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Malignant transformation of craniofacial fibrous dysplasia: a systematic review of overall survival

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Abstract

The incidence of malignant transformation of fibrous dysplasia (FD) is very rare. Thus, the available knowledge of its characteristics, management, and survival is scarce. Here, we present a systemic review of fibrous dysplasia that had undergone malignant transformation. A comprehensive search was performed on PubMed electronic database. The survival rates and hazard ratios of age, gender, past history of previous radiotherapy, type of FD, and treatment were collected from the published articles and analyzed. Forty-eight cases were eligible for inclusion in the study. Patient’s age, gender, past history radiotherapy, and type of FD did not influence the overall survival (OS). The Kaplan Meier analysis showed that the patients who had not received any treatment had poor prognosis with a median survival of 4 months. The patients that received surgery had significantly longer OS than that in the biopsy group. The prognosis of malignant transformation of FD is relatively poor, and surgery is the optimal treatment of choice. Nevertheless, the efficacy of postoperative adjuvant therapy to patient OS is still undefined.

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Authors and Affiliations

  1. Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Tiantan Xili No. 6, Beijing, 100050, China

    Zhi Li & Haiyan Qian

  2. Beijing Neurosurgical Institute, Capital Medical University, Beijing, China

    Raynald

  3. Department of Pathology, Huadian People’s Hospital, Jilin, China

    Zijuan Wang

Authors
  1. Zhi Li
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  2. Raynald
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  3. Zijuan Wang
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  4. Haiyan Qian
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Correspondence to Zhi Li.

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All authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge, or beliefs) in the subject matter or materials discussed in this manuscript.

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This study was approved by the ethics committee of Beijing Tiantan Hospital, Capital Medical University, Beijing, China. All procedures were performed in accordance with the ethical standards of the institutional and/or national research committee and the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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This study was approved by the Institutional Review Board of Beijing Tiantan Hospital, Capital Medical University. For this type of study, formal consent is not required.

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Li, Z., Raynald, Wang, Z. et al. Malignant transformation of craniofacial fibrous dysplasia: a systematic review of overall survival. Neurosurg Rev 43, 911–921 (2020). https://doi.org/10.1007/s10143-019-01089-1

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  • DOI: https://doi.org/10.1007/s10143-019-01089-1

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