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成年后持续生长22年的骨纤维异常增殖症:一例报告

Continued growth of locally aggressive fibrous dysplasia of 22 years duration after reaching adulthood: a case report.

作者信息

Yamagishi Yusuke, Okamoto Masanori, Yoshimura Yasuo, Kito Munehisa, Aoki Kaoru, Takahashi Jun

机构信息

Department of Orthopaedic Surgery, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano, 390-8621, Japan.

Department of Orthopaedic Surgery, Shinshu Ueda Medical Center, 1-27-21 Midorigaoka, Ueda, Nagano, 386-8610, Japan.

出版信息

J Surg Case Rep. 2020 Feb 11;2020(2):rjz406. doi: 10.1093/jscr/rjz406. eCollection 2020 Feb.

Abstract

Fibrous dysplasia generally stops growing when patients reach adulthood. Locally aggressive fibrous dysplasia is an extremely rare subtype of fibrous dysplasia that is characterized by progressive enlargement after bone maturation, cortical bone destruction and soft tissue invasion but without malignant transformation. At 50 years of age, a tumor was found in the rib of a patient. The tumor gradually enlarged over time and imaging findings suggested a malignant tumor. The case was further complicated by restrictive lung disorder. Biopsies from multiple sites showed no malignant findings, and marginal resection with partial curettage was performed. The final diagnosis was locally aggressive fibrous dysplasia, and the restrictive lung disorder improved postoperatively. The natural history of the disease is also unknown. This is the first report in the literature to describe a case in which a lesion exhibited long-term growth over a period of 22 years after reaching adulthood.

摘要

骨纤维异常增殖症通常在患者成年后停止生长。局部侵袭性骨纤维异常增殖症是骨纤维异常增殖症中一种极其罕见的亚型,其特征是在骨骼成熟后进行性增大、皮质骨破坏和软组织侵犯,但无恶变。一名50岁患者的肋骨发现了一个肿瘤。随着时间推移,肿瘤逐渐增大,影像学检查结果提示为恶性肿瘤。该病例还合并有限制性肺疾病。多处活检未发现恶性病变,遂行边缘切除加部分刮除术。最终诊断为局部侵袭性骨纤维异常增殖症,术后限制性肺疾病有所改善。该疾病的自然病程也尚不清楚。这是文献中首次报道的成年后病变持续生长22年的病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9472/7012086/11657cf4048d/rjz406f1.jpg

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