Hemangioblastoma of the obex mimicking anorexia nervosa.
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Adolescent↗
Biomedical subjects
R Scienza
Publications and source records attributed to R Scienza.
A multidrug combination designed for reversing resistance to BCNU in glioblastoma multiforme.
BACKGROUND: Nitrosoureas constitute the main resource of chemotherapy for glioblastoma. However, because of chemoresistance, which is intrinsic or rapidly acquired after the first administration of chemotherapy, there have been few improvements in survival. Because O(6)-alkylguanine-DNA alkyltransferase (AGT) is the main target for increasing cell sensitivity to the nitrosoureas, we postulated that preexposure to other alkylating agents might increase the therapeutic index of the nitrosoureas by saturating all the copies of AGT present in the tumor cells. OBJECTIVE: To investigate the response rate, toxic effects, time from start of chemotherapy to progression of disease or exit from the study for any reason (TTP), and progression-free survival at 6 months (PFS-6) associated with a multidrug combination that could reverse resistance to carmustine (BCNU) through AGT depletion. METHODS: We conducted a phase 2 study of patients with glioblastoma at first relapse or progression after surgery and standard radiotherapy. Patients were treated with 100 mg/m(2) of procarbazine on days 1 to 5, 80 mg/m(2) of BCNU on days 3 to 5, and 1.4 mg/m(2) of vincristine on day 3 every 8 weeks. RESULTS: Fifty-eight patients were enrolled in the study, and all were assessable for response and toxic effects. Six patients (10.3%) had a complete response, 11 (19%) had a partial response, and 17 (29.3%) had stable disease. The median TTP was 4.8 months; 42.3% of patients had PFS-6, and 15.4% had PFS at 12 months. Response to chemotherapy was the only significant prognostic factor for TTP. Neutropenia was grade 3 in 8.6% of patients and grade 4 in 5.2% of patients, and thrombocytopenia was grade 3 in 17.2% of patients and grade 4 in 12% of patients; hepatic and pulmonary toxic effects were grade 3 in 5.2% and 8.6% of patients, respectively. CONCLUSION: This regimen proved active in chemotherapy-naive patients with recurrent glioblastoma even though toxic effects were substantial.
Adolescent↗
Temozolomide as a second-line systemic regimen in recurrent high-grade glioma: a phase II study.
BACKGROUND: To investigate the efficacy of temozolomide in relation to response rate, toxicity, time to progression. and median survival time, a phase II study was conducted in patients with recurrent high-grade glioma following surgery plus radiotherapy and first-line chemotherapy based on nitrosourea, procarbazine and vincristine. PATIENTS AND METHODS: Forty-one patients with high-grade glioma, at second recurrence or progression, of which twenty-two (54%) had glioblastoma multiforme, ten (24%) anaplastic astrocytoma, and nine (22%) anaplastic oligodendroglioma were administered temozolomide, 150 mg/m2/daily for five days every four weeks. RESULTS: Response was assessed in 40 patients. The overall response rate (complete + partial response) was 22.5% (95% confidence interval (CI): 9.5%-35%). The median time to progression for all 41 patients was 22.3 weeks; progression-free survival at 6 and 12 months was 48.5% and 34.7%, respectively. Median survival time was 37.1 weeks with 80.2% at 6 and 34.9% survival at 12 months. CONCLUSIONS: On multivariate analysis, response to previous treatment was significant (P = 0.03) for time to progression and Karnofsky performance score for overall survivall (P = 0.002). Temozolomide gave a moderate response rate with acceptable toxicity as second-line chemotherapy in patients with recurrent high-grade glioma.
Adult↗
Surgical problems in the treatment of aneurysms of the posterior circulation.
BACKGROUND: Despite the recent multidisciplinary achievements, surgery for posterior circulation aneurysms remains challenging. Sac dimensions and fundus projection are highly variable and independent factors, which can determine, the operative field being deep and restricted, in some case the failure of the procedure. Other factors the surgeon must cope with are the relationships between aneurysm and surrounding arteries, cranial nerves (oculomotor nerve mainly) and bone structures. Due to the esiguity of the operative field a complete dissection of the sac can be achieved frequently only after the clipping when, by the way, the clip position must be verified in order to avoid perforants closure. Aim of this paper is to perform a retrospective study regarding patients harbouring a posterior circulation aneurysm treated in a public health environment. METHODS: Our experience resides on 78 consecutive cases (whose 37 of the basilar artery and 37 with other localizations) successfully clipped by the same surgeon (senior author: RS); 4 additional aneurysms were only explored (whose 3 originated from the basilar artery). The clinical charts of the basilar apex cases were reviewed, the angiographic and intraoperative findings were correlated to the GOS (Glasgow Outcome Scale). RESULTS: The fundus projection in the basilar apex cases was the main risk factor accounting for a 50% mortality rate in the antero-posterior direction cases. CONCLUSIONS: Posterior circulation aneurysms are intimidating vascular malformations even nowadays; in the future improvements in the endovascular technique could provide an effective alternative to an open surgery approach.
Basilar Artery↗
Intracisternal schwannoma of the spinal accessory nerve presenting as a normal pressure hydrocephalus syndrome. Case report and review of the literature.
Schwannomas of the spinal accessory nerve are rare lesions. They can be characterized by their locations as either intrajugular or intracisternal schwannomas, most of them being intrajugular. Only five cases arising in the cisterna magna are reported in literature. Clinical symptoms generally consist of 11th cranial nerve palsy associated with cerebellar signs and myelopathy. An additional case, studied by MRI, is described. The patient showed a mild cerebellar syndrome and normal pressure hydrocephalus symptoms. He was operated on and the mass completely removed with good postoperative results. The role of MRI for diagnosis of lower cranial nerve schwannomas is stressed, even though does not detect the exact nerve of origin. Total removal of these lesions is recommended, since their benign nature and unavoidable recurrence in case of partial excision.
Accessory Nerve↗
Cerebral cavernous malformations (cavernomas) in the pediatric age-group.
Cavernomas are vascular malformations composed of a compact mass of sinusoidal-type vessels that are immediately contiguous with one another and have no intervening parenchyma. Cavernous malformations were previously held to be a rare pathology occurring predominantly in adults. New neuroradiological techniques such as computed tomography (CT) and magnetic resonance imaging (MRI) demonstrate, on the contrary, that these lesions are also more frequent during childhood than was previously thought. In our institution we observed 17 cases of cavernous malformations in patients aged between 18 months and 16 years, 16 of whom were diagnosed after 1982. In 4 of these cases there was a documented familial history; in 2 multiple malformations were present. The most common site was the subcortical frontal region, but in 1 case the malformation was located in the pineal region. The most frequent (in 12 cases) initial symptom was hemorrhage, with the characteristics of an intracerebral hematoma. In 4 cases the initial symptom consisted of epileptic fits and 2 of these patients subsequently suffered hemorrhage. In one case the symptoms observed were those of an expansile process. All our patients underwent cerebral angiography and only in 1 case did this show a vascular abnormality. CT, performed in 16 patients, gave positive results in all cases. MRI, performed in 12 patients, gave highly significant images in all cases. Radical surgical removal of the malformation was performed in 15 of the 17 patients, and the results can be considered excellent in the majority of cases. Cavernous malformations are, therefore, more frequent lesions than had previously been thought, especially in pediatric patients.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
The relevance of anatomic and hemodynamic factors to a classification of cerebral arteriovenous malformations.
Potential prognostic anatomic and hemodynamic factors were evaluated in 248 patients with cerebral arteriovenous malformations (AVMs), all treated by direct microsurgical removal. The size of each AVM was calculated by its volume, obtained by the multiplication of the three AVM diameters by 0.52. A surgical classification of AVM location (in 11 groups) is proposed. Types of feeders and of drainage were classified as superficial or deep; the extent of the drainage system was classified according to a four-degree scale. The mean flow velocity in the main AVM feeder, detected using transcranial Doppler ultrasonography, was used as an indirect measure of AVM shunt flow in a small number of patients (n = 29). AVM volume was a very important prognostic factor: the incidence of hyperemic complications and the morbidity and mortality rate were significantly higher when the volume of the lesion was greater than 20 cm3 (P less than 0.0001 for hyperemic complications; P less than 0.001 for permanent morbidity and mortality). The incidence of hyperemic complications and the morbidity rate were higher in AVMs in rolandic, inferior limbic, and insular locations than in AVMs in other locations. As for other anatomic factors: a) the presence of deep feeders significantly increased the incidence of hyperemic complications, as well as the morbidity and mortality rate; b) the presence of deep drainage significantly increased permanent morbidity only; c) the extension of the venous system was significantly related to the development of hyperemic complications, and to morbidity and mortality. Transcranial Doppler examination showed that mean flow velocities greater than 120 cm/s in the main feeder were associated with a significantly higher rate of postoperative hematomas and transient deficits. A classification of cerebral AVMs that takes into account AVM volume and location, the type of feeders, the extent of the drainage system, and the main feeder flow velocity is suggested.
Adolescent↗
Treatment of cerebral arteriovenous malformations with a combination of preoperative embolization and surgery.
Forty-nine patients with cerebral arteriovenous malformations (AVMs) were treated with preoperative embolization followed by resection using a microsurgical approach. In 27 patients, the AVM was located in an eloquent area; in 32 patients, the volume of the AVM was over 20 cm3. Preoperatively, flow-directed embolization was performed in 10 patients (28 procedures), selective embolization with threads was performed in 35 patients (46 procedures), and a combination of flow-directed and selective embolization was performed in 4 patients (12 procedures). The percentage of reduction of the AVM volume averaged 36% after embolization. Five minor complications (transient neurological deficits, in 2 cases associated with ischemic areas on the CT scan) were observed after embolization. The interval between the last embolization and surgery was as follows: within 10 days in 7 patients; between 11 and 20 days in 3 patients; between 21 and 30 days in 10 patients; between 31 and 60 days in 11 patients; and 2 months later in 18 patients. The efficacy of this combined treatment (embolization plus surgery) was evaluated by the incidence of hyperemic complications and the clinical outcome. Hyperemic complications occurred more frequently in patients with an AVM volume greater than 20 cm3. When compared with flow-directed embolization, selective embolization was linked with decreased bleeding during surgery; postoperatively, the incidence of cerebral edema was also lower. Clinical outcome was better after selective embolization, with no occurrence of major deficits and no mortality. When the percentage of reduction of the AVM volume after embolization was 40% or more, the incidence of intraoperative hyperemic complications was lower; moreover, new permanent deficits were never observed in patients with this volume reduction. A retrospective clinical comparison of two groups of patients with similar AVM volumes (greater than 20 cm3)--those given combined treatment (n = 32) versus those treated by direct surgery alone (n = 27)--showed that intraoperative bleeding appeared to decrease in patients treated by embolization; the incidence of postoperative hyperemic complications was not different in the two groups. New major deficits and deaths were less frequent in patients treated by embolization (P = 0.05 for the incidence of major deficits); postoperative epilepsy was also less common in these patients. In conclusion, combined treatment with selective preoperative embolization and direct surgery may help the neurosurgeon in the treatment of large, high-flow AVMs, reducing the risks connected with their surgical removal.
Adolescent↗
Craniocerebral epidermoids and dermoids. A review of 44 cases.
We review 40 epidermoids and 4 dermoids of the skull and brain treated surgically in our Department between 1976 and 1987. Fourteen were extradural and 30 intradural. The mean duration of symptoms was 3 years for extradural and 10 years for intradural tumours. Symptoms varied with tumour site, in some sites being helpful in differential diagnosis. Skull X-rays and CT were the key diagnostic investigations in extradural and CT in intradural lesions, the latter, with few exceptions, presenting a characteristic CT scan. In 7 cases MRI supplied important details on the tumour boundaries. All the diploic and orbital lesions were removed totally, with a good outcome. Twelve of the intradural lesions were removed totally, 9 subtotally and 9 partially, with a good outcome in 21 patients and a poor outcome in 4; 5 patients died. Outcome was unrelated to degree of removal.
Adolescent↗
[Cerebral cavernous malformations (cavernomas) in children].
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Adolescent↗
Italian cooperative study on giant intracranial aneurysms: 3. Modalities of treatment.
240 patients with giant aneurysms were treated in 10 Italian centres with various therapeutic modalities: out of them, 50 patients were conservatively treated (19 with a severe intracranial haemorrhage). Direct surgery was performed in 140 patients: 67% of patients with aneurysms between 2 and 2.5 cm (A 1 group) and 50% of patients with larger aneurysms (A 2 group). The aneurysm could be secured by clip in 102 cases (56% of A 1 and 31% of A 2 patients). In patients with subarachnoid haemorrhage, surgery was done within 3 days in 24 cases, between 4 and 14 days in 21 cases, and later in 52 cases. In patients operated on directly, brain swelling was observed in 39% of cases; controlled hypotension was employed in 56 cases, and temporary vessel occlusion (mainly of M 1 tract) in 33 cases; removal of intra-aneurysmal thrombi was done in 18 cases, and intraoperative aneurysmal rupture occurred in 39 cases. Carotid ligation was performed in 31 patients, and was associated with a by-pass in 17 cases. Balloon occlusion was performed in 23 cases, and was associated with a by-pass in 10 cases. As regards aneurysmal location, intracavernous aneurysms were treated mainly by balloon occlusion or carotid ligation, while carotid/ophthalmic, middle cerebral and anterior communicating aneurysms were treated prevalently by direct surgery. 60% of treated patients were submitted to postoperative angiography, and 54% to postoperative CT scan; total obliteration of the aneurysm was documented in 83% of patients submitted to postoperative angiography.
Adult↗
Italian cooperative study on giant intracranial aneurysms: 4. Results of treatment.
240 patients with giant aneurysms admitted to 10 Italian centres were evaluated in regard to results of treatment and postoperative complications. As a whole, a complete recovery was observed in 39% of cases, various degrees of disability in 38% of cases, and death in 23% of cases, considering also patients admitted in Glasgow Coma Scale (GCS) 3-6. By excluding patients in GCS 3-6, active treatment was linked with a recovery rate of 47% and a mortality rate of 15%; the worst outcome was observed for carotid bifurcation aneurysms, the best for intracavernous aneurysms. In patients with giant carotid/ophthalmic or supraclinoid aneurysms, the outcome was similar after early surgery and after indirect methods of treatment. Factors playing a negative influence on outcome were mainly early exclusion after hemorrhage, operative brain swelling and cisternal tamponade; induced hypotension was associated with a significant decrease in mortality. Postoperative complications were observed in 48% of cases, and were mainly caused by ischaemic disturbances not associated with vasospasm (17% of cases), followed by surgical trauma and cerebral oedema; in 32 patients the postoperative neurological deterioration was fully reversible. In patients submitted to carotid occlusion association with a by-pass did not decrease the rate of ischaemic complications. In patients submitted to active treatments (open surgery or indirect methods of exclusion) the causes of morbidity were mainly: -deficits due to mass lesion, surgical complications, and ischaemic disturbances without vasospasm; the causes of mortality were mainly surgical trauma or medical complications.
Adult↗
Head and neck tumours studied by MRI spin-echo technique.
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Cranial Nerve Neoplasms↗
[Surgical treatment of supratentorial arteriovenous malformations].
The authors present their experience in the surgical treatment of supratentorial arteriovenous malformations. A few morphological data on the structure of these lesions are described, owing to their surgical relevance. The principles influencing the operative decisions--such as the age of the patient, his clinical history, the site and size of the malformation--are discussed. The operative strategy adopted for the removal of these lesions are articulated in various points: the organization for a long-lasting procedure, the use of the operative microscope and bipolar coagulation, a wide surgical exposure, a particular care for dural feeders and a wide arachnoidal opening, the trial of hypotension, the early closure of deep feeders, the saving of draining veins for as long as possible, the dissection along the sulci and into the white matter, the saving of functional arteries and accessory veins, the techniques adopted against paraventricular bleeding and hemorrhage from venous sinusoids, the coagulation of deep vessels, a particular care for possible AV fistulae under the main drainage, the "backward" technique, the "rosary-like" coagulation, a particular care for a possible division of the malformation into partitions. Particular surgical problems can occur in large AVMs, with diffuse hyperemia and hemorrhages due to anomalous perfusion of the tissue adjacent to the AVM; in these cases, controlled hypotension appears useful. The problems faced in the surgical approach to AVMs located in specific areas--such as cortical AVMs with deep extension, cortico-basal and cortico-interhemispheric AVMs, callosal AVMs, AVMs of the insula and basal ganglia, intra- and para-ventricular AVMs, juxtapeduncular and juxtasplenial AVMs--are finally discussed.
Adult↗
Intracranial aneurysms and subarachnoid hemorrhage in children and adolescents.
Thirty-eight cases of symptomatic cerebral aneurysms or spontaneous subarachnoid hemorrhage in children and adolescents were observed from 1965 to 1984; 33 cases were treated from 1970 to date. This group represents 2.6% of the total number of patients with subarachnoid hemorrhage treated at our institute in the same period. The cause of subarachnoid hemorrhage was unknown in 7 cases; an intracranial aneurysm had ruptured in 29 cases, and was unruptured but symptomatic in 2 remaining cases. Three aneurysms were mycotic. The most frequent aneurysmal locations were the internal carotid bifurcation and the anterior communicating artery; peripheral branches of the middle cerebral artery were also a relatively common location. Four patients were 3 years of age or younger: each presented peculiar clinical features, and 3 of the 4 had middle cerebral artery aneurysms. The remaining 34 patients were all above 9 years of age. Two groups were identified: (a) in 14 patients between 10 and 15 years of age, the aneurysm was most commonly at the internal carotid bifurcation (37%), and an intracerebral hematoma was observed in 50% of these cases; (b) in 20 patients between 16 and 20 years of age, the most common aneurysmal location was the anterior communicating artery (35%), and intracerebral hematomas were rare (10% of cases). Among patients with aneurysms, 19 underwent surgical exclusion by clip, with 10% morbidity and 5% mortality; 5 patients in moribund conditions were not operated on; 5 patients were conservatively treated; in 2 patients the aneurysm had disappeared at a second angiography.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
Intracranial hematomas following aneurysmal rupture: experience with 309 cases.
Three hundred and nine consecutive cases of intracranial hematomas due to aneurysmal rupture--representing 34% of the total number of patients with aneurysms observed in a 12-year period--were evaluated; of these, 211 were submitted to computed tomography scan. Hematomas were present on admission in 71% of patients and occurred at rebleeding in 29%. Ruptured middle cerebral artery aneurysms caused an intracranial hematoma more frequently than aneurysms in other locations. Ventricular hematomas were frequently observed--especially at rebleeding--in cases with anterior communicating artery aneurysms. Basal ganglia hematomas were detected in eight cases with internal carotid bifurcation aneurysms and in three with middle cerebral artery aneurysms. Subdural hematomas were observed in 32 cases, mainly due to ruptured middle-cerebral-artery and internal-carotid-artery aneurysms. As for clinical evolution, a rapid deterioration was observed in 39% of cases and a chronic course in 46%; a subacute deterioration was far less frequent. Delayed deterioration from vasospasm was observed in 8% of cases, and appeared to be related to the amount of subarachnoid bleeding associated with the hematoma. One hundred and forty-two patients were submitted to surgical treatment (evacuation of hematoma together with exclusion of aneurysm); deep coma, poor medical condition, stabilized neurological disability, or combinations of these factors accounted for the high number of patients not operated upon. Regardless of treatment, 24% of patients showed good results and 58% died. Presence of a large hematoma, ventricular hemorrhage, and shift of the ventricles represented significant risk factors, associated with a poor prognosis. A comparison between two groups of patients admitted within 3 days of hemorrhage--47 operated on early, and 149 with delayed treatment--showed that better results were achieved by early operations, especially for cases in Hunt's grades IV and V.
Adolescent↗
Ocular calcifications in von Hippel-Lindau disease.
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Adult↗
Spontaneous disappearance of intracranial arterio-venous malformations.
4 cases of spontaneous disappearance of intracranial arteriovenous malformations are reported. All the malformations were in the rolandic-parietal area, and fed in most cases by the MCA. In one case, the malformation was associated with a proximal aneurysm. One malformation was large, one medium-sized, and two small. Venous drainage was towards the longitudinal sinus in all cases. Presenting symptoms consisted of epilepsy in 2 cases, and intracerebral haemorrhage in 2 cases. Disappearance of the AVM was documented by angiography from 1 to 15 years after diagnosis, and was preceded by a new haemorrhage in 2 cases. Acute or gradual thrombosis of the AVM is suggested as the most likely cause of the disappearance of an AVM. Spontaneous thrombosis of an AVM should be considered as an aspect of the pathological entity known as "thrombosed AVM". Guidelines for the management of patients exhibiting spontaneous disappearance of an AVM are briefly discussed.
Adolescent↗