Double-Blind Phase III Randomized Trial of the Antiprogestin Agent Mifepristone in the Treatment of Unresectable Meningioma: SWOG S9005.

Purpose: Progesterone receptors are expressed in approximately 70% of meningiomas. Mifepristone is an oral antiprogestational agent reported to have modest activity in a phase II study. This multicenter, prospective, randomized, placebo-controlled phase III trial conducted by SWOG was planned to define the role of mifepristone in the treatment of unresectable meningioma.

A phase II study of cisplatin preceded by a 12-h continuous infusion of concurrent hydroxyurea and cytosine arabinoside (Ara-C) for adult patients with malignant gliomas (Southwest Oncology Group S9149).

Inhibition of DNA excision repair can modulate resistance to cisplatin. Cytosine arabinoside (Ara-C) and hydroxyurea (HU), in combination, inhibit the excision-repair system and removal of platinum-DNA adducts. Marked cytotoxic synergy had been demonstrated in vitro at clinically achievable levels.

Subependymomas: an analysis of clinical and imaging features.

Objective: Subependymomas are slow-growing, benign tumors usually found incidentally in the fourth ventricle at autopsy. They are typically associated with the ventricular system and become apparent clinically only when symptoms of hydrocephalus or mass effect develop. We review clinical, histological, and contemporary radiographic presentations of 16 subependymomas, including 2 intraparenchymal tumors.

Comparison of clinical and neuropathologic diagnoses of Alzheimer's disease in 3 epidemiologic samples.

Background: Studies of dementia in populations avoid many of the selection biases in clinical samples but require special evaluation and diagnostic methods to obtain high participation rates. To address this issue, we developed a unique in-home dementia assessment. We assessed validity of these assessments using neuropathologic confirmation of the clinical diagnosis in 3 epidemiologic samples.

Juvenile pilocytic astrocytoma of the brainstem in children.

Object: In reports involving the operative treatment of brainstem tumors, multiple histological types are often grouped together. To determine prognosis after resection, histology-specific data may be helpful.

Methods: Twenty-eight patients with juvenile pilocytic astrocytoma (JPA) of the brainstem (six in the midbrain, four in the pons, and 18 in the medulla) were identified from the medical records.

Population-based study of medical comorbidity in early dementia and "cognitive impairment, no dementia (CIND)": association with functional and cognitive impairment: The Cache County Study.

Objective: Authors investigated medical comorbidity in persons with dementia and "Cognitive Impairment, No Dementia" (CIND).

Methods: The Cache County Study is an ongoing population-based study of the epidemiology of dementia, the risk factors for conversion from CIND to dementia, and the progression of dementia. As part of the study's first incidence wave, persons with dementia (N=149), CIND (N=225), or without cognitive impairment (N=321) were identified and studied.

Intraventricular tanycytic ependymoma: case report and review of the literature.

Objective And Importance: Tanycytic ependymomas are a rare ependymoma subtype showing a marked predilection for the spine, with only a few reports of supratentorial tumors. We present a case of a tanycytic ependymoma arising from the lateral and third ventricle.

Clinical Presentation: The patient was a 55-year-old woman who complained of intermittent, progressively worsening dysequilibrium for several months.

Sciatic nerve sarcoidosis: utility of magnetic resonance peripheral nerve imaging and treatment with radiation therapy.

Sarcoidosis may involve both the central and peripheral nervous system, although peripheral nerve manifestations are usually seen late in the disease. In this report, the authors describe a case of sarcoidosis in a 22-year-old woman who presented with a foot drop. Although results of conventional lumbar magnetic resonance (MR) imaging were normal, MR peripheral nerve imaging of the thigh showed a mass in the sciatic nerve indicating tumor.

The role of methionine in ethylmalonic encephalopathy with petechiae.

Background: Among patients with ethylmalonic aciduria, a subgroup with encephalopathy, petechial skin lesions, and often death in infancy is distinct from those with short-chain acyl-coenzyme A dehydrogenase deficiency or multiple acyl-coenzyme A dehydrogenase deficiency. The nature of the molecular defect in this subgroup is unknown, and the source of the ethylmalonic acid has been unclear.

Objective: To determine whether the administration of candidate amino acids increased the excretion of ethylmalonic acid.

Oligodendroglioma and juvenile pilocytic astrocytoma presenting as synchronous primary brain tumors. Case report with histological and molecular differentiation of the tumors and review of the literature.

Multiple metastatic brain tumors and multifocal primary brain tumors of a single histological type are well described in the literature. The concurrent presence of multiple primary brain tumors with different histological characteristics, however, is very rare. The authors describe the first known case in which an oligodendroglioma and a juvenile pilocytic astrocytoma (JPA) presented as synchronous primary brain tumors in the same patient.

Anaplastic oligodendrogliomas: prognostic factors for tumor recurrence and survival.

Objectives: Anaplastic oligodendrogliomas (AO) are uncommon primary brain tumors whose natural history, prognosis, and optimal management are not yet fully understood. However, they are associated with a better prognosis and response to multimodality therapy based on specific molecular changes. In this multicenter retrospective study, we analyzed the clinical characteristics of patients with AO to identify prognostic factors that influence time to progression (TTP) and survival.

Spontaneous hemorrhage associated with a pilomyxoid astrocytoma. Case report.

Pilomyxoid astrocytomas have been identified as a more aggressive variant of pilocytic astrocytomas that occur in infants and in young children. These tumors are characterized by a perivascular arrangement of pilocytic cells that has a monomorphous architecture with uniform, elongated bipolar cells loosely ranged within a prominent myxoid background. The authors present the case of a 24-year-old man with a pilomyxoid astrocytoma of the temporal lobe, who presented with a hemorrhage.

A novel central nervous system-enriched spinocerebellar ataxia type 7 gene product.

Context: Polyglutamine-mediated neurodegeneration in spinocerebellar ataxia type 7 (SCA7) involves specific central nervous system structures despite widespread expression of the mutant ataxin-7 protein.

Objective: To determine whether expression of multiple gene products could contribute to selective neurodegeneration in SCA7.

Results: We identified a novel SCA7 transcript and protein, both of which are enriched within the central nervous system.

Autopsy-proven, sporadic pick disease with onset at age 25 years.

Context: Pick disease is uncommon and accounts for less than 2% of adult-onset dementias. Reports of Pick disease in young adults have apparently increased in the last decade.

Objective: To document the presentation and course of a patient with tau-positive Pick disease presenting at an extremely young age.

Phase II trial of amonafide in central nervous system tumors: a Southwest Oncology Group study.

Amonafide 300 mg/M2 was administered intravenously on a daily x 5 schedule to 27 eligible patients with recurrent or progressive central nervous system tumors. There were no objective responses. The most common toxicities were gastrointestinal, hematologic and neurologic.