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肋骨单发性骨纤维异常增殖症:一例报告

Monostotic fibrous dysplasia of the rib: a case report.

作者信息

Mahadevappa Asha, Patel Sapna, Ravishankar Sunila, Manjunath Gubbanna V

机构信息

Department of Pathology, JSS Medical College, JSS University, No. 1036, 5th Main 10th Cross, 1st Stage Vijayanagar, Karnataka, Mysore 570017, India.

出版信息

Case Rep Orthop. 2012;2012:690914. doi: 10.1155/2012/690914. Epub 2012 Dec 10.

Abstract

Fibrous dysplasia is a noninherited bone disease in which abnormal differentiation of osteoblasts leads to replacement of normal marrow and cancellous bone by immature bone with fibrous stroma. Monostotic fibrous dysplasia accounts for 28% in the ribs. It is often asymptomatic and incidentally detected on radiographs. As with many bone abnormalities, it can be superimposed by the formation of aneurysmal bone cysts. We report a case of a 70-year-old lady who presented with swelling on the chest wall of 20-ear duration and sudden increase in size for 8 months. Radiologically, X-ray and CT scan showed an expansible lesion of the medullary cavity with a ground-glass centre and thinning of cortex of the 5th rib. The resected lesion was a firm, well-defined solid, grey-white expansile mass replacing the medullary cavity. Histopathologically, benign fibrous spindle areas with disorganized irregular bony trabeculae were seen. Hemorrhagic spaces lined by osteoclast-like multinucleated giant cells were also noted. The diagnosis was fibrous dysplasia with aneurysmal bone cyst changes. Although fibrous dysplasia with aneurysmal bone cyst is rare, it should be taken into account in differential diagnosis of the rapidly growing solitary rib lesion.

摘要

骨纤维异常增殖症是一种非遗传性骨病,其中成骨细胞的异常分化导致正常骨髓和松质骨被带有纤维基质的不成熟骨所替代。单发性骨纤维异常增殖症在肋骨中占28%。它通常无症状,常在X线片上偶然发现。与许多骨异常情况一样,它可合并动脉瘤样骨囊肿形成。我们报告一例70岁女性,其胸壁肿胀20年,大小在8个月内突然增大。放射学检查方面,X线和CT扫描显示第5肋骨髓腔有一膨胀性病变,中心呈磨玻璃样,皮质变薄。切除的病变为质地硬、边界清晰的实性灰白色膨胀性肿块,取代了髓腔。组织病理学检查可见良性纤维梭形区域,伴有排列紊乱的不规则骨小梁。还可见由破骨细胞样多核巨细胞衬里的出血性腔隙。诊断为骨纤维异常增殖症伴动脉瘤样骨囊肿改变。虽然骨纤维异常增殖症伴动脉瘤样骨囊肿罕见,但在快速生长的孤立性肋骨病变的鉴别诊断中应予以考虑。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a0fc/3529877/f5e517763598/CRIM.ORTHOPEDICS2012-690914.001.jpg

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