Rare central nervous system tumors in adults:a population-based study of ependymomas, pilocytic astrocytomas, medulloblastomas, and intracranial germ cell tumors

BACKGROUND: Ependymomas, pilocytic astrocytomas, medulloblastomas, and intracranial germ cell tumors occur relative frequently in children, but are rare central nervous system (CNS) tumors in adults. In this population-based survey, we established incidence, treatment, and survival patterns for these tumors diagnosed in adult patients (≥18 years) over a 30-year period (1989-2018). METHODS: Data on 1384 ependymomas, 454 pilocytic astrocytomas, 205 medulloblastomas, and 112 intracranial germ cell tumors were obtained from the Netherlands Cancer Registry (NCR) on the basis of a histopathological diagnosis. For each tumor type, age-standardized incidence rates and estimated annual percentage change were calculated. Trends in incidence and main treatment modalities were reported per 5-year periods. Overall survival was calculated using the Kaplan-Meier method, and relative survival rates were estimated using the Pohar-Perme estimator. RESULTS: Incidence and survival rates remained generally stable for pilocytic astrocytomas, medulloblastomas, and germ cell tumors. Increasing incidence was observed for spinal ependymomas, mostly for myxopapillary ependymomas, and survival improved over time for grade II ependymomas (P < .01). Treatment patterns varied over time with shifting roles for surgery in ependymomas and for chemotherapy and radiation in medulloblastomas and germinomas. CONCLUSIONS: The study provides baseline information for highly needed national and international standard treatment protocols, and thus for further improving patient outcomes in these rare CNS tumors

Hemangioblastomas. Studies on the molecular genetic background.

Contains fulltext : 27452.pdf (publisher's version ) (Open Access)RU Radboud Universiteit Nijmegen, 20 juni 2006Promotor : Padberg, G.W.A.M. Co-promotores : Wesseling, P., Jeuken, J.W.M.167 p

Primary central nervous system T-cell lymphoma.

Item does not contain fulltextMost primary CNS lymphomas (PCNSL) are B-cell neoplasms; T-cell lymphomas are quite rare. The authors report two young patients with T-cell PCNSL who had a complete response to chemo- and radiotherapy but developed recurrent disease and died 11 and 13 months from diagnosis. The prognosis of T-cell PCNSL may be worse than that of comparable B-cell tumors

Cyclosporine neurotoxicity: a review.

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Pneumocystis jirovecii pneumonia prophylaxis during temozolomide treatment for high-grade gliomas

Item does not contain fulltextHigh-grade glioma patients receiving concomitant chemoradiotherapy with temozolomide 75mg/m(2) during six to seven weeks or dose-dense temozolomide regimens especially in combination with chronic use of corticosteroids have a high risk for developing Pneumocystis jirovecii pneumonia. In this review, we define risk groups and propose a guideline for prophylaxis using risk stratification

Mononeuropathy multiplex as the initial manifestation of neurofibromatosis type 2.

Item does not contain fulltextThe authors report a patient with neurofibromatosis type 2 (NF2) presenting with an axonal mononeuropathy multiplex. Sural nerve biopsy showed small scattered groups of Schwann cells transformed into irregular branching cells with abnormal cell-cell contacts. The authors hypothesize that defective Schwann cell function, due to inactivation of the NF2 gene product merlin, leads to changes in morphology, cell-cell contact, and growth, and finally to degeneration of axons

Extensive epidural cufflike growth of malignant pleural mesothelioma causing spinal cord compression.

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Survival of breast cancer patients with synchronous or metachronous central nervous system metastases

Item does not contain fulltextBACKGROUND: Central nervous system (CNS) metastases represent a devastating complication for advanced breast cancer patients. This observational study examines the influence of patient, tumour and treatment characteristics on overall survival after synchronous or metachronous CNS metastases. METHODS: Information on 992 breast cancer patients with CNS metastases (whose primary tumour was diagnosed between 2004 and 2010) was retrieved from the Netherlands Cancer Registry (NCR). Overall survival was calculated from the date of CNS metastatic diagnosis, and the impact of prognostic factors on survival was assessed using univariate and multivariate extended Cox-regression models. RESULTS: We identified 165 patients with synchronous and 827 patients with metachronous CNS metastases. The majority of patients (88%) presented with brain metastases only, 12% had leptomeningeal metastases. Overall median survival was 5.0months. Non-triple-negative breast cancer and systemic therapy were associated with improved survival in both groups. In patients with synchronous CNS metastases, surgery for the primary tumour and the metastases also improved survival. In patients with metachronous metastases, younger age (1year), and absence of extracranial metastases were associated with improved survival. Metastasectomy and radiation therapy did not provide benefit beyond the first six months. CONCLUSIONS: No difference in survival was established between synchronous and metachronous CNS metastases. Triple-negative disease is prognostically unfavourable in both groups, while those receiving treatment have a better outcome. Metastasectomy and radiotherapy improve survival within the first six months, and additional benefit may be derived from systemic therapy