Figure 1113

Pelvic CT showing an extensive destructive lytic lesion that involves the sacral bone and invades the right sacroiliac joint. The mass bulges anteriorly into the pelvis and posteriorly into the subcutaneous fatty tissue. Note some amorphous intratumoral calcifications. Courtesy Dr. D. Lerer.

Clinical Presentation

Most patients present with back pain, radicular pain, lower extremity weakness, and bladder, bowel, or sexual dysfunction.

Imaging Studies

Plain films may reveal a destructive sacral lesion or clivus mass, which may be lucent with some amorphous calcification. The mass can destroy the bone and invade adjacent structures. Quite often the sacral tumor penetrates into the epidural space and/or the para-vertebral tissues. On CT, the tumor mass appears hypodense to the adjacent normal tissue and enhances mildly or moderately following contrast administration (Figure 11-13). MRI is the procedure of choice in establishing the size of the tumor and its extent. Most chordomas appear heterogeneous on MRI. They tend to be isointense on T1WI with foci of high signal intensity, and hyperintense on T2WI. Low signal septations may appear on T2WI. Postcontrast enhancement may vary from mild (blush) to intense enhancement (Figures 11-14A, 11-14B, and 11-14C). T2WI with fat saturation may help delineate the extension of the tumor into the adjacent soft tissues. Frequently the tumor appears lobulated.

Management

Early diagnosis combined with radical en bloc resection and, at times, radiation improves disease-free intervals and enhances quality of life.

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