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Background and PurposeThe informal care demands of primary malignant brain tumor (PMBT) patients include unique issues associated with neurological and cognitive symptoms. Existing caregiver needs questionnaires do not include these disease-specific symptoms, which are particularly distressing. Therefore, we have developed the neuro-oncology Caregiver Needs Screen (CNS) and evaluated its psychometric properties.MethodsThe 32-item instrument was developed based on PMBT caregiver interviews (N= 109) and expert review. The CNS was tested along measures of depression, anxiety, burden, and mastery in 122 PMBT caregivers. Principal components analysis was used to examine item properties and internal structure. Internal consistency reliability and construct validity were assessed.ResultsSix subscales were identified with internal consistency ranging between alpha = .653 and .857. Convergent validity was verified by moderate/high correlations between measures of caregiver well-being and CNS scale scores.ConclusionsFindings provide preliminary evidence of reliability and validity for the CNS. This instrument can be useful when assessing caregivers' needs for supportive care.

The activity of single-agent targeted molecular therapies in glioblastoma has been limited to date. The North American Brain Tumor Consortium examined the safety, pharmacokinetics, and efficacy of combination therapy with sorafenib, a small molecule inhibitor of Raf, vascular endothelial growth factor receptor 2, and platelet-derived growth factor receptor-β, and temsirolimus (CCI-779), an inhibitor of mammalian target of rapamycin. This was a phase I/II study. The phase I component used a standard 3 × 3 dose escalation scheme to determine the safety and tolerability of this combination therapy. The phase II component used a 2-stage design; the primary endpoint was 6-month progression-free survival (PFS6) rate. Thirteen patients enrolled in the phase I component. The maximum tolerated dosage (MTD) for combination therapy was sorafenib 800 mg daily and temsirolimus 25 mg once weekly. At the MTD, grade 3 thrombocytopenia was the dose-limiting toxicity. Eighteen patients were treated in the phase II component. At interim analysis, the study was terminated and did not proceed to the second stage. No patients remained progression free at 6 months. Median PFS was 8 weeks. The toxicity of this combination therapy resulted in a maximum tolerated dose of temsirolimus that was only one-tenth of the single-agent dose. Minimal activity in recurrent glioblastoma multiforme was seen at the MTD of the 2 combined agents.

(2000). Advances in Neurofibromatosis 2 (NF2): A Workshop Report. Journal of Neurogenetics: Vol. 14, No. 2, pp. 63-106.

Estimating survival time in cancer patients is crucial for clinicians, patients, families, and payers. To provide appropriate and cost-effective care, various data sources are used to provide rational, reliable, and reproducible estimates. The accuracy of such estimates is unknown.The authors prospectively estimated survival in 150 consecutive cancer patients (median age 62 years) with brain metastases undergoing radiosurgery. They recorded cancer type, number of brain metastases, neurological presentation, extracranial disease status, Karnofsky Performance Scale score, Recursive Partitioning Analysis class, prior whole-brain radiotherapy, and synchronous or metachronous presentation. Finally, the authors asked 18 medical, radiation, or surgical oncologists to predict survival from the time of treatment.The actual median patient survival was 10.3 months (95% CI 6.4-14). The median physician-predicted survival was 9.7 months (neurosurgeons = 11.8 months, radiation oncologists = 11.0 months, and medical oncologist = 7.2 months). For patients who died before 10 months, both neurosurgeons and radiation oncologists generally predicted survivals that were more optimistic and medical oncologists that were less so, although no group could accurately predict survivors alive at 14 months. All physicians had individual patient survival predictions that were incorrect by as much as 12-18 months, and 14 of 18 physicians had individual predictions that were in error by more than 18 months. Of the 2700 predictions, 1226 (45%) were off by more than 6 months and 488 (18%) were off by more than 12 months.Although crucial, predicting the survival of cancer patients is difficult. In this study all physicians were unable to accurately predict longer-term survivors. Despite valuable clinical data and predictive scoring techniques, brain and systemic management often led to patient survivals well beyond estimated survivals.

In this study the authors evaluated the role of stereotactic radiosurgery (SRS) in the management of progressive or newly diagnosed small-volume oligodendrogliomas. Tumor control, survival, and complications were assessed in patients with oligodendroglioma who underwent Gamma Knife radiosurgery as a primary or adjuvant procedure.The authors retrospectively reviewed 30 patients with oligodendroglioma (12 Grade II and 18 Grade III) who underwent SRS between 1992 and June 2006 at the University of Pittsburgh. The median patient age was 43.2 years (range 10.8-75.4 years). Twenty-four patients had previously undergone resection of the tumor, whereas tumors in 6 were diagnosed based on biopsy findings. The SRS was performed in 25 patients who had imaging-defined tumor progression despite prior fractionated radiation (22 patients) and/or chemotherapy (20 patients). The median target volume was 15.4 cm(3) (range 0.07-48.7 cm(3)) and the median margin dose was 14.5 Gy (range 11-20 Gy).At an average of 39.2 months of follow-up (range 12-133 months), 17 patients were dead and 13 were living. The overall survival rates from diagnosis to 5 and 10 years were 90.9 and 68.2%, respectively, for Grade II and 52.1% at 5 years and 26.1% at 10 years for Grade III. Factors associated with an improved progression-free survival included lower tumor grade and smaller tumor volume. In 13 patients who had loss of heterozygosity testing, patients with 1p19q loss of heterozygosity had a significantly improved survival after diagnosis (p = 0.04).The SRS modality is a minimally invasive additional option for patients with residual or recurrent oligodendrogliomas. It may also be considered as an alternative to initial resection in small-volume tumors located in the cortical brain region.

PurposeFenretinide induces apoptosis in malignant gliomas in vitro. This two-stage phase II trial was conducted to determine the efficacy of fenretinide in adults with recurrent malignant gliomas.Patients and MethodsTwenty-two patients with anaplastic gliomas (AG) and 23 patients with glioblastoma (GBM) whose tumors had recurred after radiotherapy and no more than two chemotherapy regimens were enrolled. Fenretinide was given orally on days 1 to 7 and 22 to 28 in 6-week cycles in doses of 600 or 900 mg/m2bid.ResultsSix of 21 (29%) patients in the AG arm and two of 23 (9%) patients in the GBM arm had stable disease at 6 months. One patient with AG treated at 900 mg/m2bid dosage had a partial radiologic response. Median progression-free survival (PFS) was 6 weeks for the AG arm and 6 weeks for the GBM arm. PFS at 6 months was 10% for the AG arm and 0% for the GBM arm. Grade 1 or 2 fatigue, dryness of skin, anemia, and hypoalbuminemia were the most frequent toxicities reported. The trial was closed after the first stage because of the inadequate activity at the fenretinide doses used. The first-administration mean plasma Cmaxfor fenretinide was 832 ± 360 ng/mL at the 600 mg/m2bid dosage and 1,213 ± 261 ng/mL at the 900 mg/m2bid dosage.ConclusionFenretinide was inactive against recurrent malignant gliomas at the dosage used in this trial. However, additional studies using higher doses of the agent are warranted based on the tolerability of the agent and the potential for activity of a higher fenretinide dosage, as suggested in this trial.

Headaches in a cancer hospital are common and often require extensive diagnostic evaluation to exclude metastatic intracranial disease or infection. However, one does not expect to encounter headache in an individual with no other neurologic illness who donates bone marrow. We have seen two patients develop positional headaches, which were typical of low pressure headaches occurring after lumbar puncture, after bone marrow harvest. In both patients, the headaches resolved without specific therapy. ### Patient 1. A 44-year-old woman underwent elective bone marrow harvest to provide marrow for an HLA compatible sibling. She was morbidly obese. For several years, she suffered diffuse headache on awakening that would abate as she stood up and began her daily activities. The physician performing the bone marrow harvest noted the patient's obesity obscured the usual landmarks. When the surgical drapes were removed, several puncture marks were noted over the lateral sacrum. When the patient awoke from general anesthesia, she complained of severe …

We report the cases of two adult patients with cystic fibrosis affecting the pancreas and liver, who also had severe vitamin E deficiency and neurologic disease. The most prominent clinical features were abnormal eye movements, diminished reflexes, decreased vibratory and position sense, ataxia, and muscle weakness. Treatment with intramuscular injections of vitamin E partially corrected the neurologic deficits. Vitamin E absorption tests documented severe malabsorption, which was later alleviated by the addition of dessicated ox bile to the regimen of alpha-tocopheryl acetate. These studies suggest that a decreased intraluminal concentration of bile salts is an important factor in the development of severe vitamin E deficiency and in the poor response to oral replacement therapy that is seen in some patients with cystic fibrosis.