Spontaneous Malignant Transformation in Craniomaxillofacial Fibrous Dysplasia
Spontaneous Malignant Transformation in Craniomaxillofacial Fibrous Dysplasia
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DOI：
10.1097/scs.0b013e3182646126
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发表时间：
2013-01-01
影响因子：
0.9
通讯作者：
Shen, Guofang
中科院分区：
文献类型：
--
作者：
Cheng, Jie;Yu, Hongbo;Shen, Guofang
颅颌面骨纤维异常增殖症（FD）的自发性恶变是非常罕见的，其临床病理特征仍然很大程度上未知。在这里，我们的目的是通过提供来自中国三级转诊医院的数据和回顾中英文文献来描述既存FD引起的恶性肿瘤的流行病学和临床病理学特征。检索上海市第九人民医院颅颌面疾病登记中心1993年1月至2010年12月诊断为颅颌面FD恶变患者的相关信息。进行英文和中文文献综述，检索过去20年发表的相关病例，并预设纳入标准。对所有纳入病例的相关临床和病理变量进行进一步分析。在我们的机构中发现了3例因既往颅颌面FD而发生骨肉瘤的病例，并通过文献回顾确定了其他35例病例。这些不常见的实体通常发生在平均年龄为39.8岁的成年人和平等的性别优势。上颌骨仍然是最常见的恶性肿瘤的网站，其次是下颌骨和胶质瘤。恶性肿瘤以骨肉瘤最多，其次为纤维肉瘤、软骨肉瘤及恶性纤维组织细胞瘤。单纯根治性切除或术后放疗/化疗仍是主要治疗手段，但局部复发和远处转移多，预后不良。总之，我们的研究结果可能首次提供有关颅颌面FD恶性肿瘤的流行病学，临床病理特征，治疗和预后的全面信息。进一步的调查是必要的，以提高早期诊断和适当的治疗这些罕见的实体。
Spontaneous malignant transformation in craniomaxillofacial fibrous dysplasia (FD) is extremely rare and its clinicopathological characteristics remain largely unknown. Here, we aimed to characterize the epidemiology and clinicopathological features of malignancies arising from preexisting FD by presenting data from a Chinese tertiary referral hospital and review of English and Chinese literatures. The craniomaxillofacial disease registry of Shanghai Ninth People's Hospital was searched and reviewed to collect relevant information for patients diagnosed as malignant transformation in craniomaxillofacial FD between January 1993 and December 2010. An English and Chinese literature review was conducted to retrieve pertinent cases published in the past 2 decades with preset inclusion criteria. All included cases were further analyzed with regard to associated clinical and pathological variables. Three cases with osteosarcoma arising from previous craniomaxillofacial FD were found at our institution and 35 other cases were identified by literature review. These uncommon entities usually occurred in adults with a mean age of 39.8 years and equal gender preponderance. Maxilla remained the most common sites for malignancies followed by mandible and zygoma. Most malignancies were diagnosed as osteosarcoma followed by fibrosarcoma, chondrosarcoma, and malignant fibrous histiocytoma. Radical resection alone or with postoperative radiotherapy/chemotherapy remains the main treatment strategy with unfavorable prognosis due to local recurrence and distant metastasis. Taken together, our findings might for the first time provide the comprehensive information regarding the epidemiology, clinicopathological features, treatment, and prognosis of malignancies in craniomaxillofacial FD. Further investigations are warranted to improve early diagnosis and proper treatment for these uncommon entities.