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口腔颌面部 Mazabraud 综合征:一例报告及文献复习。

Maxillofacial Mazabraud's syndrome: A case report & review.

机构信息

Universidad de Chile's Bucco-Maxillofacial Surgery and Traumatology Department, Sergio Roberto Livingstone Polhammer 943, Independencia, Metropolitan Region, Chile.

Private Practice, Padre Mariano 391, Providencia, Metropolitan Region, Chile.

出版信息

J Stomatol Oral Maxillofac Surg. 2018 Feb;119(1):44-48. doi: 10.1016/j.jormas.2017.10.002. Epub 2017 Oct 10.

Abstract

Mazabraud's syndrome is characterized by myxomas of intramuscular type present in association with fibrous dysplasia. Up to this day, approximately 80 cases of Mazabraud's syndrome have been reported, although in the head and neck territory intramuscular myxoma reports in association with fibrous dysplasia of the bone are very scarce. An unusual case of Mazabraud's syndrome in a 63 years old female displaying fibrous dysplasia of the mandible and soft tissue myxoma in the edentulous alveolar ridge in the molar area is reported. After four years of follow-up, the clinical, imagenological and microscopical findings that led to the diagnosis and treatment are discussed. This report exemplifies the diagnostic and treatment challenge of this rare disease and enhances our clinical knowledge due to its long follow-up, highlighting the need of understanding better its behavior in order to establish proper guidelines for its treatment.

摘要

马扎布劳德综合征的特征是存在肌肉内黏液瘤,同时伴有纤维结构不良。迄今为止,已经报道了大约 80 例马扎布劳德综合征病例,尽管在头颈部,与骨纤维结构不良相关的肌肉内黏液瘤非常罕见。本文报告了一例罕见的马扎布劳德综合征病例,患者为 63 岁女性,表现为下颌骨纤维结构不良和磨牙区无牙牙槽嵴软组织黏液瘤。经过四年的随访,讨论了导致诊断和治疗的临床、影像学和显微镜检查结果。本报告说明了这种罕见疾病的诊断和治疗挑战,并通过长期随访增加了我们的临床知识,强调了更好地了解其行为的必要性,以便为其治疗制定适当的指南。

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