Penn David L, Tartarini Richard J, Glass Carolyn H, De Girolami Umberto, Zamani Amir A, Dunn Ian F
Department of Neurological Surgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Surg Neurol Int. 2017 Sep 6;8:209. doi: 10.4103/sni.sni_7_17. eCollection 2017.
Fibrous dysplasia (FD) is a rare developmental disease characterized by the replacement of bone marrow with proliferating fibro-osseous tissue. There exist three forms of FD-monostotic, polyostotic, and that associated with McCune-Albright syndrome. The disease can present in different locations and with a variety of symptoms. One of the more common locations of FD occurrence is the craniofacial region. Treatment of asymptomatic FD often involves conservative management with serial imaging. Medical management with bisphosphonates is an option, though long-term efficacy data are lacking. Surgical resection is usually reserved for very large or symptomatic lesions.
We discuss the most unusual case of a 52-year-old male found to have a left pterional mass while being worked up for sinus headaches. The patient elected to follow this lesion conservatively, and imaging several years later showed obvious growth which accelerated in the last 4 years during an 18-year observational period. He ultimately underwent successful resection of an extradural and intradural FD.
The significant growth potential of these lesions was revealed in this patient, in whom conservative management had been adopted. Despite optimal surgical resection and outcome in this case, the importance of surveillance imaging and perhaps earlier intervention cannot be underestimated when managing cranial FD.
骨纤维异常增殖症（FD）是一种罕见的发育性疾病，其特征是骨髓被增生的纤维骨组织所取代。FD有三种形式——单骨型、多骨型以及与McCune-Albright综合征相关的类型。该疾病可出现在不同部位并伴有多种症状。FD较常见的发病部位之一是颅面部区域。无症状FD的治疗通常包括采用系列影像学检查进行保守处理。使用双膦酸盐进行药物治疗是一种选择，不过缺乏长期疗效数据。手术切除通常仅用于非常大的或有症状的病变。
我们讨论了一名52岁男性的极其罕见的病例，该患者在因鼻窦头痛接受检查时被发现有左侧翼点肿块。患者选择对该病变进行保守观察，在18年的观察期内，数年后的影像学检查显示肿块有明显生长，且在过去4年中生长加速。他最终成功接受了硬膜外和硬膜内FD切除术。
该患者采用了保守治疗，但其病变显示出显著的生长潜能。尽管此病例手术切除效果理想，但在处理颅骨FD时，监测影像学检查的重要性以及或许更早进行干预的重要性不可低估。
