Artikel
Anaplastic gangliogliomas - Report of seven cases
Anaplastische Gangliogliome: Bericht von sieben Fällen
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Autoren
Veröffentlicht: | 23. April 2004 |
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Gliederung
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Objective
Gangliogliomas (GG) are a rare glioma entity and are the most common cause of chronic focal seizures in childhood. These tumors are mostly low grade gliomas (WHO °I, or °II if atypical features are identified) with only a low tendency to recur. Anaplastic tumors (Who°III) can only be identified in a small subset of GG.
Methods
Morphological characteristics, localisation, clinical course and therapeutic strategies of 7 cases (3 females, 4 males, 31-76 years of age) with anaplastic GG(WHO°III) were retrospectively evaluated.
Results
The GG displayed different morphologies in MRI: One case showed contrast ring enhancement comparable to a glioblastoma multiforme. One case presented a quite unusual distribution of a number of Gd-DTPA enhancing lesions. The remaining 5 cases showed a rather homogenous distribution of Gd-DTPA. In contrast to the typical low grade GG which are predominantly localised in the temporal lobe, the tumors in this series showed no preferred localisation. After resection all patients received radiation therapy. In five cases the tumors recurred within 3-12 months (mean 7,6 months) after primary resection. In 4 out of the 5 cases the recurrent tumor tissue was adjacent to the resection cavity, suitable for re-operation. Two of these patients were re-operated once, one twice again for further recurrent tumors. Time to progression after re-operation varied from 4 to 24 (mean 12,4) months. Two of these patients received chemotherapy (PCV), with a time to progression of 10 months in one case and 24 months of ongoing progression free survival. Three of six cases died after 1, 2 and 11 years.
Conclusions
The clinical courses of the anaplastic GG presented in this series were comparable to those of other anaplastic glioma entities, but differed in morphological and clinical details. Recurrent anaplastic GG were mostly circumscribed and occurred adjacent to the resection cavity, suitable for one or several re- resections. The time to progression after resection of recurrent tumor suggests an aggressive treatment regimen.