Clival Chordomas: Long-Term Functional Outcome after Multimodal Therapy

Introduction: Chordomas are very rare tumors infiltrating the central cranial base. Surgical resection of these tumors is challenging, and long-term control difficult. The aim of this study was to evaluate clinical presentation, radiological features, and functional outcome after microsurgery and adjuvant radiotherapy in a series of patients harboring a clival chordomas.

Methods: Clinical, radiological, surgical, and follow-up data in a consecutive series of 28 patients operated on between January 1989 and June 2008 in 2 neurosurgical institutions were retrospectively reviewed.

Results: Main presenting symptoms in the 15 women and 13 men (mean age, 48 years) were diplopia in 10 (36%) and nuchal pain in 5 (18%) patients. Clinical examination revealed palsy of the 6th and 12th cranial nerves in 12 (43%) and 9 (32%) cases, respectively. Preoperative magnetic resonance imaging and bone window computed tomography scanning demonstrated involvement of the upper third of the clivus in 18 (64%), the middle third in 18 (64%), and lower third in 12 (43%) patients. Brainstem compression was present in 19 (68%) cases. Tumor resection was most commonly performed via the transsphenoidal route (50%). Postoperative radiological assessment revealed complete tumor resection in 8 (29%) patients. Adjuvant radiotherapy was administered in 16 (57%) cases. One patient died after surgery. The mean Karnofsky performance score in the remaining patients improved from 79 preoperatively to 87 after surgery. After a mean follow-up period of 51 months with clinical and radiological assessment, tumor progression was observed in 9 patients and tumor control was sustained in 12 patients. Four patients underwent a second surgery for tumor recurrence.

Discussion: Clival chordomas display a heterogeneous clinical behavior irrespective of the extent of surgical resection. Adjuvant radiotherapy with heavy particles apparently prolongs progression-free survival.