Clinical features and surgical outcomes of patients with skull base chordoma: a retrospective analysis of 238 patients

Abstract

OBJECTIVE Skull base chordoma is relatively rare, and a limited number of reports have been published regarding its clinical features. Moreover, the factors associated with extent of resection, as well as the value of marginal resection for long-term survival, are still in question for this disease. The objective of this study was to investigate these factors by evaluating their clinical features and surgical outcomes. METHODS A retrospective analysis of 238 patients with skull base chordomas, who met the inclusion criteria, was performed. This study summarized the clinical features, selection of approaches, degree of resection, and postoperative complications by statistical description analyses; proposed modified classifications of tumor location and bone invasion; studied the contributions of the clinical and radiological factors to the extent of resection by Pearson χ, ANOVA, rank test, and binary logistic regression analysis; and estimated the differences in overall survival and progression-free survival rates with respect to therapeutic history, classification of tumor location, extent of bone invasion, and extent of tumor resection by the Kaplan-Meier method. A p value < 0.05 was considered statistically significant. RESULTS The study included 140 male and 98 female patients with a mean age of 38.1 years. Headache and neck pain (33.2%) and diplopia (29%) were the most common initial symptoms. Sphenoclival type accounted for the largest proportion of tumor location (59.2%); endophytic chordoma was the more common type of bone invasion (81.5%). Lateral open approaches were performed in two-thirds of the study population (78.6%). The rate of marginal resection was 66%, composed of gross-total resection (11.8%) and near-total resection (54.2%). Meningitis (8%) and CSF leakage (3.8%) were the most frequent complications. The mean follow-up period was 43.7 months. The overall survival and progression-free survival rates at 5 years were 76% and 45%, respectively. Recurrent tumor and larger tumor volume (≥ 40 cm) were identified as risk factors of marginal resection. Patients who presented with recurrent tumor and underwent intralesional resection had a worse long-term outcome. CONCLUSIONS The classifications of both tumor location and bone invasion demonstrated clinical value. Marginal resection was more likely to be achieved for primary lesions with smaller volumes (< 40 cm). The rate of CSF leakage declined due to improved dura mater repair with free fat grafts. Marginal resection, or gross-total resection when possible, should be performed in patients with primary chordomas to achieve better long-term survival.

Application of endoscopic endonasal approach in skull base surgeries: summary of 1886 cases in a single center for 10 consecutive years

“…In case of chordomas which origin from extracranial, we can totally or subtotally resect the tumor through an endoscopic endonasal approach without traction or exposure of the brain tissue. The total/subtotal resection rate of chordomas in this group was 66.8%, and the incidence of complications was lower than that reported in the literature [28]. Most chordomas originated from the clivus and presented with a variety of different sizes, locations, and tumor texture consistency and blood supply.…”

Long Follow-up Surgical Results in 284 Cases of Clival Chordomas: The Risk Factors for Outcome and Tumor Recurrence

“…When the tumor was mainly located in the paramedian region, we chose the MLOA. The resection rate was divided into four grades according to the postsurgical MRI and CT combined with the intraoperative impressions of surgeons [30]. Gross-total resection (GTR) indicated that the entire tumor was resected, the surrounding heathy tissue was obviously exposed, and no suspected tumor could be found on postsurgical images.…”

“…No residual tumor was found during surgery, and > 90% tumoral resection on images was de ned as near-total resection (NTR); > 70% tumoral resection on images was de ned as subtotal resection (STR), and a lesser extent of resection was de ned as partial resection (PR). For the sake of comparison, GTR and NTR were pooled and classi ed as marginal resection, and STR and PR were classi ed as intralesional resection [30,24,25].…”

“…Skull-base chordomas (SBCs) account for 32% of all chordomas [20]. Local recurrence is common with a late recurrence rate > 50% [24,9,30,14,10]. The average survival after surgery with or without radiation therapy (RT) is approximately 7.7 years [35].…”

Endoscopic endonasal transclival removal of tumors of the clivus and anterior region of the posterior cranial fossa (results of surgical treatment of 140 patients)

“…With the development of modern endoscopic techniques, more and more studies have started to emerge in the world literature regarding the experience of surgical removal of centrally located tumors of the base of the skull using the endoscopic transclival approach [10][11][12][13][14][15][16][17][18][19][20]. According to Sanmillan et al [15], the results of using the endoscopic transclival approach for the removal of central tumors of the skull base are comparable to the results of using various transcranial approaches, and in a number of cases, they are clearly superior.…”