Chordoma is a rare tumor arising from the remnant of primitive notochord. Peak incidence is in the 5th - 6th decades with a Male:Female ratio of 2:1. Usually it arises in the clivus (35%) and in the sacrococcygeal spine (53%), at the two ends of the notochord. Histologically, it consists of physaliphorous cells containing intracellular mucin. It is slow growing and locally osteodestructive. Its behavior causes a high recurrence rate (90%). This type of tumor rarely tends to metastasize in the lung, liver and bone. Three types are described: typical; chondroid; dedifferentiated.
The imaging studies reveal in MRI: T1WI - heterogeneous hypo- to isointense; T2WI - hyperintense to cerebro-spinal fluid; T1 + contrast: variable enhancement (blush to intense).
The treatment is total surgical removal, which is difficult due to its localization. Early postoperative radiation (4500-5500 rads) is associated with longer survival. Currently, proton beam therapy may be more effective than conventional postoperative radiation therapy alone.
The imaging studies reveal in MRI: T1WI - heterogeneous hypo- to isointense; T2WI - hyperintense to cerebro-spinal fluid; T1 + contrast: variable enhancement (blush to intense).
The treatment is total surgical removal, which is difficult due to its localization. Early postoperative radiation (4500-5500 rads) is associated with longer survival. Currently, proton beam therapy may be more effective than conventional postoperative radiation therapy alone.
References
- O’Neill P, Bell BA, Miller JD, et al. Fifty Years of Experience with Chordomas in Southeast Scotland. Neurosurgery 16:166-70, 1985.
- Cheng EY, Özerdemoglu RA, Transfeldt EE, et al.. Lumbosacral chordoma. Prognostic factors and Treatment. Spine 24:1639-45, 1999.
- Handbook of Neurosurgery - fifth edition - Mark S. Greenberg - ed. Thieme.
- Hugh EB, Loredo LN, Slater JD, et al. Proton Radiation Therapy for Chordomas and Chondrosarcomas of the Skull Base. J Neurosurg 91:432-9, 1999.
- Ross JS, Brant-Zawadzki M, Moore KR, et al. Diagnostic Imaging Spine. First edition - Amirsys - Elsevier Saunders - 2004.
