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单骨型纤维结构不良与头颈部孤立性肌内黏液瘤:马扎布罗德综合征的一种独特表现及文献综述

Monosotic fibrous dysplasia and solitary intramuscular myxoma of the head and neck: A unique presentation of Mazabraud's syndrome and a literature review.

作者信息

Fu Shuiting, Tian Zhuowei, Zhang Chenping, He Yue

机构信息

Department of Oral and Maxillofacial Head and Neck Surgery, Shanghai 9th People's Hospital, Shanghai Jiao-Tong University School of Medicine, Shanghai 200011, P.R. China.

出版信息

Oncol Lett. 2015 Nov;10(5):3087-3094. doi: 10.3892/ol.2015.3633. Epub 2015 Aug 25.

DOI:10.3892/ol.2015.3633
PMID:26722294
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4665340/
Abstract

Mazabraud's syndrome (MS) is a rare disease that is a combination of fibrous dysplasia and intramuscular myxomas. MS is a benign lesion and there is little data on the disease due to its low incidence. In the present study, the case of a 38-year-old patient who presented with a soft-tissue mass involving the masseter and swelling at the mandibular body and mandibular ramus is reported. Since the mandible is an important aesthetic and functional organ in the oral and maxillofacial region, surgery was primarily aimed at resecting the tumor, with good safety margins, and reconstructing the resultant defect. The lesions were pathologically diagnosed as MS. The unique features of this case included the painless and monostotic fibrous dysplasia, the solitary intramuscular myxomas involving the jaw and the male gender of the patient. MS usually occurs in the lower extremities, with an unusual predilection for the right limb; however, it rarely occurs in the head and neck region. A retrospective analysis of the clinical features and management of MS was also performed in the present study, together with a literature review. From the literature, it was concluded that the incidence of MS is ~2.3-fold greater in female patients than in male patients, and that the age of onset of MS ranges between 17 and 82 years, with an average age of 46.25 years.

摘要

马扎布劳德综合征(MS)是一种罕见疾病,它是骨纤维发育不良和肌内黏液瘤的组合。MS是一种良性病变,由于其发病率低,关于该疾病的数据很少。在本研究中,报告了一名38岁患者的病例,该患者表现为累及咬肌的软组织肿块以及下颌体和下颌支肿胀。由于下颌骨是口腔颌面部重要的美学和功能器官,手术主要旨在切除肿瘤并保留足够的安全切缘,以及修复由此产生的缺损。病变经病理诊断为MS。该病例的独特特征包括无痛性单骨型骨纤维发育不良、累及颌骨的孤立性肌内黏液瘤以及患者为男性。MS通常发生在下肢,尤其好发于右下肢;然而,它很少发生在头颈部区域。本研究还对MS的临床特征和治疗进行了回顾性分析,并进行了文献综述。从文献中得出的结论是,MS在女性患者中的发病率比男性患者高约2.3倍,MS的发病年龄在17至82岁之间,平均年龄为46.25岁。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b595/4665340/5136dc698169/ol-10-05-3087-g02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b595/4665340/243d9b67af3c/ol-10-05-3087-g00.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b595/4665340/c29abefd7a01/ol-10-05-3087-g01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b595/4665340/5136dc698169/ol-10-05-3087-g02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b595/4665340/243d9b67af3c/ol-10-05-3087-g00.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b595/4665340/c29abefd7a01/ol-10-05-3087-g01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b595/4665340/5136dc698169/ol-10-05-3087-g02.jpg

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