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纤维软骨发育异常:我们目前了解多少?

Fibrocartilaginous dysplasia: What do we know so far?

作者信息

Alshalan Asma, Asiri Yasser, AlGarni Ayed, Tayara Bader

机构信息

Department of Radiology, King Fahad Specialist Hospital, Ammar Bin Thabit St.. Al Merikbat, Dammam 32253, Saudi Arabia.

Department of Pathology, King Fahad Specialist Hospital, Dammam, Saudi Arabia.

出版信息

Radiol Case Rep. 2023 Feb 28;18(5):1763-1766. doi: 10.1016/j.radcr.2023.01.074. eCollection 2023 May.

DOI:10.1016/j.radcr.2023.01.074
PMID:36926535
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10011050/
Abstract

Fibrocartilaginous dysplasia has been described as a rare variant of fibrous dysplasia. This lesion will appear in imaging as ground glass matrix similar to fibrous dysplasia, but it will also show rings and arcs calcifications. In turn, this can lead to misdiagnosing fibrocartilaginous dysplasia as primary cartilaginous lesion such as enchondroma or chondrosarcoma, neccesating histopathological confirmation. We report a case of fibrocartilaginous dysplasia in a 19 years old male with polyostotic fibrous dysplasia with prior pathologic fracture of the left femur. The patient presented with progressive swelling of the left thigh, imaging was done and showed enlargement of the fibrous dysplasia in the left femur with new rings and arcs matrix mineralization. The lesion was biopsied and microscopic evaluation revealed mainly cartilage islands with fibro-osseous tissue. We also discuss the possible origin of the cartilaginous component in this lesion, and its clinical course.

摘要

纤维软骨发育异常已被描述为骨纤维发育不良的一种罕见变体。该病变在影像学上表现为类似于骨纤维发育不良的磨玻璃样基质,但也会出现环状和弧形钙化。反过来,这可能导致将纤维软骨发育异常误诊为原发性软骨病变,如内生软骨瘤或软骨肉瘤,因此需要组织病理学确诊。我们报告一例19岁男性的纤维软骨发育异常病例,该患者患有多骨性骨纤维发育不良,之前左股骨发生过病理性骨折。患者表现为左大腿进行性肿胀,进行了影像学检查,结果显示左股骨的骨纤维发育不良增大,出现了新的环状和弧形基质矿化。对病变进行了活检,显微镜评估显示主要为软骨岛伴纤维骨组织。我们还讨论了该病变中软骨成分的可能起源及其临床病程。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1720/10011050/5f6aaf879ea1/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1720/10011050/d5fdf30d0b5a/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1720/10011050/cce59344c6a9/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1720/10011050/a92aaf084351/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1720/10011050/5f6aaf879ea1/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1720/10011050/d5fdf30d0b5a/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1720/10011050/cce59344c6a9/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1720/10011050/a92aaf084351/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1720/10011050/5f6aaf879ea1/gr4.jpg

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本文引用的文献

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Fibrous Dysplasia with Massive Cartilaginous Differentiation (Fibrocartilaginous Dysplasia) in the Proximal Femur: A Case Report and Review of the Literature.股骨近端伴有大量软骨分化的骨纤维发育不良(纤维软骨发育不良):一例报告并文献复习
Case Rep Oncol. 2016 Feb 19;9(1):126-33. doi: 10.1159/000443476. eCollection 2016 Jan-Apr.
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