PDF(Pubmed)
Abstract：
OBJECTIVE: Fibrous dysplasia (FD) is a rare genetic disease with benign bone tumors. FD can affect one (monostotic FD) or multiple bones (polyostotic FD), with craniofacial lesions being common. Because of its rarity, there are only few clinical reports on FD in the head and neck region and its clinical characteristics remain incompletely defined. This study aimed to determine patient demographics, symptoms, diagnostics, and given treatment in patients with FD of the head and neck in a Finnish population.
METHODS: A retrospective review on all patients diagnosed with or treated for FD of the head and neck at the Helsinki University Hospital during 2005-2020.
RESULTS: In total 74 patients were identified; 54% were male and the mean age 45 years. Overall 95% had monostotic FD. Mandibula and maxilla were the most common anatomic sites. Majority of patients had symptoms, most commonly pain and lesion growth, and 49% had extra-skeletal symptoms. For all, diagnosis was primarily based on imaging findings, biopsies were obtained from 41%. Altogether 54 patients (73%) were managed by observation only, 20 patients (27%) received treatment; ten bisphosphonates, six surgery and four both.
CONCLUSIONS: Although highly variable in its clinical manifestations, head and neck FD lesions are often symptomatic and impose risk for extra-skeletal complications. Treatment is often conservative but should be individually tailored. Future studies are encouraged to better define the disease characteristics and hopefully offer new treatment possibilities.
METHODS: A retrospective review on all patients diagnosed with or treated for FD of the head and neck at the Helsinki University Hospital during 2005-2020.
RESULTS: In total 74 patients were identified; 54% were male and the mean age 45 years. Overall 95% had monostotic FD. Mandibula and maxilla were the most common anatomic sites. Majority of patients had symptoms, most commonly pain and lesion growth, and 49% had extra-skeletal symptoms. For all, diagnosis was primarily based on imaging findings, biopsies were obtained from 41%. Altogether 54 patients (73%) were managed by observation only, 20 patients (27%) received treatment; ten bisphosphonates, six surgery and four both.
CONCLUSIONS: Although highly variable in its clinical manifestations, head and neck FD lesions are often symptomatic and impose risk for extra-skeletal complications. Treatment is often conservative but should be individually tailored. Future studies are encouraged to better define the disease characteristics and hopefully offer new treatment possibilities.
摘要：
目标：纤维发育不良（FD）是一种罕见的遗传性良性骨肿瘤。FD可以影响一个（单骨FD）或多个骨（多骨FD），颅面病变很常见。因为它的稀有性,关于头颈部FD的临床报道很少，其临床特征仍未完全确定。这项研究旨在确定患者的人口统计学，症状,诊断,并对芬兰人群的头颈部FD患者进行治疗。
方法：对2005-2020年在赫尔辛基大学医院诊断或治疗头颈部FD的所有患者进行回顾性回顾。
结果：共确定了74例患者；54％为男性，平均年龄45岁。总体上95%患有单核型FD。下颌骨和上颌骨是最常见的解剖部位。大多数病人都有症状,最常见的疼痛和病变生长，49%有骨骼外症状。对于所有人,诊断主要基于影像学发现，活检来自41%.总共54名患者（73％）仅通过观察进行治疗，20名患者(27%)接受治疗；10名双磷酸盐，六个手术和四个都是。
结论：尽管其临床表现差异很大，头颈部FD病变通常是有症状的，并且存在骨骼外并发症的风险。治疗通常是保守的，但应该单独定制。鼓励未来的研究更好地定义疾病特征，并希望提供新的治疗可能性。
方法：对2005-2020年在赫尔辛基大学医院诊断或治疗头颈部FD的所有患者进行回顾性回顾。
结果：共确定了74例患者；54％为男性，平均年龄45岁。总体上95%患有单核型FD。下颌骨和上颌骨是最常见的解剖部位。大多数病人都有症状,最常见的疼痛和病变生长，49%有骨骼外症状。对于所有人,诊断主要基于影像学发现，活检来自41%.总共54名患者（73％）仅通过观察进行治疗，20名患者(27%)接受治疗；10名双磷酸盐，六个手术和四个都是。
结论：尽管其临床表现差异很大，头颈部FD病变通常是有症状的，并且存在骨骼外并发症的风险。治疗通常是保守的，但应该单独定制。鼓励未来的研究更好地定义疾病特征，并希望提供新的治疗可能性。
