Retroperitoneal High Grade Undifferentiated Pleomorphic Sarcoma as a Second Metachronous Neoplasm Following Intracranial Meningioma

Introduction: Meningiomas and retroperitoneal sarcomas are two distinct rare tumors, with an incidence of 2 and 0.4 cases per 100,000 respectively. Case Report: We report the case of a 39 year old woman with a history of intracranial meningioma who was found two months later to have a large retroperitoneal sarcoma on CT scan after presenting to the emergency department complaining of abdominal pain. Pathologic evaluation was consistent with a high grade undifferentiated sarcoma. Discussion: It is highly unusual for a 39 year old otherwise healthy patient to present with two rare, seemingly unrelated tumors. It is our suspicion that there is a genetic component involved, however to date no association has been described in the literature between intracranial meningiomas and extracranial sarcomas. Conclusion: Besides being the first report of a retroperitoneal sarcoma occurring as a second metachronous neoplasm following an intracranial meningioma, we suspect that there is a genetic component involved. Further studies may indicate a genetic mutational link.