AIM: To address the diagnostic and therapeutic challenges posed by primary clival bone lesions, specifically chordoma, chondrosarcoma, giant cell tumor, and benign notochordal cell tumor, by providing a comprehensive review of their clinical, radiological, and biological characteristics.

METHODS: A systematic review was performed using the Cochrane Library, Scielo, and Medline databases. The search incorporated MeSH terms such as “chordomas”, “chondrosarcoma”, “giant cell tumor of bone”, and “bone neoplasms”. From 2630 initial results, 33 studies were selected and categorized by tumor type, histology, clinical presentation, imaging findings, diagnosis, treatment, and prognostic factors.

RESULTS: Despite extensive literature on individual aspects of these tumors, comprehensive reviews encompassing all four primary clival lesions are scarce. These tumors exhibit overlapping radiological features and complex anatomical locations, each with distinct biological behavior. The systematic review highlights the importance of differentiating among these lesions to inform diagnosis and management, as well as the current limitations in evidence-based treatment strategies due to the rarity and complexity of these entities.

CONCLUSIONS: Understanding the key characteristics of primary clival bone lesions is essential for clinicians and neurosurgeons to select optimal diagnostic and therapeutic approaches, ultimately aiming to improve patient outcomes.