Management of sinonasal and skull base nonmesenchymal chondrosarcoma, a narrative review

Abstract

Background: Chondrosarcoma (CS) is a rare malignant cartilage forming tumor accounting for 6% of skull base neoplasia. CS often presents insidiously, it almost never metastasizes but it has an indolent progressive course leading to significant morbidity and mortality due to invasion of the clivus and central skull base. This study performed a review of the management of CS. Methods: A review of English language literature identified 20 studies to which we added a case series from one institution. Results: Total number of patients included was 734. Main treatment modality was surgery performed in combination with postoperative radiotherapy (64.0%) or surgery alone (30.4%). The majority of patients recorded were either alive and well (68.1%) or alive with disease (16.6%) with 15.3% dead of disease. Conclusions: On present evidence, surgery should form the primary treatment with a goal of complete resection. Inaccessible recurrences or high grade tumors are candidates for proton beam radiotherapy following surgery.