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伴有继发性骨囊肿的骨纤维发育不良——1例罕见病例报告及文献复习

Fibrous dysplasia with secondary aneurysmal bone cyst-a rare case report and literature review.

作者信息

Rau Levy Hermes, Reinheimer Angélica, Meurer Maria Inês, Marodin Aline Luiza, Espezim Catherine Schmitz, Klüppel Leandro Eduardo, Dos Santos Vaz Fernandes Paula Cristina, Rivero Elena Riet Correa

机构信息

Department of Maxillofacial Surgery, Joana de Gusmão Children's Hospital, Florianópolis, SC, Brazil.

Department of Implant Dentistry, Latin American Institute of Dental Research and Education, Curitiba, PR, Brazil.

出版信息

Oral Maxillofac Surg. 2019 Mar;23(1):101-107. doi: 10.1007/s10006-019-00741-w. Epub 2019 Feb 13.

Abstract

INTRODUCTION

Fibrous dysplasia (FD) and aneurysmal bone cyst (ABC) are uncommon non-neoplastic intraosseous lesions, and the occurrence of concomitant FD and ABC is extremely rare.

CASE REPORT

An 11-year-old boy presented with gradual progressive enlargement of his right zygomatic bone over 4 years prior to presentation. Computed tomography revealed a lesion with a central bony area showing a ground-glass appearance surrounded by a well-defined expansile lesion with internal septations. An incisional biopsy was performed and suggested a fibro-osseous lesion. Resection was performed, followed by immediate reconstruction using autogenous bone graft from the iliac crest. Histopathological examination revealed irregularly shaped trabeculae comprising immature woven bone in a fibroblastic cell-rich stroma. Blood-filled sinusoidal spaces lined by fibrous septa containing scattered multinucleated giant cells were observed peripherally. These findings were compatible with concomitant FD and ABC. The patient was disease-free at the time of his 10-month follow-up.

CONCLUSIONS

Based on our literature search, this report is the first to describe concomitant monostotic FD and ABC in the zygomatic bone. Accurate diagnosis requires careful investigation and examination of clinical, radiographical, and histopathological features. The treatment of choice should provide an esthetic and functional improvement in the patient.

摘要

引言

骨纤维异常增殖症(FD)和骨动脉瘤样囊肿(ABC)是罕见的非肿瘤性骨内病变,FD与ABC同时发生极为罕见。

病例报告

一名11岁男孩在就诊前4年出现右侧颧骨逐渐进行性增大。计算机断层扫描显示一个病变,中央骨质区域呈磨玻璃样外观,周围是边界清晰的膨胀性病变,内部有分隔。进行了切开活检,提示为纤维骨性病变。实施了切除手术,随后立即用取自髂嵴的自体骨移植进行重建。组织病理学检查显示,在富含成纤维细胞的基质中有不规则形状的小梁,由不成熟的编织骨组成。在周边观察到由含有散在多核巨细胞的纤维间隔内衬的充满血液的窦状间隙。这些发现符合FD与ABC同时存在。在10个月的随访时,该患者无疾病复发。

结论

根据我们的文献检索,本报告首次描述了颧骨同时发生单骨型FD和ABC。准确诊断需要仔细研究和检查临床、影像学和组织病理学特征。治疗选择应能改善患者的美观和功能。

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