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Treatment strategy and long-term outcomes of primary intracranial rhabdomyosarcoma: a single-institution experience and systematic review.
J Neurosurg 2019; :1-11JN

Abstract

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/).

Authors+Show Affiliations

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

Pub Type(s)

Journal Article

Language

eng

PubMed ID

31518985

Citation

Zheng, Jing-Jie, et al. "Treatment Strategy and Long-term Outcomes of Primary Intracranial Rhabdomyosarcoma: a Single-institution Experience and Systematic Review." Journal of Neurosurgery, 2019, pp. 1-11.
Zheng JJ, Zhang GJ, Huo XL, et al. Treatment strategy and long-term outcomes of primary intracranial rhabdomyosarcoma: a single-institution experience and systematic review. J Neurosurg. 2019.
Zheng, J. J., Zhang, G. J., Huo, X. L., Wang, L., Hao, S. Y., Zhang, L. W., ... Li, D. (2019). Treatment strategy and long-term outcomes of primary intracranial rhabdomyosarcoma: a single-institution experience and systematic review. Journal of Neurosurgery, pp. 1-11. doi:10.3171/2019.6.JNS19752.
Zheng JJ, et al. Treatment Strategy and Long-term Outcomes of Primary Intracranial Rhabdomyosarcoma: a Single-institution Experience and Systematic Review. J Neurosurg. 2019 Sep 13;1-11. PubMed PMID: 31518985.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Treatment strategy and long-term outcomes of primary intracranial rhabdomyosarcoma: a single-institution experience and systematic review. AU - Zheng,Jing-Jie, AU - Zhang,Gui-Jun, AU - Huo,Xu-Lei, AU - Wang,Liang, AU - Hao,Shu-Yu, AU - Zhang,Li-Wei, AU - Wu,Zhen, AU - Wu,Yu-Mei, AU - Zhang,Jun-Ting, AU - Li,Da, Y1 - 2019/09/13/ PY - 2019/03/16/received PY - 2019/06/11/accepted PY - 2019/9/14/entrez PY - 2019/9/14/pubmed PY - 2019/9/14/medline KW - CI = confidence interval KW - CN = cranial nerve KW - CPA = cerebellopontine angle KW - CTA = CT angiography KW - DMFS = distant metastasis-free survival KW - EOR = extent of resection KW - GCS = Glasgow Coma Scale KW - GTR = gross-total resection KW - HR = hazard ratio KW - KPS = Karnofsky Performance Scale KW - LR-PFS = local-regional PFS KW - MRA = MR angiography KW - OS = overall survival KW - PFS = progression-free survival KW - PIRMS = primary intracranial RMS KW - PR = partial resection KW - RMS = rhabdomyosarcoma KW - SEER = Surveillance, Epidemiology, and End Results KW - STR = subtotal resection KW - VAC = vincristine, actinomycin D, and cyclophosphamide KW - VICE = vincristine, ifosfamide, carboplatin, and etoposide KW - chemotherapy KW - long-term outcomes KW - oncology KW - overall survival KW - primary intracranial rhabdomyosarcoma KW - treatment strategy SP - 1 EP - 11 JF - Journal of neurosurgery JO - J. Neurosurg. N2 - 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/). SN - 1933-0693 UR - https://www.unboundmedicine.com/medline/citation/31518985/Treatment_strategy_and_long-term_outcomes_of_primary_intracranial_rhabdomyosarcoma:_a_single-institution_experience_and_systematic_review L2 - https://thejns.org/doi/10.3171/2019.6.JNS19752 DB - PRIME DP - Unbound Medicine ER -