Advances in the management of primary bone sarcomas of the skull base.

Skull base primary malignancies represent a heterogeneous group of histologic diagnoses and sarcomas of the skull base are specific malignant tumors that arise from mesenchymal cells and can be classified by site of origin into bony and soft tissue sarcomas. The most common bony sarcomas include: chondrosarcoma, osteosarcoma, chordoma, Ewing's sarcoma. Given the relative rarity of each histologic diagnosis, especially in the skull base, there is limited published data to guide the management of patients with skull base sarcomas. An electronic search of the literature was performed to obtain key publications in the management of bony sarcomas of the skull base published within the last decade. This article is thus a review of the multi-disciplinary management principles of primary bony sarcomas of the skull base. Of note, there have been several recent advancements in the realm of skull base sarcoma management that have resulted in improved survival. These include advances in: imaging and diagnostic techniques, surgical techniques that incorporate oncologic surgical principles, conformal radiation paradigms and targeted systemic therapies. Early access to coordinated multi-disciplinary subspecialty care immediately at suspicion of diagnosis has further improved outcomes. There are several ongoing trials in the realms of radiation therapy and systemic therapy that will hopefully provide further insight about the optimal management of bony sarcomas of the skull base.