Results following surgical resection of recurrent chordoma of the spine: experience in a single institution

Abstract

Background: Chordoma of the spine is a low-grade malignant tumor with vague and indolent symptoms; thus, large tumor mass is encountered at the time of diagnosis in almost cases and makes it difficult for en-bloc free-margin resection. Salvage therapy for recurrent chordoma is very challenging due to its relentless nature and refractory to adjuvant therapies. The aim of this present study was to report the oncologic outcome following surgical resection of chordoma of the spine.

Materials and methods: Retrospective review of 10 consecutive cases of recurrent chordoma patients who underwent surgical treatment between 2003 and 2018 at one tertiary-care center was conducted.

Results: There were 10 patients; 4 females and 6 males were included in this study. Eight patients had local recurrence. The recurrence was encountered at the muscle, surrounding soft tissue, and remaining bony structure. Distant metastases were found in 2 patients. The median time to recurrence or metastasis was 30 months after first surgery.

Conclusion: En-bloc free-margin resection is mandatory to prevent recurrence. The clinical vigilance and investigation to identify tumor recurrent should be performed every 3 to 6 months, especially in the first 30 months and annually thereafter. Detection of recurrent in early stage with a small mass may be the best chance to perform an en-bloc margin-free resection to prevent further recurrence.

Keywords: Chordoma; Oncologic outcome; Radiotherapy; Recurrence; Surgical resection.

Fig. 1

Measurement method, maximum length in axial image (a) on anterior-posterior (AP) dimension; L, in coronal dimension; W, and in sagittal reconstruction image (b); H, as illustrated in white arrow. The proximal vertebral level is defined as the upper most vertebrae at the end of the tumor mass (dot line in b)

Fig. 2

Illustrated skin incision used in the present study. The longitudinal incision (a), transverse incision (b), and inverted Y incision (c). Note: The angle between 2 distal incisions was 120° to avoid skin edge problems

Fig. 3

Illustrated surgical result of case No. 2. The initial MRI scan as shown in Fig. 1 in axial (Fig. 1a) and sagittal (Fig. 1b) CT image after surgery (a) demonstrated residual tumor at S4 (star). The residual tumor was gradual increase in size as shown in MRI images (b, c). The surgical removal of remaining mass was performed and revealed large seroma after surgery (d) which gradually resolved at 21 months, postoperatively (e)

Fig. 4

Illustrated oncologic result of case No. 9. The initial surgery was sacrectomy (S3), and 5 years later L2 metastasis was found; therefore, en-bloc spondylectomy was performed (a) and (b). Two years later T9 metastasis was detected and en-bloc spondylectomy at T9 vertebra was performed with rod extension (c, d). Three years later, wide excision of tumor with endoprosthesis replacement was performed due to proximal humerus metastasis (e, f) and total sacrectomy with extended distal fixation to ilium was performed at 3 months, subsequently (g, h)

Fig. 5

Illustrated oncologic outcome of case No. 8. A 35-year-old male presented with chordoma at C1-C3 with compressive myelopathy, initial MRI (a). The surgery was performed by decompressive laminectomy, tumor removal with cervical fixation from C1-C4 and anterior subtotal tumor removal but remaining residual tumor (b). The radiotherapy was delayed and resulted in enlargement of the tumor (c)

Fig. 6

Illustrated oncologic result of case No. 10. A 47-year-old female presented with concomitant chordoma at distal sacrum and L4 vertebra (a), the sacral mass and L4 vertebra were removed by en-bloc resection within 1 month after presentation (b). However, subsequent bone scan revealed generalized bone metastasis (c)