Clinical Outcomes following Surgery, Radiotherapy, and Medical Therapy for Clival Chordoma

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.

Publikationsverlauf

Artikel online veröffentlicht:
07. Februar 2025