumbbell-shaped hypoglossal neurinoma: surgical removal via a dorsolateral transcondylar approach. A case report and review of the literature.

A case of dumbbell-shaped hypoglossal neurinoma with intra- and extracranial extension is reported. The tumour was surgically completely removed in a one-stage operation via a dorsolateral sub-occipital transcondylar approach. Clinical presentation and the role of high-resolution CT-scan, MRI and angio-MRI in diagnosis and surgical planning are discussed. We include a review of the literature concerning these rare tumours of the foramen magnum region.

Prognostic factors in childhood intracranial ependymomas: the role of age and tumor location.

Despite several clinical reports on intracranial ependymomas in children, the factors which affect prognosis, and the possibility that certain combinations of factors might limit survival, are still a matter of debate. Between 1976 and 1996 we operated on 35 children with intracranial ependymomas. Postoperative irradiation was given to 27 patients, with associated chemotherapy in 6 cases. Mean follow-up was 62 months. In 12 patients a 5-year follow-up was possible. In October 1996, 18 patients (51.4%) were still alive, the longest disease-free follow-up being 20 years, and the shortest 8 months. We analyzed the prognostic relevance of eight factors. For each factor, different subgroups were distinguished and compared as follows: age at diagnosis (<4 vs. >/=4 years), sex, tumor location (supratentorial vs. infratentorial), tumor size (<4 vs. 4-7 vs. >7 cm), surgical removal (total vs. subtotal), histology (low-grade vs. anaplastic), morphology (solid vs. cystic), adjuvant therapies (treatment vs. no treatment). Two-way contingency tables were made to identify associations between variables. The only significant association was between age and tumor location (p = 0.022): in children under 4, tumors were almost invariably located in the posterior fossa (9 out of 10 cases) with a clear preference for the lateral recess (8 cases). Other correlations were not significant. Kaplan-Meier survival curves were compared to assess the prognostic relevance of each factor. Survival was significantly lower for children under 4, for those with posterior fossa tumors, and for patients with residual tumor (p < 0.05). A multivariate analysis compared variables which significantly affected survival, revealing that age is the most important factor affecting prognosis (p < 0.05), while tumor location and surgical removal do not add any significance to the effect of age on survival. We conclude that age has the strongest prognostic relevance in childhood intracranial ependymomas, while the effect of tumor location on survival may be related to the high incidence of lateral recess ependymomas in younger children.

Brain tumors in children under 3 years of age. Recent experience (1987-1997) in 39 patients.

Brain tumors in children under 3 years of age differ in clinical presentation and pathological behavior from those in older patients. In this study, we reviewed data from 39 children (24 males and 15 females) under 3 years of age who were treated for intracranial brain tumors since the introduction of magnetic resonance imaging. The purpose was to assess correlations between clinicoradiological and treatment-related factors, and their impact on prognosis. The following factors were analyzed: sex, age, duration of symptoms, intracranial hypertension on admission, tumor location, surgical removal and histology. Associations between these factors and correlations with prognosis were determined using bivariate analyses (chi(2) test) and Kaplan-Meier survival curves. Collins' concept of a period of risk recurrence was tested. Mean follow-up was 41 months (range 0-136). In March 1998, 20 children were still alive (51.2%) with a mean survival time of 65 months (range 2-136). The incidence of supratentorial tumors was significantly higher in children less than 1 year old (p = 0.027). Lateral tumors were 9/10 (90%) supratentorial versus only 7/26 (27%) midline tumors (p = 0.001). Outcome (dead or alive) was significantly better (p = 0.037) for low-grade astrocytomas (9/12 = 75% survival) when compared to ependymomas (2/6 = 33%) and primitive neuroectodermal tumors (3/12 = 25%). Total tumor removal was achieved in 20 cases and was associated with a better outcome (65 vs. 33% survival; p = 0.049). Survival analysis confirmed a worse prognosis for children with ependymomas and primitive neuroectodermal tumors (p = 0.011) and revealed a worse survival for children with intracranial hypertension on admission (p = 0.047). Total tumor removal was associated with a longer survival, although not significantly (p = 0. 077). Finally, we found no exceptions to Collins' law.