Chordoma of the mobile spine and sacrum: clinical management and prognosis

Abstract

Background: Chordomas are rare malignant tumors of the spinal column often afflicting the upper cervical spine and sacrum. There are few large single center series on chordomas due to its rarity. The purpose of this study is to report a single center's experience with the operative and non-operative management of chordomas.

Methods: We evaluated our institution's pathology database from 1994 to 2016 to identify patients diagnosed with chordomas. Inclusion criteria were chordomas of the mobile spine and sacrum. Exclusion criteria were chordomas of the clivus and resection performed at another institution. We collected patient demographics as well as the type of resection performed, intra-operative complications, wound complications and recurrence/prognosis.

Results: We identified 18 patients diagnosed with chordomas at our institution, and 12 met our inclusion criteria. There were four females and eight males with an average age of 64 [32-87] years. All patients were Caucasian and 10 of the 12 had surgery. Of the two patients with sacral chordomas that did not have surgery, one received chemotherapy and the other did not elect for any treatment. One is alive 161 months following diagnosis and the second died 96 months following diagnosis. Five of the lesions were in the mobile spine (one cervical, two thoracic, two lumbar) and seven were in the sacrum. Six patients underwent an en bloc resection [two via total en bloc spondylectomy (TES)]. Average length of follow up is 60 [3-161] months and eight of 12 patients are alive at latest follow up. Intraoperative complications included cardiac arrest, pleural tear, and excessive blood loss (8 L). Two patients, with sacral chordomas, had wound complications. Recurrence occurred in one patient with piecemeal resection and one patient with incomplete resection had post-operative metastatic lesions to the liver and lung.

Conclusions: We found less recurrence in patients managed with an en bloc resection as opposed to piece meal or intralesional resections. Sacral chordoma patients had higher wound complication rates as compared to chordomas of the mobile spine. The long life expectancy of non-surgically managed patients underscores the indolent nature of chordomas.

Keywords: Complications; chordoma; en bloc spondylectomy; mobile spine; outcomes; sacrum.

Figure 1

A 69-year-old male diagnosed with chordoma after episodes of neurogenic bowel and bladder. Due to multiple medical co-morbidities the decision was made to manage him with chemotherapy rather than en bloc excision. The patient also declined radiation treatment. (A) Sagittal T2 weighted MRI and (B) axial T2 weighted MRI demonstrating the sacral lesion in 9/2008. Four years later, 9/2012, an increase in the size of the lesion is noted (C,D) on sagittal and axial images. The patient passed away 96 months following diagnosis. MRI, magnetic resonance imaging.

Figure 2

A 50-year-old man with T6 chordoma. (A) T2-weigthed sagittal magnetic resonance imaging (MRI) demonstrates the lesion at T6 with epidural extension; (B) he underwent all posterior total en bloc spondylectomy (TES) of T6; (C) this was followed by instrumentation and fusion from T3 to T9 and expandable cage placement from T5 to T7.

Figure 3

Histology from T6 chordoma resection demonstrating (arrow) the physaliferous cells (soap-bubble appearance) found in chordomas. H&E staining using a ×20 objective lens.