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67. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC)
Joint Meeting mit der Koreanischen Gesellschaft für Neurochirurgie (KNS)

Deutsche Gesellschaft für Neurochirurgie (DGNC) e. V.

12. - 15. Juni 2016, Frankfurt am Main

Treatment options for adult patients with anaplastic pilocytic astrocytomas WHO°III

Meeting Abstract

  • Almuth F. Kessler - Universitätsklinik Würzburg, Neurochirurgische Klinik und Poliklinik, Tumorbiologisches Labor, Würzburg, Germany
  • Jonas Feldheim - Universitätsklinik Würzburg, Neurochirurgische Klinik und Poliklinik, Tumorbiologisches Labor, Würzburg, Germany
  • Thomas Linsenmann - Universitätsklinik Würzburg, Neurochirurgische Klinik und Poliklinik, Tumorbiologisches Labor, Würzburg, Germany
  • Carsten Hagemann - Universitätsklinik Würzburg, Neurochirurgische Klinik und Poliklinik, Tumorbiologisches Labor, Würzburg, Germany
  • Ralf-Ingo Ernestus - Universitätsklinik Würzburg, Neurochirurgische Klinik und Poliklinik, Tumorbiologisches Labor, Würzburg, Germany
  • Mario Löhr - Universitätsklinik Würzburg, Neurochirurgische Klinik und Poliklinik, Tumorbiologisches Labor, Würzburg, Germany

Deutsche Gesellschaft für Neurochirurgie. 67. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC), 1. Joint Meeting mit der Koreanischen Gesellschaft für Neurochirurgie (KNS). Frankfurt am Main, 12.-15.06.2016. Düsseldorf: German Medical Science GMS Publishing House; 2016. DocP 119

doi: 10.3205/16dgnc494, urn:nbn:de:0183-16dgnc4941

Veröffentlicht: 8. Juni 2016

© 2016 Kessler et al.
Dieser Artikel ist ein Open-Access-Artikel und steht unter den Lizenzbedingungen der Creative Commons Attribution 4.0 License (Namensnennung). Lizenz-Angaben siehe http://creativecommons.org/licenses/by/4.0/.


Gliederung

Text

Objective: Due to its rareness, there is uncertainty about the clinical course and adjuvant therapy-options of anaplastic pilocytic astrocytoma WHO°III (APA). Whereas in pilocytic astrocytoma WHO °I (PA) the prognosis remains comparatively well even in case of leptomeningeal spreading (LMS) with a median survival of 65 months, it seems to be dismal in APA. As KIAA1549-BRAF fusion gene-based activated kinase is characteristic for pediatric PA, BRAF-targeted Vemurafenib might be the next treatment-generation. Little is known about BRAF-mutations in adult APA as well as about the impact of further prognostically significant alterations like MGMT-promoter methylation (MGMT-PM) and IDH-mutations.

Method: We performed a retrospective evaluation of adult APA-patients treated at our institution between 1995 – 2012 concerning their clinical courses and treatment regimes. BRAFV600E and fusion of KIAA1549-BRAF genes were analyzed by PCR-sequencing. High resolution melting analysis was used for determining MGMT-PM, and immunohistochemistry for IDH1(R132H) mutation.

Results: Five APA-patients aged 24 - 63 years at first diagnosis could be identified. The tumors were located in the posterior fossa in three patients, in the thalamus in one patient and presented as LMS as first APA-diagnosis in one patient. Median survival after APA-diagnosis was eight months (range two to eight months) with 2 patients still being alive. In three patients, APA evolved from a cerebellar PA, in one case 25 years after prior irradiation. In all patients, treatment consisted of gross or subtotal resection, followed by irradiation and adjuvant alkylating chemotherapy in four of them. Two patients developed a local recurrence that could be controlled by temozolomide so far. However, the occurrence of LMS in APA proved to be a poor prognostic sign, since the three affected patients died within four months irrespective of treatment. Concerning the molecular markers, neither IDH1(R132H) mutation nor BRAF-alterations could be detected, whereas an MGMT-PM was positive (25%) in one patient.

Conclusions: Since APA can evolve from a benign precursor lesion, former pediatric patients having been treated for PA should be monitored by regular MRI-F/U even in adulthood. More than half of the patients in our small series developed LMS associated with a limited prognosis; therefore we recommend treating APA with a multimodal aggressive protocol. However, according to the lack of BRAF-alterations, Vemurafenib cannot be considered as an option.