Clinical Work-Up, Diagnostic Imaging, and Biopsy

Chordomas are rare malignancies belonging to a family of lesions derived from the primitive notochord. This family includes both benign notochordal cell tumors (BNCTs), e.g., giant vertebral notochordal rest tumors, eccordosis physaliphora, and the various subtypes of chordoma (classic, chondroid, and undifferentiated). Similarities in the imaging characteristic of these lesions may lead them to occasionally be completed with other neoplastic or infectious processes. Hence, the image-guided biopsy is necessary for definitive diagnosis. The conjunction of radiographic and histologic features allows for the differentiation of benign and malignant notochordal neoplasms from one another and from other entities in the differential. Additionally, thorough radiographic evaluation with magnetic resonance imaging, high-resolution computed tomography, and nuclear medicine or other staging scans enhances treatment planning. Using anatomic sequences, diagnostic radiologists can assist their surgical and radiation oncology colleagues in defining tumor boundaries, assessing the involvement of adjacent structures, and ultimately determining the feasibility of en bloc resection. In the future, immunohistological staining and gene sequencing may also facilitate the development of individualized therapies for those with disseminated disease at the time of diagnosis.