Sacral chordoma in an adult showing an aggressive clinical course: A case report

Abstract

The current report presents a case of a 78-year-old male with sacral chordoma, showing an aggressive clinical course. The patient underwent sacral resection, however, nine months later, multiple metastases were detected by magnetic resonance imaging. The metastases progressed rapidly and 15 months following surgery the patient succumbed to respiratory dysfunction. An autopsy revealed multiple metastases of the lung, liver, heart, kidneys and vertebrae. Pathologically, the tumors did not show proliferation of anaplastic cells or dedifferentiation; however, the metastatic tumor cells were smaller than the primary tumor cells. The Ki-67 labeling indices were <5% in all of the patient's tumors, therefore, the capacity for cellular proliferation of the tumors was considered to be low. Chordoma in adults are generally slow-growing tumors and are associated with a relatively prolonged course and frequent local recurrences. Therefore, it must be recognized that chordoma may grow rapidly and show an aggressive clinical course, even when the Ki-67 labeling index is low.

Keywords: Ki-67; aggressive; chordoma; sacrum.

Figure 1

Magnetic resonance image demonstrated a large sacral tumor that spread into the soft tissue and compressed the rectum.

Figure 2

Magnetic resonance image of the vertebrae nine months following surgery revealed multiple vertebral metastases; the metastatic lesion at the 4th thoracic vertebra had compressed the spinal cord.

Figure 3

Histology of the tumors. (A) Physaliferous cells were demonstrated in a myxoid matrix of the primary tumor (hematoxylin and eosin [H&E] stain; magnification, ×200). (B) An area of atypical features was identified in the fibrous cells of the primary tumor. However, no apparent proliferation of anaplastic cells was identified. In addition, tumor cells did not demonstrate bizarre nuclei or sarcomatous features (H&E stain; magnification ×100). (C) The metastatic tumor cells were smaller than the cells of the primary tumor. These stellate cells were predominant in the metastatic lesions (H&E stain; magnification, ×200).

Figure 4

Immunohistochemistry (Ki-67 immunolabeling) demonstrated sparse staining for Ki-67 in the tumor cells of the primary tumor.