chordoma
Pronunciation: kor-DOH-mah
A rare malignant bone tumor arising from notochordal remnants, typically occurring in the spine and skull base.
Full Definition
Chordoma is a rare primary malignant bone tumor that develops from remnants of the embryonic notochord, most commonly occurring in the sacrococcygeal region, clivus, and mobile spine. These slow-growing but locally aggressive tumors are characterized by physaliphorous cells containing intracytoplasmic vacuoles and mucoid intercellular matrix. Chordomas are notorious for local recurrence due to their intimate relationship with critical neurological structures, making complete surgical excision challenging. They are generally resistant to conventional chemotherapy and radiation therapy, though proton beam therapy may be beneficial.
Usage
Usage note: Specify anatomical location (sacral, clival, or mobile spine) as this affects treatment approach.
In Context
- "The sacral mass demonstrated characteristic features of chordoma on MRI." — Imaging report
- "Immunohistochemistry confirmed chordoma with positive brachyury staining." — Pathology report