title: Primary intracranial extraskeletal myxoid chondrosarcoma.
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 radio-therapy 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.
authors.affiliation: Department of Neurosurgery, Val-de-Grâce Hospital, Paris, France. renaud.dulou@gmail.com
authors.forename: Renaud
authors.initials: R
authors.lastname: Dulou
authors.forename: Cyrus
authors.initials: C
authors.lastname: Chargari
authors.forename: Arnaud
authors.initials: A
authors.lastname: Dagain
authors.forename: Christophe
authors.initials: C
authors.lastname: Teriitehau
authors.forename: Olivier
authors.initials: O
authors.lastname: Goasguen
authors.forename: Olivier
authors.initials: O
authors.lastname: Jeanjean
authors.forename: Lionel
authors.initials: L
authors.lastname: Védrine
mesh_terms: D000368:Aged; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery*; D002813:Chondrosarcoma / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery*; D005260:Female; D005625:Frontal Lobe; D006801:Humans; D009236:Myxosarcoma / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery*