Management of Recurrent Skull Base Chordomas: An Analysis of Factors Affecting Progression Free and Overall Survival

Abstract BACKGROUND Skull base chordomas in children are extremely rare. Their course, management, and outcome have not been defined. OBJECTIVE To describe the preeminent clinical and radiological features in a series of pediatric patients with skull base chordomas and analyze the outcome of a cohort who underwent uniform treatment. We emphasize predictors of overall survival and progression-free survival, which aligns with Collins’ law for embryonal tumors. METHODS Thirty-one patients with a mean age of 10.7 yr (range 0.8-22) harboring skull base chordomas were evaluated. We retrospectively analyzed the outcomes and prognostic factors for 18 patients treated by the senior author, with uniform management of surgery with the aim of gross total resection and adjuvant proton-beam radiotherapy. Mean follow-up was 119.2 mo (range 8-263). RESULTS Abducens nerve palsy was the most common presenting symptom. Imaging disclosed large tumors that often involve multiple anatomical compartments. Patients undergoing gross total resection had significantly increased progression-free survival (P = .02) and overall survival (P = .05) compared with those having subtotal resection. Those who lived through the period of risk for recurrence without disease progression had a higher probability of living entirely free of progression (P = .03; odds ratio = 16.0). Age, sex, and histopathological variant did not yield statistical significance in survival. CONCLUSION Long-term overall and progression-free survival in children harboring skull base chordomas can be achieved with gross surgical resection and proton-beam radiotherapy, despite an advanced stage at presentation. Collins’ law does apply to pediatric skull base chordomas, and children with this disease have a high hope for cure.

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Why is female sex an independent predictor of shortened overall survival after proton/photon radiation therapy for skull base chordomas?

International Journal of Radiation Oncology*Biology*Physics ◽

10.1016/s0360-3016(97)00041-2 ◽

1997 ◽

Vol 38 (2) ◽

pp. 225-230 ◽

Cited By ~ 31

Author(s):

Edward C. Halperin

Keyword(s):

Overall Survival ◽

Radiation Therapy ◽

Skull Base ◽

Independent Predictor ◽

Photon Radiation ◽

Female Sex ◽

Skull Base Chordomas

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Skull base chordomas: clinical outcome in a consecutive series of 45 patients with long-term follow-up and evaluation of clinical and biological prognostic factors

Journal of Neurosurgery ◽

10.3171/2015.6.jns142370 ◽

2016 ◽

Vol 125 (2) ◽

pp. 450-460 ◽

Cited By ~ 24

Author(s):

Nicola Boari ◽

Filippo Gagliardi ◽

Andrea Cavalli ◽

Marco Gemma ◽

Luca Ferrari ◽

...

Keyword(s):

Overall Survival ◽

Multivariate Analysis ◽

Radiation Therapy ◽

Clinical Outcome ◽

Skull Base ◽

Survival Rates ◽

Progression Free Survival ◽

Free Survival ◽

Skull Base Chordomas

OBJECTIVE Skull base chordomas (SBCs) are rare dysembryogenetic invasive tumors with a variable tendency for recurrence. According to previous studies, the recurrence rate seems to be affected by both clinical variables and tumor biological features. The authors present the results of treatment of SBCs in a large series of patients and investigate the role of 1p36 chromosomal region loss of heterozygosity (LOH) as a prognostic factor. METHODS Between 1990 and 2011, 45 patients were treated for SBCs. The mean follow-up was 76 months (range 1–240 months). An LOH analysis was performed in 27 cases. Survival analysis was performed to determine clinical and biological parameters correlating with clinical outcome. RESULTS The 5- and 10-year overall survival rates were 67% and 57%, respectively. Five- and 10-year progression-free survival rates were 58% and 44%, respectively. Multivariate analysis showed that extent of resection, adjuvant radiation therapy, and absence of rhinopharynx invasion were positive independent predictors of overall survival. The latter 2 variables and a younger patient age were positive independent predictors of progression-free survival. Twenty-one patients showed 1p36 LOH. All events of recurrence and death clustered in the group of patients with 1p36 LOH; however, this biological marker was not statistically significant on multivariate analysis. CONCLUSIONS Resection is the treatment of choice in primary and recurrent SBC. Patient age, rhinopharynx invasion at diagnosis, extent of tumor removal, and postoperative radiation therapy influence SBC prognosis. Genetic analysis, even while showing interesting results, did not reveal 1p36 LOH as an independent predictor of clinical outcome.

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Use of Salvage Surgery or Stereotactic Radiosurgery for Multiply Recurrent Skull Base Chordomas: A Single-Institution Experience and Review of the Literature

Journal of Neurological Surgery Part B Skull Base ◽

10.1055/s-0039-3402019 ◽

2020 ◽

Author(s):

Stella K. Yoo ◽

Ben A. Strickland ◽

Gabriel Zada ◽

Shelly X. Bian ◽

Adam Garsa ◽

...

Keyword(s):

Overall Survival ◽

Skull Base ◽

Stereotactic Radiosurgery ◽

Salvage Surgery ◽

Medical Center ◽

Academic Medical Center ◽

Univariate Analysis ◽

Recurrence Rates ◽

Tertiary Center ◽

Skull Base Chordomas

Abstract Introduction Chordomas are locally destructive neoplasms characterized by appreciable recurrence rates after initial multimodality treatment. We examined the outcome of salvage treatment in recurrent/progressive skull base chordomas. Methods This is a retrospective review of recurrent/progressive skull base chordomas at a tertiary urban academic medical center. The outcomes evaluated were overall survival, progression-free survival (PFS), and incidence of new toxicity. Results Eighteen consecutive patients who underwent ≥1 course of treatment (35.3% salvage surgery, 23.5% salvage radiation, and 41.2% both) were included. The median follow-up was 98.6 months (range 16–215 months). After initial treatment, the median PFS was 17.7 months (95% confidence interval [CI]: 4.9–22.6 months). Following initial therapy, age ≥ 40 had improved PFS on univariate analysis (p = 0.03). All patients had local recurrence, with 15 undergoing salvage surgical resections and 16 undergoing salvage radiation treatments (mostly stereotactic radiosurgery [SRS]). The median PFS was 59.2 months (95% CI: 4.0–99.3 months) after salvage surgery, 58.4 months (95% CI: 25.9–195 months) after salvage radiation, and 58.4 months (95% CI: 25.9.0–98.4 months) combined. Overall survival for the total cohort was 98.7% ± 1.7% at 2 years and 92.8% ± 5.5% at 5 years. Salvage treatments were well-tolerated with two patients (11%) reporting tinnitus and one patient each (6%) reporting headaches, visual field deficits, hearing loss, anosmia, dysphagia, or memory loss. Conclusion Refractory skull base chordomas present a challenging treatment dilemma. Repeat surgical resection or SRS seems to provide adequate salvage therapy that is well-tolerated when treated at a tertiary center offering multimodality care.

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