Abstract
BackgroundTreating chordoma through surgery alone is often ineffective. Thus, surgery often performed with irradiation, with a reported 5-year survival rate of 60–75%. The clinical course varies, and disease rarity prevents larger number of clinical investigations.MethodsIn total, 19 patients with clival chordomas were retrospectively extracted from our institutional database. They were initially treated with maximal tumor removal using the extended transsphenoidal approach between March 2006 and January 2021. When total tumor removal was achieved, prophylactic irradiation was not performed. If tumor remnants or recurrence were confirmed, Gamma Knife (GK) radiosurgery was performed. The mean follow-up period was 106.7 months (ranged 27–224 months). The clinical course and prognostic factors were investigated.ResultsTotal removal was achieved in 10 patients, whereas 4 patients suffered recurrence and required GK. GK was applied to 11 patients with a 50% isodose of 13–18 Gy (mean: 15.4 Gy), and eight patients remained progression free, whereas three patients suffered repeated local recurrence and died of tumor-related complications. The mean overall progression-free interval was 57.2 months (range: 6–169 months). One male patient died of tumor un-related lung cancer 36 months after the initial treatment, and other patients survived throughout the observational periods. The mean overall survival was 106.7 months (range: 27–224 months). Thus, the 5-year survival rate was 94.7%. Statistical analysis indicated that sex (men), > 15 Gy of 50% isodose by GK, and screening brain examinations as prophylactic medicine were significant favorable prognostic factors.ConclusionsThe favorable outcomes in this investigation suggest the importance of early detection and treatment. Surgery may enable better conditions for sufficient GK doses.
Highlights
Treating chordoma through surgery alone is often ineffective
When tumor recurrence was confirmed, Gamma Knife (GK) radiosurgery was subsequently applied to the visualized tumor bulk; this occurred in all patients except for one female patient, who was treated with postoperative fractionated irradiation because of her older age (85 years)
Initial treatments were applied in 17 patients; the remaining 2 patients had suffered from re-growth after step-wise tumor removal and radiation therapy (Table 1)
Summary
Treating chordoma through surgery alone is often ineffective. surgery often performed with irradiation, with a reported 5-year survival rate of 60–75%. The incidence rate is only 0.5% among all intracranial tumors and 1–4% among skeletal tumors [1, 16, 19, 22] This rarity of the disease has prevented extensive clinical investigations, causing a great discrepancy between the indolent pathological features and the malignant clinical course [5, 7,8,9,10, 12,13,14,15], with a reported 5-year survival rate of 60–75%. Tumor control by surgery alone is considered difficult, and most patients are treated using combined therapy with irradiation [6,7,8,9, 14, 17]. The rarity of this disease hinders the accumulation of clinical experiences, and health insurance barely covers treatments with ion beam therapy
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
