Treatment strategy and long-term outcomes of primary intracranial rhabdomyosarcoma: a single-institution experience and systematic review

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  • 1 Department of Gynecologic Oncology, Beijing Obstetrics and Gynecology Hospital, Capital Medical University; and
  • 2 Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China
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OBJECTIVE

Primary intracranial rhabdomyosarcoma (PIRMS) is rare, and the effects of the treatment strategy on overall survival (OS) are unclear. This study aimed to evaluate risk factors pertinent to OS and to propose an optimal treatment strategy.

METHODS

Clinical data of patients with PIRMS treated at Beijing Tiantan Hospital and from the English-language literature between 1946 and 2018 were reviewed. A literature review was performed via Ovid, MEDLINE, Embase, PubMed, Web of Science, and Cochrane databases using the terms “rhabdomyosarcoma,” “intracranial,” “cerebral,” and “brain.” Previously published data were processed and used according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.

RESULTS

There were 8 males (66.7%) and 4 females with PIRMS at our institution, with a mean age of 24.3 years. Gross-total resection was achieved in 4 patients (33.3%), and adjuvant radiation and chemotherapy were administered in 5 (45.5%) and 3 (27.3%) patients, respectively. After a mean follow-up period of 13.7 months, all patients developed local-regional recurrence and died of the disease. Twenty-nine cases (14 female and 15 male) were reported in the literature with a median age of 9.0 years. After a mean follow-up duration of 18.6 months, 13 patients (44.8%) developed recurrences, 7 patients (24.1%) had extracranial metastasis, and 14 patients (48.3%) died. In the pooled cases, adjuvant radiation (hazard ratio [HR] 0.089, 95% confidence interval [CI] 0.027–0.288, p < 0.001) and age < 10 years (HR 0.227, 95% CI 0.077–0.666, p = 0.007) were independent predictors of good local-regional progression-free survival (LR-PFS). Adjuvant radiation therapy (HR 0.301, 95% CI 0.110–0.828, p = 0.020) and age < 10 years (HR 0.359, 95% CI 0.131–0.983, p = 0.046) were significant predictors for favorable OS in the multivariate model.

CONCLUSIONS

Due to the rarity of the disease, a poor outcome of PIRMS was demonstrated based on the pooled cohort. Use of radiation was associated with improved outcomes and should be considered to improve OS/LR-PFS. Further study is required to identify the optimal treatment regimen.

Systematic review no.: CRD42019121249 (crd.york.ac.uk/PROSPERO/)

ABBREVIATIONS CI = confidence interval; CN = cranial nerve; CPA = cerebellopontine angle; CTA = CT angiography; DMFS = distant metastasis-free survival; EOR = extent of resection; GCS = Glasgow Coma Scale; GTR = gross-total resection; HR = hazard ratio; KPS = Karnofsky Performance Scale; LR-PFS = local-regional PFS; MRA = MR angiography; OS = overall survival; PFS = progression-free survival; PIRMS = primary intracranial RMS; PR = partial resection; RMS = rhabdomyosarcoma; SEER = Surveillance, Epidemiology, and End Results; STR = subtotal resection; VAC = vincristine, actinomycin D, and cyclophosphamide; VICE = vincristine, ifosfamide, carboplatin, and etoposide.

Supplementary Materials

    • pdf Supplementary Tables 1 and 2 (PDF 461 KB)

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Contributor Notes

Correspondence Da Li: Beijing Tiantan Hospital, Capital Medical University, Beijing, China. lida@bjtth.org.

INCLUDE WHEN CITING Published online September 13, 2019; DOI: 10.3171/2019.6.JNS19752.

J.J.Z. and G.J.Z. contributed equally to this work.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

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