Introduction: Skull-base chordoma is a pathology associated with high morbidity and limited treatment
options. The current standard of care is maximal surgical resection with adjuvant
radiotherapy. While long-term control is achieved for some patients, durable disease
remission is heavily dependent on initial extent of resection and completion of adjuvant
radiation.
Objective: This article aims to compare the long-term outcomes of clival chordoma in patients
undergoing maximal surgical resection and postoperative radiotherapy as well as identification
of outcomes of patients requiring salvage medical therapy.
Methods: A retrospective, single-center cohort study of patients undergoing surgical resection
for clival chordoma from 1994 to 2022 was performed.
Results: A total of 80 patients were included for study with an average follow-up of 53.8
months. A total of 56% of the cohort were men and the average age of all patients
was 44.3 years. The average tumor volume was 20.6 cm3. The most common presenting symptom was diplopia. Given the time range of patient
inclusion, the most common surgical approach was craniotomy (n = 48, 60%) followed by endoscopic transsphenoidal approach (n = 32, 37.5%), and combined open/endoscopic approach (n = 2, 2.5%). Gross total resection was achieved in 36 (45%) patients. In the 44 (55%)
patients who underwent subtotal resection, 32 (40%) required an additional surgical
resection, and 9 (11.3%) required three or more resections. Postoperative radiotherapy
was performed in 59 (73.8%) patients with proton radiotherapy being the most common
treatment modality (69.5%). Tumor recurrence occurred in 6 (16.7%) patients with prior
GTR, while 15 (34.1%) patients previously undergoing STR had progression of disease
(p = 0.033, [Fig. 1]). Outcomes following either GTR or STR were improved with adjuvant radiotherapy
(p < 0.0001, [Fig. 2]). GTR at the time of the index surgery significantly improved the rate of overall
mortality as compared to STR (p = 0.041, [Fig. 3]). Four patients in the cohort were treated with medical therapy for multiple-recurrent
tumors who were not a candidate for additional surgery or radiation. Agents included
afatinib, imatinib, etoposide, adriamycin, and ifosfamide. Of this subgroup, one case
experienced disease stability. Two patients died within 2 years of initiation of medical
therapy.
Fig. 1 Progression/Recurrence-free survival by extent of resection.
Fig. 2 Recurrence-free survival based on radiotherapy status.
Fig. 3 Overall survival by extent of resection.
Conclusion: Extent of resection and radiotherapy are significant risk factors for disease recurrence
and overall mortality. Outcomes remain poor in the setting of multiple-recurrent lesions
requiring salvage medical therapy.
