Artikel
Intramedullary solitary fibrous tumor – a benign form of hemangiopericytoma? Case report and review of the literature
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Autoren
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Markus Bruder
- Klinik für Neurochirurgie, Johann Wolfgang Goethe Universität, Frankfurt am Main
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Dominique Tews
- Institut für Neuropathologie, Johann Wolfgang Goethe Universität, Frankfurt am Main
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Michel Mittelbronn
- Institut für Neuropathologie, Johann Wolfgang Goethe Universität, Frankfurt am Main
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David Capper
- Institut für Neuropathologie, Universitätsklinikum Heidelberg
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Volker Seifert
- Klinik für Neurochirurgie, Johann Wolfgang Goethe Universität, Frankfurt am Main
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Gerhard Marquardt
- Klinik für Neurochirurgie, Johann Wolfgang Goethe Universität, Frankfurt am Main
| Veröffentlicht: | 2. Juni 2015 |
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Gliederung
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Objective: Solitary fibrous tumors (SFT) are mesenchymal tumors first described as a primary neoplasm arising from the pleura. The occurrence within the spinal cord or partial intramedullary location is very rare and has been reported in 16 cases so far to the best of our knowledge. SFT of the central nervous system are principally benign tumors, nevertheless the histological findings of SFT show many similarities to the more malignant hemangiopericytoma (HPC). Therefore in the last decade more and more pathologists assume a common spectrum of these tumors. We present a case of an intramedullary tumor with typical histopathological and immunohistochemical findings for SFT. Nevertheless, 450k methylation analysis revealed a high classifier score for HPC.
Method: We present a case of a 83-year-old female patient with paralysis of the lower extremities due to a intraspinal solitary fibrous tumor. Furthermore we performed a review of the literature.
Results: We present the case of a 83-year-old female patient presenting with acute spastic paralysis of the lower extremities after a history of progressive weakness and incontinence. MRI findings were typical for meningioma, but at the operation an intradural, chiefly intramedullary tumor was found . A second operation was performed with neurophysiological monitoring and complete removal of the tumor was achieved. Neurological conditions improved after the procedure but the patient remained paraparetic. Histological and immunohistochemical findings revealed a SFT. Nevertheless, 450k methylation analysis showed a high classifier score for the more malignant hemangiopericytoma (HPC).
Conclusions: There is evidence that SFT and HPC are not different entities but should be considered as different graduations of a common spectrum. The extent of resection is a prognostic factor for recurrence-free survival in SFT, therefore we recommend surgery with complete resection whenever possible depending on the results of mandatory intraoperative neurophysiological monitoring in these cases.
