Search

In the last few years much has been published to validate new technology in brain mapping for clinical purposes, but there have been few clinical results. In this report we describe our five-year experience in the surgical management of malignant gliomas around motor areas with an evaluation of the impact of functional magnetic resonance imaging (fMRI) plus navigator and intraoperative neurophysiology (IN). End-points were extent of removal, morbidity, and survival. Variables describing patient and tumor characteristics and treatment modalities were statistically weighted in relation to treatment outcome. Tumor depth (P = 0.01), midline shift >/=1 cm. (P = 0.05), and insular location (P = 0.001) negatively affected extent of removal, whereas IN (P < 0.001) and fMRI plus navigator (P = 0.02) contributed to increasing the rate of total removal (73\%, 71\% vs. 40\%). Postoperative motor impairment was mild and transient in a minority of cases (20\%). General complications, as defined by the Glioma Outcome Project, occurred in 23\% of cases. IN was the only factor associated with acute postoperative motor deterioration (P < 0.001). IN and age >65 years (P = 0.01) were associated with the occurrence of complications. Overall survival was significantly higher in patients operated with IN or fMRI plus navigator (P < 0.01). Comparing different surgical strategies used in the same period, we observed that supportive technologies in glioma surgery have their primary impact on the quality of resection and survival. IN led to transient motor impairment and some additional complications which did not affect functional outcome.

Purpose We performed a new phase II trial enrolling patients with newly diagnosed high-grade glioma (HGG) to test the efficacy of a weekly alternating temozolomide (TMZ) schedule after surgery and concomitant chemoradiotherapy. Methods From January 2005 to January 2007, 34 patients (21 men, 13 women; age range 30–70, mean age 53) were enrolled. There were 32 glioblastoma multiforme and two anaplastic astrocytoma. Each patient after surgery received standard concurrent chemoradiotherapy. After a 4-week break, patients were then to receive 12 cycles of 1-week-on/1-week-off TMZ, with 75 mg/m2 for the first cycle, 100 mg/m2 for the second, 125 mg/m2 for the third, and 150 mg/m2 from the fourth to the 12th. Hematological toxicity was monitored every week during concomitant chemoradiotherapy and then every 4 weeks. Results After 12 months from the end of radiotherapy, the overall survival (OS) rate was 59% (20/38), distributed as follows: 60% (18/30) for recursive partitioning analysis (RPA) class 4 patients and 33% (1/3) for RPA class 6 patients; the only RPA class 1 patient was alive and disease free at the time of writing. Median OS was 13 months [95% confidence interval (CI) 11.02–14.98 months]. Hematological toxicity was seen in six patients (18%): grade 1 neutropenia in four, grade 2 thrombocytopenia in one, and grade 4 thrombocytopenia plus grade 1 neutropenia in one. There was one case of opportunistic infection (Pneumocystis carinii pneumonitis). Conclusion The toxicity of the TMZ dose-dense regimen was very low. Results seem to be encouraging for RPA lower classes (patients with good prognostic factors).

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.

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.

O(6)-methylguanine DNA-methyltransferase (MGMT) promoter methylation status is a prognostic factor in newly diagnosed glioblastoma patients. However, it is not yet clear whether, and if so how, MGMT methylation status may change. Moreover, it is unknown whether the prognostic role of this epigenetic feature is retained during the disease course. A retrospective analysis was made using a database of 614 glioblastoma patients treated prospectively from January 2000 to August 2008. We evaluated only patients who met the following inclusion criteria: age > or = 18 years; performance status 0-2; histological diagnosis of glioblastoma at both first and second surgery for recurrence; postoperative treatment consisting of: (i) radiotherapy (RT) followed by adjuvant temozolomide (TMZ) until 2005 and (ii) TMZ concurrent with and adjuvant to RT after 2005; a time interval > or = 3 months between first and second surgery. MGMT status was evaluated at first and second surgery in all 44 patients (M:F 32:12, median age: 49 years, range: 27-67 years). In 38 patients (86.4%), MGMT promoter status was assessable at both first and second surgery. MGMT methylation status, changed in 14 patients (37%) of second surgery samples and more frequently in methylated than in unmethylated patients (61.5% vs 24%, P = .03). The median survival was significantly influenced only by MGMT methylation status determined at first surgery (P = .04). Significant changes in MGMT methylation status during the course of GBM occur more frequently in MGMT methylated than unmethylated cases. MGMT methylation status determined at first surgery appears to be of prognostic value; however, it is not predictive of outcome following second surgery.

Purpose To assess in a prospective trial the value of prognostic factors and the outcome of medulloblastoma in adults. Methods and materials Patients (≥18 years) with a histologic diagnosis of medulloblastoma were staged according to Chang et al. 's classification (low risk: T1, T2, T3a, M0, and no residual disease after surgery; high risk: T3b–T4, any M+ or postoperative presence of residual tumor). In low-risk patients, treatment consisted of 36 Gy to the craniospinal axis, supplemented by a local tumor dose of 18.8 Gy (total dose of 54.8 Gy). In high-risk patients, 2 cycles of “up-front chemotherapy” were delivered before the same radiation therapy, followed by maintenance chemotherapy if M1, M2, or M3 disease was present. Results Over a 12-year period, 36 evaluable patients were enrolled. Progression-free survival (PFS) at 5 years was higher in low-risk patients compared to the high-risk group: 76% ± 14% (95% confidence interval [CI] = 52%–100%) vs. 61% ± 11% (95% CI = 42%–87%). Patients with M− disease showed a significantly better outcome than M+ patients, with 75% showing PFS at 5 years vs. 45% ( p = 0.01). Conclusions The overall PFS observed is comparable to that obtained in pediatric series and suggests that a more effective therapy must be developed for high-risk patients.

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.

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.

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 followup 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 disable. 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.

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.

This is a review of 1,000 consecutive cases of severe head injury admitted to our Neurosurgical Department between January 1973 and August 1976, before the advent of CT scanning. All patients were comatose following head injury (GCS⩽8) and were treated homogeneously by the same neurosurgical team by a protocol that included immediate resuscitation on arrival, diagnosis of intracranial lesions by angiography, early surgery when needed, mechanical ventilation, steroids, and mannitol. Extracranial lesions, even if preponderant, were treated by various specialists in the Neurosurgical Department, which for all practical purposes operated as an Emergency Department. Admission criteria were very broad with no preadmission selection. The overall mortality for this series was 45%. A little less than half the patients made good recoveries or remained moderately disabled (47%); 6% were severely disabled, and 2% survived in a persistent vegetative state. More than two-thirds of the patients were brought to our Neurosurgical Department after a short stay at a general hospital; 72% were admitted within 6 hours of injury; 71% were traffic accident victims; and 34% had significant associated extracranial injuries. Carotid angiography was performed in 78% of the patients and indicated the presence of an intracranial haematoma requiring surgery in 36% of the whole series. Mortality was significantly higher in operated than in unoperated patients (56% versus 39%); those treated surgically, however, were older, in worse clinical condition, and showed a higher incidence of acute subdural haematomas associated with brain contusion. Carotid angiography proved very effective in revealing the presence of an expansive lesion but failed to reflect the severity of brain damage, since the group with “negative” angiograms showed a high mortality (52%). Patients with a lucid interval had a higher percentage of surgical lesions than those with immediate coma (58% versus 26%); but fully 42% of them did not require surgery, and 25% had negative angiograms. From the prognostic point of view the clinical data elicited after initial resuscitation were highly predictive of the outcome: some individual neurological signs, such as mydriasis, posturing and eye movements, were not inferior to the GCS score in that respect. Age also proved a strong predictor, since elderly patients are more likely to have severe subdural and parenchymal lesions and their clinical severity is accordingly greater. Our series amounts to a data bank of cases both contemporary to and in good agreement with that collected by Jennett and his associates in their 1977 multinational study; and it affords a useful reference in the assessment of epidemiological variations and alternative management in relation to outcome.

This is a study of the changes that have occurred in the field of severe head injury since the advent of CT scanning, comparing two homogeneous series of patients selected by clinical status (Glasgow Coma Scale less than or equal to 8), namely a series of 1,000 cases admitted to our Department between 1973 and 1976, already published in this Journal, and one of 385 cases cared for between 1979 and 1980, when CT scanning had become generally available. The two series of patients compare very closely in many respects, particularly in the incidence of surgical cases. In the more recent series the overall outcome was better both in surgical and in non-surgical cases. Among patients in the CT scan series the incidence of brain contusion associated with haematoma was greater than that of pure subdural haematomas. In non-surgical patients the CT scan, unlike cerebral angiography, afforded better identification of traumatic lesions and the grouping of patients into homogeneous categories correlating with a given outcome. On admission, cerebral angiography and CT scanning were equally effective in detecting lesions of surgical import; later in the course of the illness, however, CT scanning proved far more effective in detecting changes, with fully 15% of the patients being referred for surgery in the light of repeat CT scan findings as opposed to only 4% undergoing surgery on the indications of repeat angiography. Also, in the new series the mean interval from injury to surgery was shorter, with 64% of patients being operated on within 6 hours of the injury.(ABSTRACT TRUNCATED AT 250 WORDS)

Patients in the early stage of severe head injuries occasionally present with a clinical picture analogous to that in vascular or neoplastic pontine lesions and they can be identified by careful clinical examination and by repeated electroencephalography (E.E.G.). The commonest features in these cases were preservation of consciousness, constriction of the pupils, disturbances of reflex ocular motility, severe pyramidal deficits, decerebrate rigidity, myoclonic manifestations, irregular breathing, and cranial nerve palsies. An E.E.G. tracing similar to the normal pattern was a common finding. These observations suggest that pontine syndromes may be directly produced by blunt head injuries. These findings also give a warning of the possibility of mistaking a non-reacting, but conscious, patient for one who is deeply comatose.

✓ A comprehensive study of the motor patterns, usually grouped under the heading “decerebrate rigidity,” was carried out in a series of 800 patients with severe head injuries. The incidence of these manifestations was 39.6%, and when they were present chances of survival were reduced from 79.4% to 28.1%. Clinical and electromyographic investigations revealed heterogeneous and unstable motor manifestations that did not fit into the classical groups of experimental models of decerebrate rigidity. Combinations of extensor and flexor attitudes and/or responses were frequently found in the same patient, but could be separated into homogeneous groups. Each recognized postural pattern had its own distinct neurological signs and prognosis. Age did not significantly affect the outcome; however, intracranial expanding lesions (73.5%), impairment of the brain-stem oculomotor system (49.8%), and deep coma (88.9%) all contributed to an unfavorable course. Surgical treatment was effective when performed for intracranial hematomas and in patients with incomplete extensor rigidity. Good recovery was achieved in 16% of decerebrate patients, while 12.1% survived in prolonged coma or with severe disabilities. All clinical and neuropathological data suggest that extensor motor abnormalities in the acute phase of cerebral traumatic disease do not always conclusively indicate structural brain-stem damage. A critical analysis of so-called “decerebrate rigidity” (rejecting in some instances its Sherringtonian implications) may allow for a more accurate clinical assessment of the severity of head injury.

Doppel (PRND) is a paralogue of the mammalian prion (PRNP) gene. It is abundant in testis and, unlike PRNP, it is expressed at low levels in the adult central nervous system (CNS). Besides, doppel overexpression correlates with some prion-disease pathological features, such as ataxia and death of cerebellar neurons. Recently, ectopic expression of doppel was found in two different tumor types, specifically in glial and haematological cancers. In order to address clinical important issues, PRND mRNA expression was investigated in a panel of 111 astrocytoma tissue samples, histologically classified according to the World Health Organization (WHO) criteria (6 grade I pilocytic astrocytomas, 15 grade II low-grade astrocytomas, 26 grade III anaplastic astrocytomas and 64 grade IV glioblastoma multiforme). Real-time PRND gene expression profiling, after normalisation with GAPDH, revealed large differences between low (WHO I and II) and high grade (III and IV) of malignancy (P