gms | German Medical Science

Objective: Clivus chordomas are rare and (semi-)malignant tumors that originate from remnants of the chorda dorsalis (Notochordoa). Due to the invasive and extensive growth, treatment remains challenging and is affiliated with poor prognosis. Treatment consists of surgical resection followed by carbon ion irradiation. During the last decades, surgical strategies and treatment options have changed from large trans-facial to endoscopic trans-nasal approaches. In this series we show our experiences in the management of clivus chordomas emphasizing changes in surgical strategies.

Methods: This retrospective analysis includes a total of 38 patients (21 male, 17 female) with a mean age of 43 years (range 16-77 years) treated in our department between 2006 and 2016. The mean follow-up interval was 52 months (range 6-168 month). The study includes symptom analysis, tumor location and classification, surgical approaches, analysis of peri- and postoperative deficits as well as adjuvant treatment options and long-term outcome.

Results: Most common symptoms were double vision (58%) and headaches (39.5%), with a mean duration of 17.9 ± 8 months. Tumors were classified into four groups considering tumor extension: 1. tumors of the clivus with dorsal extension (34%); 2. clivus tumors with frontotemporal extension (26.5%); 3. clivus tumor with growth into the cerebellopontine angle (10.5%); 4. extensive tumors growth involving the entire skull base (29%). In the majority of cases, the tumor has infiltrated the dura (76%) and the cavernous sinus (60.5%). According to the tumor extension, the surgical approach was chosen with a trend from large transfacial to endoscopic transnasial approaches within the observation time. Surgery resulted in significantly improved headaches (44.7% vs. 21.1%) and reduction of sixth nerve palsy (55.3% vs. 31.6%). Following primary resection, 68.2% of patients received adjuvant radiotherapy (80% carbon ion radiation) after a period of 4.9 ± 3.9 months. This resulted in a 40.7% recurrent-free survival after 5 years. Severe surgical complications (hemorrhage, stroke) with remaining neurological deficits occurred in only 6.9% of all interventions and exclusively in recurrent surgery.

Conclusion: Surgical resection of clivus chordomas is one of the most challenging skull base procedures. However, interdisciplinary treatment of Neuro-, ENT- and Maxillofacial-surgeons as well as radiotherapist can achieve clinical improvement and tumor control.