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

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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 χ2, 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 cm3) 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 cm3). 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.

ABBREVIATIONS EEA = endoscopic endonasal approach; ES = ethmoid-sphenoid; GTR = gross-total resection; KPS = Karnofsky Performance Scale; MargR = marginal resection; NTR = near-total resection; OC = occipitocervical; OS = overall survival; PFS = progression-free survival; PO = petrous-occipital; PR = partial resection; SC = sphenoclival; SP = sphenopetrous; STR = subtotal resection.

Article Information

Correspondence Junting Zhang, Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Tiantan Xili 6, Dongcheng District, Beijing 100050, People's Republic of China. email: zhangjunting2003@aliyun.com.

INCLUDE WHEN CITING Published online January 6, 2017; DOI: 10.3171/2016.9.JNS16559.

Drs. L. Wang and Z. Wu contributed equally to this work.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

© AANS, except where prohibited by US copyright law.

Headings

Figures

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    Tumor location and bone invasion classifications for skull base chordomas. Left: Classifications of tumor location. 1) SC type—tumor in the upper two-thirds of the clivus, main body in the middle line, anterior to the sphenoid sinus and posterior to the prepontine cistern; 2) OC type—tumor in the lower one-third of the clivus and the foramen magnum, main body in the middle line, anterior to the pharynx and posterior to the premedullary cistern; 3) SP type—tumor in the parasellar or petrous apex, main body in the middle fossa, medial to the cavernous sinus and intracavernous segments of the carotid artery displaced medially or laterally; 4) PO type—tumor in the posterior fossa laterally, from the lateral side of the Meckel cavity to the cerebellopontine angle and to the jugular foramen; 5) ES type—tumor in the anterior fossa, and posterior to the pituitary fossa; and 6) extensive type (not shown)—tumor with a huge volume involving at least 2 of the 5 parts mentioned above. Right: Classifications of bone invasion. 1) endophytic type (Type I), invades the bones through every direction, with clivus transformation described as a “bubble” or a “dumbbell”; 2) intrinsic type (Type II), which is relatively rare and is represented by inner bony lesions with neither extraosseous nor intraosseous extension trends; and 3) exophytic type (Type III), which has limited bone invasiveness, showing a “bulge-like” image in the retroclival region. Copyright Da Li. Published with permission.

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    MR images of bone invasion classifications. A: A case labeled as endophytic type because the tumor had extensively invaded the clivus, involved the sphenoid sinus anteriorly, and compressed the brainstem posteriorly. B: A case of intrinsic type, which was totally confined within the clivus. C: A case classified as exophytic type. D: An MR image at 10-year follow-up of the same case shown in A. E: The same case shown in B, demonstrating marked change in the extent of bone invasion 4.5 years later. F: After a GTR, there was no sign of recurrence at the 7-year follow-up of the case shown in C.

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    Bar graphs demonstrating the distribution of tumors by tumor location system, with initial symptoms (upper) and surgical approaches (lower).

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    Kaplan-Meier curves of positive factors associated with OS and PFS. A and B: Differences in OS and PFS between primary and recurrent tumors. C: Graph demonstrating that the number of prior operations was inversely related to OS. D: Graph showing that the exophytic-type tumor was associated with better PFS. E and F: Graphs illustrating the influence of resection degree on OS and PFS, respectively. It was obvious that more radical resection could provide longer survival benefit in terms of both OS and PFS.

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