Lumbosacral chordoma - Prognostic factors and treatment

Study Design. Retrospective analysis. Objectives. To analyze the prognostic factors in patients with chordomas, t he success of various treatments, the diagnostic Value of open versus needl e biopsy, the neurologic impairment after sacral nerve resection, and the,c linical presentation and site of origin. Summary of Background Data. Staging of chordomas has not been of much value , compared with other bone tumors, because for chordomas, grade is similar, metastasis is infrequent at presentation, and the prognostic significance of size is uncertain. Methods. A review of patients with chordoma from 1965 through 1996 found 23 cases (mean age of patients, 55 years). The mean follow-up was 84 months. Mean tumor size was 81 mm (range, 35-135 mm), location was lumbar (n = 6), S1 (n = 4), S2 (n = 3), S3 (n = 7), S4 (n = 2), and S5 (n = 2). Results. No tumors were found in the higher sacrum (S1-S2) alone, without i nvolvement of the lower sacrum. Survival analysis at 5 years showed overall survival (OS) 86%, continuous disease-free survival (CDFS) 58%, and local recurrence-free survival (LRFS) 60%. The location of tumor, defined by high est level of involvement (lumbar vs, sacrum) was of prognostic significance for OS (P = 0.01; log-rank lest), CDFS (P = 0.036), but not for LRFS (P = 0.189). Results of multivariate regression showed that location was signifi cant for OS (P = 0.007), CDFS (P = 0.008), and LRFS (P = 0.001). For patien ts with positive margins (n = 16), initial radiation correlated with longer CDFS(P = 0.002; Mantel-Cox) and LRFS(P = 0.005, Mantel-Cox), but was not s ignificant for OS (P = 0.41). For patients who received no radiation, a pos itive margin correlated with a shorter CDFS (P = 0.04), a trend to shorter LRFS (P = 0.08), but no difference in OS. Therefore, both a tumor-free marg in and initial radiation correlated with a longer survival. No patients had urinary or bowel dysfunction when both S3 nerves were preserved. If one S3 nerve was preserved, 1 of 3 patients had partial urinary incontinence and 2 of 3 patients required bowel medications. If both S3 nerves were resected , all patients required intermittent urinary catheterization and bower medi cations. If both S2 nerves were resected, there was complete urinary and bo wel incontinence. Conclusions. The highest revel of tumor; involvement was prognostically sig nificant for OS, CDFS, and LRFS. Radiation was of value when complete excis ion was not achieved. Bilateral S3 nerve preservation is necessary to ensur e retention of normal urinary and bowel function.