Pattern of care and effectiveness of treatment for glioblastoma patients in the real world: Results from a prospective population-based registry. Could survival differ in a high-volume center?

BACKGROUND: As yet, no population-based prospective studies have been conducted to investigate the incidence and clinical outcome of glioblastoma (GBM) or the diffusion and impact of the current standard therapeutic approach in newly diagnosed patients younger than aged 70 years. METHODS: Data on all new cases of primary brain tumors observed from January 1, 2009, to December 31, 2010, in adults residing within the Emilia-Romagna region were recorded in a prospective registry in the Project of Emilia Romagna on Neuro-Oncology (PERNO). Based on the data from this registry, a prospective evaluation was made of the treatment efficacy and outcome in GBM patients. RESULTS: Two hundred sixty-seven GBM patients (median age, 64 y; range, 29-84 y) were enrolled. The median overall survival (OS) was 10.7 months (95% CI, 9.2-12.4). The 139 patients ≤aged 70 years who were given standard temozolomide treatment concomitant with and adjuvant to radiotherapy had a median OS of 16.4 months (95% CI, 14.0-18.5). With multivariate analysis, OS correlated significantly with KPS (HR = 0.458; 95% CI, 0.248-0.847; P = .0127), MGMT methylation status (HR = 0.612; 95% CI, 0.388-0.966; P = .0350), and treatment received in a high versus low-volume center (HR = 0.56; 95% CI, 0.328-0.986; P = .0446). CONCLUSIONS: The median OS following standard temozolomide treatment concurrent with and adjuvant to radiotherapy given to (72.8% of) patients aged ≤70 years is consistent with findings reported from randomized phase III trials. The volume and expertise of the treatment center should be further investigated as a prognostic factor.

Evaluation of acute perihematomal regional apparent diffusion coefficient abnormalities by diffusion-weighted imaging.

In this study, we investigated 40 patients (18 male, 22 female; mean age = 64.5 +/- 11.0; GCS = 9 to 14) with acute supratentorial spontaneous intracerebral hemorrhage (SICH) at admission by using a 1-tesla magnetic resonance imaging (MRI) unit equipped for single-shot echo-planar spin-echo isotropic diffusion-weighted imaging (DWI) sequences. All DWI studies were obtained within 48 hours after symptom onset. Regional apparent diffusion coefficient (rADC) values were measured in 3 different regions of interest (ROIs) drawn freehand on the T2-weighted images at b 0 s/mm2 on every section in which hematoma was visible: 1) the perihematomal hyperintense area; 2) 1 cm of normal appearing brain tissue surrounding the perilesional hyperintense rim; 3) an area mirroring the region including the clot and perihematomal hyperintense area placed in the contralateral hemisphere. rADC mean values were higher in perihematomal hyperintense and in contralateral than in normal appearing areas (p < 0.001), with increased rADC mean levels in all regions examined. Our findings show that rADC values indicative of vasogenic edema were present in the perihematomal area and in normal appearing brain tissue located both ipsilateral and contralateral to the hematoma, with lower levels in non-injured areas located in the T2 hyperintense rim around the clot.

Assessment of acute spontaneous intracerebral hematoma by CT perfusion imaging.

A single-section deconvolution-derived computerized tomographic perfusion imaging was performed in 45 patients (22 male and 23 female; mean age=69.89+/-10.07 years) with acute supratentorial spontaneous intracerebral hemorrhage. Mean rCBF and rCBV were lower in the hemorrhagic core than in the perihematomal low density area (p<0.001), and in the perihematomal low density area than in normal appearing brain parenchyma (p<0.001). Mean rMTT values were higher in perihematomal low density area than in normal appearing area (p<0.01) and in both hemorrhagic and perihematomal area than in controlateral ROI (p<0.001). There were no differences in rMTT mean values between hemorrhagic core and perihematomal area, as well as between normal appearing and controlateral areas. We found a concentric distribution of all CT perfusion parameters characterized by an improvement from the core to the periphery, with low perihematomal rCBF and rCBV values suggesting edema formation.

Evaluation with MR, perfusion MR and cerebral flowSPECT in NPSLE patients.

PURPOSE: To compare the performance of MR, cerebral flow SPECT and perfusion MRI (PWI) in NPSLE patients by using image co-registration. MATERIALS AND METHODS: 20 SLE patients underwent MR (T2-weighted FLAIR), perfusion and diffusion MR, and SPECT (after ( 99m)Tc-HMPAO intravenous injection). Two experienced operators analysed the images both independently and jointly after multi-modal volumetric co-registration ("Statistical Parametric Mapping" software, based on the Maximization of mutual information method). RESULTS: The FLAIR examination depicted 82 small lesions in 11/20 patients. Perfusion SPECT showed 43 hypoperfused areas in 17/20 patients. PWI showed 13 hypoperfused areas in 10/20 patients. After co-registration of images, anatomical agreement between SPECT and PWI was found in 10 hypoperfused areas (8 patients). Co-registration with FLAIR showed some false positives, more frequent in SPECT (9/43 areas) than in PWI (2/13 areas). DISCUSSION AND CONCLUSIONS: The FLAIR examination confirmed its high sensitivity in detecting lesions. Perfusion SPECT confirmed high sensitivity for the detection of hypoperfused cerebral areas. PWI showed fewer areas of cerebral hypoperfusion than did SPECT. The disagreement between SPECT and PWI may be related to the different modalities for disease detection. The anatomical agreement of hypoperfused areas between SPECT and PWI, assessed after co-registration, can suggest the prognostic hypothesis of delayed appearance of permanent parenchymal lesions. Co-registration modality, being able to show some false positives, seems to be a valid support for the interpretation of SPECT and PWI findings; the joint analysis of SPECT and PWI highlighted the capability of PWI for the interpretation of uncertain cases.

SPET imaging of intracranial tumours with 99Tcm-sestamibi.

Single photon emission tomography (SPET) employing 99Tcm-sestamibi (MIBI) injected intravenously was performed in 27 patients for pre-surgical evaluation of intraparenchymal brain tumours. A computerized tomography (CT) scan was performed in 26 patients, magnetic resonance imaging (MRI) in 8 patients and digital subtraction angiography (DSA) in 14 patients. Visual analysis of the SPET scans was performed using a 4-point scale relating to background activity, to evaluate MIBI uptake in the tumour. The vascular supply and the cellular component were also evaluated using DSA and CT scans. In normal controls, MIBI uptake was observed in the scalp, in the choroid plexus and in the pituitary gland, but never in normal parenchyma. Among the astrocytoma group of patients, a trend between MIBI uptake and grade of tumour was noted. MIBI uptake in meningiomas depends primarily on the vascular supply. Our results support the hypothesis that vascular supply, integrity of the blood-brain barrier, the degree of malignancy of the neoplasm and the viability of the tumour cells may be related to MIBI uptake.

Brain single-photon emission tomography with 99mTc-HMPAO in neuropsychiatric systemic lupus erythematosus: relations with EEG and MRI findings and clinical manifestations.

Central nervous system (CNS) involvement in patients with systemic lupus erythematosus (SLE) is often difficult to evaluate because of protean neuropsychiatric (NP) manifestations and lack of reliable diagnostic markers. In the reported study the role of single-photon emission tomography (SPET) with technetium-99m hexamethylpropylene amine oxime (HMPAO) in the evaluation of CNS involvement in SLE was assessed and the relations between SPET perfusion defects, EEG examination, magnetic resonance imaging (MRI) findings and clinical presentation were examined. Twenty SLE patients with different NP manifestations were studied. Multiple areas of hypoperfusion, especially in the territory of the middle cerebral artery, were demonstrated by SPET analysis in all 20 patients. The number of hypoperfused areas and the degree of hypoperfusion, expressed by an asymmetry index (AI), were more marked in patients with multiple NP manifestations. MRI and EEG evaluations were positive for 14 of 18 and for 12 of 20 patients, respectively. In the patients with positive SPET and MRI, 87 MRI focal lesions and 63 hypoperfused areas were found, and for 51 of these 63 at least one MRI lesion was found in the same anatomical region. SPET examination of patients with a normal EEG showed fewer hypoperfused areas and a lower degree of asymmetry compared to patients with an abnormal EEG. SPET of patients with focal EEG abnormalities showed more hypoperfused areas (difference not statistically significant) and a higher AI than did SPET of the patients with diffuse EEG abnormalities. Seven of 11 anatomical regions with focal EEG abnormalities.(ABSTRACT TRUNCATED AT 250 WORDS)

Intracranial gliomas in Ferrara, Italy, 1976 to 1991.

INTRODUCTION: We planned a descriptive study on the incidence of intracranial gliomas spanning a 16-year period (1976-1991) in the Local Health Service 31 of Ferrara, Northern Italy. MATERIAL AND METHODS: We used a complete enumeration approach by reviewing all the possible sources of case collection available in the study area. RESULTS: The mean annual incidence rate was 5.8 new cases per 100,000 population (6.96 for men and 4.78 for women; p < 0.05), resulting in 4.7 per 100,000 when directly adjusted to the Italian population. The age-specific incidences showed a small peak in childhood, an increase with age, reaching a maximum in the age group 60 to 64 and then a decline in the elderly. This pattern is similar for both sexes. The adjusted rates increased from 3.94 per 100,000 population in the first five-year period to 5.6 per 100,000 in the third (a nonsignificant difference). The distribution of cases within the study area was substantially uniform. CONCLUSION: The incidence rates of Ferrara fell into the middle-high values so far reported and confirmed the male preponderance found in previous studies. The age-related pattern is similar to that observed, with few exceptions, in other surveys. Like other authors we did not find a significant temporal trend, although the incidence rates tended to increase with time. The data encourage further, wider epidemiological studies of a prospective nature.

Aneurysm of the azygos pericallosal artery. One case.

The Authors describe a case of aneurysmal rupture of the azygos pericallosal artery, a variant of the anterior cerebral artery. The association between aneurysm and this anatomical anomaly is of interest because of its rarity and clinical-therapeutic implications.

[The empty sella syndrome. Clinical, radiological and endocrinologic analysis in 20 cases]. / La sindrome della sella vuota. Analisi clinica, radiologica ed endocrinologica in 20 casi.

Empty sella syndrome is an anatomical entity in which the pituitary fossa is enlarged and partially filled with cerebrospinal fluid owing to the arachnoid herniation, while the pituitary gland is compressed against the posterior rim of the fossa. This condition can be due to an inherent weakness of the diaphragm sella and/or to an increase in intracranial pressure which promote the herniation of the arachnoid membrane into the pituitary fossa (primary empty sella) or it can results following surgery, radiation or vascular and tumorous pituitary diseases (secondary empty sella). Empty sella can be associated with neuroradiological and endocrine symptoms. This study reports the clinical, endocrine, and roentgenographic features in 20 patients with primary empty sella syndrome. Disturbances of hypothalamic-pituitary function were detected in 6 patients (hyperprolactinemia, hypopituitarism, central diabetes insipidus, hypothalamic hypothyroidism). Three patients exhibited hypergonadotropic hypogonadism. This report supports the following conclusions: a) there is no correlation between size of pituitary fossa, type an extension of arachnoid herniation and the degree of hypothalamic-pituitary dysfunction; b) endocrine alterations are frequent in the empty sella syndrome; c) the association of empty sella and primary diabetes insipidus is not a very rare event.

[The skull in renal osteodystrophy]. / Il cranio nell'osteodistrofia renale.

Skull X-rays of 60 patients with chronic renal failure were examined. Alterations included diminished or increased bone density, radiolucent areas, pepper pot skull and the disappearance of vascular grooves and sutures. It is suggested that the radiological aspect of the skull is of very little diagnostic use in the assessment of uremic osteopathy since specific alterations are rare and tardive and show no correlation with clinical and laboratory findings. Skull X-rays can be useful in assessing the effects of treatment (vitamin D derivatives, parathyroidectomy) and for the identification of focal lesions (brown tumors).

[Behavior and significance of phalangeal bone changes in uremic patients under periodic hemodialysis treatment]. / Comportamento e significato delle alterazioni scheletriche falangee nell'uremico in trattamento emodialitico periodico.

Bone damage in hand phalanges has been evaluated with reference to age and duration of hemodialysis (on the basis of 248 radiological observations), in 93 cases with chronic renal failure (age: 20-59 years). These patients were on regular dialytic treatment (RDT) from 1 to 138 months. 72% of the patients underwent several periodic (annual) controls using the mammographic technique. The radiologic evaluations have been arranged into groups according to age. Skeletal damage was more evident when RDT was prolonged. Bone damage increases with age in the first 48 months; afterwards, on the contrary, bone changes were more evident in middle aged patients. At the beginning of RDT, acroosteolysis was the most important change always present. Both subperiosteal and intracortical resorption are more evident increasing age and duration of RDT. Radiological changes give a clear picture of the possible histo-morphologic pattern that characterizes uremic osteodystrophy.

Early skeletal changes of the hand phalanges in patients with chronic renal failure.

Appearance time and the severity of skeletal changes in patients with chronic renal failure are still not well defined. In 61 patients with incipient to advanced renal insufficiency (glomerular filtration rate 70 to 5 ml/min X 1.73 m2), bone alterations (acro-osteolysis, subeperiosteal and intracortical resorption) were mammographically studied in hand phalanges. A high correlation (p less than 0.001) was found between the radiological score and the severity of the renal insufficiency. The earliest changes of renal osteodystrophy occurred in the tufts. Biopsy specimens were taken from the iliac crest in 22 patients. Qualitative bone histology correlated significantly (p less than 0.001) with the total radiological score. Mammography offers an effective and non-invasive means of studying early skeletal changes in patients with chronic renal failure.

Xeroradiography in beta-thalassaemia.

Xeroradiographic investigations of the skull, hand, and elbow were performed on 27 patients with homozygous beta-thalassaemia. The results were compared with plain radiographic examinations. Xeroradiography, because of its technical properties (i.e. edge contrast enhancement and wide latitude), was shown to demonstrate cortical thinning of long bones, swelling of the diploic space in the skull, and reticulated patterns in the elbow better than standard radiography. Moreover, the use of "positive" mode imaging was shown to have advantages in the study of the skull and extremities.

Periosteal new bone in uraemic osteodystrophy.

Out of 54 patients requiring haemodialysis approximately one quarter were found to have periosteal new bone associated with severe renal osteodystrophy. This feature has been analysed and correlated with biochemical, clinical, and histological parameters. No direct correlation was found with the duration of haemodialysis, but there is a good correlation with histological indices of osteitis fibrosa and serum parathormone levels. Periosteal new bone regressed or totally disappeared in eight patients after parathyroidectomy and renal transplantation. It is suggested that periosteal new bone may be a useful indicator of hyperparathyroidism in this type of patient.