open access
Primary intracranial extraskeletal myxoid chondrosarcoma
- department of neurosurgery
- Department of Oncology and Radiotherapy, Lukaszczyk Oncological Center, Romanowskiej 2, 85-796 Bydgoszcz, Poland
- 1 st Department of Radiology, Medical University of Lublin, ul. Jaczewskiego 8, 20-090 Lublin, Poland
- Palliative Care Centre, La Martiniere Institute, Saclay, France
open access
Abstract
Extraskeletal myxoid chondrosarcomas (EMC) are extremely rare and are usually located in the deep soft tissues of the lower extremities. Less than 10 cases of intracranial EMC have been reported in the literature, making their management and early diagnosis difficult. We present a new case of intracranial EMC occurring in a 70-year-old woman presenting with a right frontal mass initially assumed to be a brain metastasis from breast adenocarcinoma. The optimal management of these tumours is also discussed. Analysis from the literature suggests that complete resection should be recommended, whenever feasible. Although the high risk for relapse after surgery encourages postoperative treatments, relative resistance to both radiotherapy and chemotherapy characterizes EMC. Future perspectives might include multimodal treatments with highly conformal radiotherapy modalities for dose escalation strategies or use of new molecules. Knowledge of these unusual malignant tumours will be the first step for improving patients’ outcome.
Abstract
Extraskeletal myxoid chondrosarcomas (EMC) are extremely rare and are usually located in the deep soft tissues of the lower extremities. Less than 10 cases of intracranial EMC have been reported in the literature, making their management and early diagnosis difficult. We present a new case of intracranial EMC occurring in a 70-year-old woman presenting with a right frontal mass initially assumed to be a brain metastasis from breast adenocarcinoma. The optimal management of these tumours is also discussed. Analysis from the literature suggests that complete resection should be recommended, whenever feasible. Although the high risk for relapse after surgery encourages postoperative treatments, relative resistance to both radiotherapy and chemotherapy characterizes EMC. Future perspectives might include multimodal treatments with highly conformal radiotherapy modalities for dose escalation strategies or use of new molecules. Knowledge of these unusual malignant tumours will be the first step for improving patients’ outcome.
Keywords
intracranial tumour, extraskeletal myxoid chondrosarcoma, brain neoplasm
Title
Primary intracranial extraskeletal myxoid chondrosarcoma
Journal
Neurologia i Neurochirurgia Polska
Issue
Pages
76-81
Page views
411
Article views/downloads
313
DOI
10.5114/ninp.2012.27176
Bibliographic record
Neurol Neurochir Pol 2012;46(1):76-81.
Keywords
intracranial tumour
extraskeletal myxoid chondrosarcoma
brain neoplasm
Authors
Renaud Dulou
Cyrus Chargari
Arnaud Dagain
Christophe Teriitehau
Olivier Goasguen
Olivier Jeanjean
Lionel Védrine