gms | German Medical Science

63. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC)
Joint Meeting mit der Japanischen Gesellschaft für Neurochirurgie (JNS)

Deutsche Gesellschaft für Neurochirurgie (DGNC) e. V.

13. - 16. Juni 2012, Leipzig

Intracranial manifestation of a low-grade myofibrosarcoma (LGMS) – Case report

Meeting Abstract

  • M. Renovanz - Klinik für Neurochirurgie, Klinikum Stuttgart, Katharinenhospital
  • M.N. Nadji-Ohl - Klinik für Neurochirurgie, Klinikum Stuttgart, Katharinenhospital
  • P. Kohlhof - Institut für Pathologie, Klinikum Stuttgart, Katharinenhospital
  • N. Hopf - Klinik für Neurochirurgie, Klinikum Stuttgart, Katharinenhospital

Deutsche Gesellschaft für Neurochirurgie. Japanische Gesellschaft für Neurochirurgie. 63. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC), Joint Meeting mit der Japanischen Gesellschaft für Neurochirurgie (JNS). Leipzig, 13.-16.06.2012. Düsseldorf: German Medical Science GMS Publishing House; 2012. DocP 077

DOI: 10.3205/12dgnc464, URN: urn:nbn:de:0183-12dgnc4641

Veröffentlicht: 4. Juni 2012

© 2012 Renovanz et al.
Dieser Artikel ist ein Open Access-Artikel und steht unter den Creative Commons Lizenzbedingungen (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.de). Er darf vervielfältigt, verbreitet und öffentlich zugänglich gemacht werden, vorausgesetzt dass Autor und Quelle genannt werden.


Gliederung

Text

Objective: Low-grade myofibrosarcoma (LGMS) is a distinctive neoplasm, which has been described in soft tissues and bone that infiltrates locally but rarely metastasizes. Clinically, myofibrosarcomas are generally indolent but can relapse and metastasize even after a long period. Myofibrosarcomas can resemble benign myofibroblastic lesions, including nodular fasciitis and fibromatosis, as well as leiomyosarcoma and fibrosarcoma. To date there are no distinguished described reports of intracranial manifestations discussing diagnostics and treatment. Here we present the case of a young male patient with an intracranial lesion.

Methods: A 25-year-old man with the diagnosis of a LGMS since 2000 had manifestations of his tumor in the base of the skull (temporal and foramen magnum). In the MRI they were contrast enhancing and showing similar characteristics as meningiomas. Over the years regular controls with MRI were performed and the left temporal manifestation showed progression in 2010. A complete tumor resection was performed without any complication. In the follow-up the patient showed a stable disease without any progress of the other manifestation in the skullbase, a tumor staging via MRI showed no additional lesion.

Results: There are several cases with LGMS evaluated in literature, the clinical management of intracranial manifestations has not been described. We presented a case of a very young man with several lesions in the skullbase, one of them progressing. In consideration of the close relation of the tumor to the brain, although the dura was respected, surgery was planned without any evidence of neurological deficit. The aim was to avoid further progression and compression of the brain. As the patient is young regular short-term controls were performed. The concept is, if the other lesions show a tendency to grow, further surgery will be performed to avoid neurological deficits. As the LGMS is a very rare tumor there are no guidelines available. However, we recommend for intracranial lesions e.g. skullbase manifestations surgery at an early stage to avoid neurological deficits.

Conclusions: Intracranial LGMS are rare but due to their localisation potentially dangerous manifestations which should be treated before neurological deficits occur as there is no effective alternative treatment option like chemotherapy or irradiation to date. Regular controls and interdisciplinary collaboration are indispensable.