Chordomas of the skull base and cervical spine: clinical outcomes associated with a multimodal surgical resection combined with proton-beam radiation in 40 patients

Abstract

Previous studies of chordoma have focused on either surgery, radiotherapy, or particular tumor locations. This paper reviewed the outcomes of surgery and proton radiotherapy with various tumor locations. Between 2001 and 2008, 40 patients with chordomas of the skull base and cervical spine had surgery at our hospital. Most patients received proton therapy. Their clinical course was reviewed. Age, sex, tumor location, timing of surgery, extent of resection, and chondroid appearance were evaluated in regard to the progression-free survival (PFS) and overall survival (OS). The primary surgery (PS) group was analyzed independently. The extensive resection rate was 42.5%. Permanent neurological morbidity was seen in 3.8%. Radiotherapy was performed in 75% and the mean dose was 68.9 cobalt gray equivalents. The median follow-up was 56.5 months. The 5-year PFS and OS rates were 70% and 83.4%, respectively. Metastasis was seen in 12.5%. The tumor location at the cranio-cervical junction (CCJ) was associated with a lower PFS (P = 0.007). In the PS group, a younger age and the CCJ location were related to a lower PFS (P = 0.008 and P < 0.001, respectively). The CCJ location was also related to a lower OS (P = 0.043) and it was more common in young patients (P = 0.002). Among the survivors, the median of the last Karnofsky Performance Scale score was 80 with 25.7% of patients experiencing an increase and 11.4% experiencing a decrease. Multimodal surgery and proton therapy thus improved the chordoma treatment. The CCJ location and a younger age are risks for disease progression.

Determinants of quality of life in patients with skull base chordoma

“…7,14,43 The physical functioning of the patients in this study was reflected by a mean KPS score of 82, which is similar to that reported in the literature for other patients with skull base chordoma (mean KPS score range 69-84). 7,14,43,60 The congruence of the study cohort clinical characteristics with published data supports the external validity of our questionnaire. Internal validity was supported using previously validated functional and quality of life outcome instruments.…”

Incidence and relative survival of chordomas

“…The estimated OS rate was 89.5% for males versus 66.7% for females (P ¼ .5). 44 Likewise, the Heidelberg group did not observe a significant OS difference in 44 patients with chordoma who received carbon ion radiation therapy. 45 Those results are in keeping with other published series.…”

Systemic therapy for selected skull base sarcomas: Chondrosarcoma, chordoma, giant cell tumour and solitary fibrous tumour/hemangiopericytoma

“…17 Although chordoma does have a metastatic potential, its long term outcomes are mostly dependent on its local aggressiveness and pattern of local recurrence, affecting >50% of patients and requiring repeated surgical procedures and/or radiation therapy. 18 Due to their deep anatomic site, the proportion of local relapse is higher in skull base chordomas, 19 even after macroscopic complete surgery and/or definitive radiotherapy. Metastases have been reported in 30-40% of patients, with a late clinical presentation, commonly to the lungs, liver, bone, sub-cutis, lymph nodes and other sites.…”