We present a case of a primary leptomingeal fibrosarcoma in a patient, who had previously undergone radical resection of a liposarcoma of the chest and abdominal wall 18 years prior. In the year following resection of his intracranial fibrosarcoma and treatment with adjuvant radiotherapy, the patient was diagnosed with an in-situ recurrence of his original chest and abdominal wall liposarcoma. Primary intracranial fibrosarcomas are rare, particularly in the setting of a previous metachronous systemic sarcoma. Moreover this case is of particular interest since the patient was being treated for diabetes with agents that inhibit peroxisome proliferator-activated receptors (PPARs) and recent reports have suggested a potential relationship between these nuclear receptors and the development of fibrosarcomas and liposarcomas. With reference to the current case report, we briefly review the current guidelines for the management of intracranial fibrosarcoma and discuss the postulated relationship between PPAR agonists and sarcomas.  

Key words: Primary intracranial fibrosarcoma, liposarcoma, PPAR, thiazolidinedione.