Monostotic Fibrous Dysplasia of the Lumbar Spine With Secondary Features of Solid Variant Aneurysmal Bone Cyst

IF 1.9 Q3 PATHOLOGY Clinical Pathology Pub Date : 2019-07-01 DOI:10.1177/2632010X19861109
N. Maloney, K. Linos
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引用次数: 2

Abstract

Fibrous dysplasia is a benign, mass-forming disease of bone composed of abnormal fibrous and osseous elements that can be accompanied by endocrine dysfunction, skin pigmentation, and intramuscular myxomas. It is usually encountered as a solitary lesion in the tibia or femur but can develop in any bone and can be unifocal or multifocal. Difficulty arises when a solitary lesion is identified in an uncommon site or when there are prominent secondary changes, such as aneurysmal bone cyst (ABC). Molecular studies are available as an adjunct to histomorphology to aid distinction from other entities. GNAS mutations, present in greater than 70% of fibrous dysplasia cases, help in the distinction from primary ABC and low-grade osteosarcoma, which exhibit different molecular abnormalities. We report a case of monostotic fibrous dysplasia in a lumbar vertebral body with secondary change consisting of the solid variant of ABC.
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以实体变异性动脉瘤性骨囊肿为次要特征的腰椎单一性纤维发育不良
纤维发育不良是一种由异常纤维和骨元素组成的良性骨块形成疾病,可伴有内分泌功能障碍、皮肤色素沉着和肌内粘液瘤。它通常是胫骨或股骨的孤立性病变,但可以在任何骨骼中发展,可以是单灶性或多灶性。当在不常见的部位发现孤立性病变或有明显的继发性变化时,如动脉瘤样骨囊肿(ABC),就会出现困难。分子研究可以作为组织形态学的辅助手段来帮助区分其他实体。GNAS突变存在于70%以上的纤维发育不良病例中,有助于区分原发性ABC和低度骨肉瘤,后者表现出不同的分子异常。我们报告了一例腰椎单发性纤维发育不良,继发性改变由ABC的实体变体组成。
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来源期刊
Clinical Pathology
Clinical Pathology PATHOLOGY-
CiteScore
2.20
自引率
7.70%
发文量
66
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