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Objective Chordomas are locally aggressive, highly recurrent tumors requiring adjuvant radiotherapy following resection for successful management. We retrospectively reviewed patients treated for intracranial chordomas with adjuvant stereotactic radiosurgery (SRS) and stereotactic radiation therapy (SRT). Methods A total of 57 patients underwent 83 treatments at the UCLA Medical Center between February 1990 and August 2011. Mean follow-up was 57.8 months. Mean tumor diameter was 3.36 cm. Overall, 8 and 34 patients received adjuvant SRS and SRT, and the mean maximal dose of radiation therapy was 1783.3 cGy and 6339 cGy, respectively. Results Overall rate of recurrence was 51.8%, and 1- and 5-year progression-free survival (PFS) was 88.2% and 35.2%, respectively. Gross total resection was achieved in 30.9% of patients. Adjuvant radiotherapy improved outcomes following subtotal resection (5-year PFS 62.5% versus 20.1%; p = 0.036). SRS and SRT produced comparable rates of tumor control (p = 0.28). Higher dose SRT (> 6,000 cGy) (p = 0.013) and younger age (< 45 years) (p = 0.03) was associated with improved rates of tumor control. Conclusion Adjuvant radiotherapy is critical following subtotal resection of intracranial chordomas. Adjuvant SRT and SRS were safe and improved PFS following subtotal resection. Higher total doses of SRT and younger patient age were associated with improved rates of tumor control.

Introduction Papillary meningiomas (PMs) are characterized by their aggressive nature and high rate of recurrence. Due to their rarity, studies examining the relationship between treatment and clinical outcomes for this disease are limited. Gross total resection (GTR) with or without radiotherapy (RT) is considered the standard treatment; however, when GTR is not feasible, subtotal resection (STR) followed by RT may be an effective alternative. In this study, we analyzed the clinical outcomes in patients who either underwent GTR alone, GTR followed by RT, STR alone, or STR followed by RT. Methods A systematic analysis was performed to identify PM patients with sufficient follow-up and outcome data, as measured by recurrence. Patient data lacking extent of resection, follow-up, or recurrence information were excluded. Results A total of 29 patients with PM were treated with resections (23 GTRs and 6 STRs).The mean age and mean follow-up of patients in this study were 32.3 years and 42.1 months, respectively. Of these patients, 58.6% experienced recurrence. Overall, 47.8% of patients who underwent GTR experienced recurrence. These patients also demonstrated improved survival compared with STR. Among patients whose tumors were only partially excised, a recurrence rate of 83% was observed. Conclusion Our results confirm that GTR results in fewer recurrences compared with STR, supporting GTR as the treatment of choice for PM. Furthermore, GTR in conjunction with RT resulted in improved survival compared with GTR alone. When GTR was not feasible, STR with RT was associated with improved survival compared with STR alone. Future studies with more outcome data are needed to elucidate the optimal treatment for this rare disease.

Management of intracranial chordomas remains challenging, despite improvements in microsurgical techniques and radiotherapy. Here, we analyzed the prognostic factors associated with improved rates of tumor control in patients with intracranial chordomas, who received either gross (GTR) or subtotal resections (STR). A retrospective review was performed to identify all patients who were undergoing resection of their intracranial chordomas at the Ronald Reagan University of California Los Angeles Medical Center from 1990 to 2011. In total, 57 patients undergoing 81 resections were included. There were 24 females and 33 males with a mean age of 44.6 years, and the mean tumor diameter was 3.36 cm. The extent of resection was not associated with recurrence. For all 81 operations, the 1 and 5 year progression free survival (PFS) was 87.5 and 40.4%, and 88.0 and 33.6% for STR and GTR, respectively ( p = 0.90). Adjuvant radiotherapy was associated with improved rates of PFS (hazard ratio [HR] 0.20; p = 0.009). Additionally, age >45 years (HR 5.88; p = 0.01) and the presence of visual deficits (HR 7.59; p = 0.03) were associated with worse rates of tumor control. Tumor size, sex, tumor histology, and recurrent tumors were not predictors of recurrence. Younger age, lack of visual symptoms on presentation and adjuvant radiotherapy were associated with improved rates of tumor control following surgery. However, GTR and STR produced comparable rates of tumor control. The surgical management of intracranial chordomas should take a conservative approach, with the aim of maximal but safe cytoreductive resection with adjuvant radiation therapy, and a major focus on quality of life. [ABSTRACT FROM AUTHOR]