家族性巨大型牙骨质瘤患者各时期临床特点分析

中国口腔颌面外科杂志 ›› 2015, Vol. 13 ›› Issue (6): 534-538.

家族性巨大型牙骨质瘤患者各时期临床特点分析

王宏伟¹，杨嵘¹，贺光²，张陈平¹*，秦兴军¹*

1.上海交通大学医学院附属第九人民医院?口腔医学院口腔颌面-头颈肿瘤科，上海市口腔医学重点实验室，上海 200011；
2.上海交通大学 Bio-X研究院/遗传发育与精神疾病教育部重点实验室，上海 200030

收稿日期:2015-06-17 出版日期:2015-11-20 发布日期:2015-12-03
作者简介:王宏伟（1988-），女，住院医师，E-mail: xnngo-go@163.com
基金资助:
Supported by Scientific Research Projects of Shanghai Health and Family Planning Commission (SHFPC) (201440403) and 2014 - "Future Talent" Funding of Department of Oromaxillofacial Head and Neck Oncology, Shanghai Ninth People's Hospital.

Clinical features of patients with familial gigantiform cementoma

WANG Hong-wei¹, YANG Rong¹, HE Guang², ZHANG Chen-ping¹, QIN Xing-jun¹.

1.Department of Oromaxillofacial Head and Neck Oncology, Shanghai Ninth People’s Hospital, College of Stomatology, Shanghai Jiao Tong University School of Medicine;Shanghai Key Laboratory of Stomatology. Shanghai 200011;
2.Bio-X Institutes, Key Laboratory for the Genetics of Developmental and Neuropsy- chiatric Disorders Ministry of Education Shanghai Jiao Tong University. Shanghai 200030, China

Received:2015-06-17 Online:2015-11-20 Published:2015-12-03
Contact: 上海市卫计委2014年科研基金（201440403）；

摘要/Abstract

摘要： 目的：分析总结家族性巨大型牙骨质瘤患者各时期的临床特点。方法：采集既往发现家系中患者的病史，不同阶段的临床表现、影像学和血液学等检查资料，并予归纳。结果：① 发病初期患者开始出现颌骨病变但临床表现并不显著，影像学表现为X线透射的低密度灶，血清碱性磷酸酶略升高，骨密度较正常同龄人变化不明显；② 迅速增长期上、下颌骨病灶加速生长，患者颜面外观和口腔功能受到严重影响，可伴发长骨骨折，病灶表现为高、低密度混合影像，血清碱性磷酸酶水平显著升高，骨密度明显降低；③ 生长停滞期上、下颌骨病灶大小不再有明显变化，病灶几乎全部由X线阻射影像组成，血清碱性磷酸酶恢复至正常水平，骨密度逐渐升高，并最终达到正常水平。结论：家族性巨大型牙骨质瘤患者在其病情进展的各时期均表现出典型的临床特点。根据这些特点，可以判断疾病的进程，有利于疾病的诊断和治疗方案的制订。

关键词: 家族性巨大型牙骨质瘤, 临床时期, 临床特点

Abstract: PURPOSE : To summarize the clinical features of patients with familial gigantiform cementoma (FGC). METHODS : We performed a retrospective study in a series of 11 patients. The hematology indicators, as well as the clinical and radiographic findings in different stages were analyzed. RESULTS : During initial onset, the lesions involved in the patients' jaws. CT scans showed multiple radiolucent swellings in the mandible and maxilla. The level of blood alkaline phosphatase was raised slightly. The bone mineral content was almost within normal limits. During rapid expansion, a marked enlargement of the masses with severe facial deformity and malocclusion was present. Patients always sustained multiple pathological long bone fractures. CT demonstrated multiple, expansile, mixed radiolucent-radiopaque masses in all four quadrants of their jaws. Patients had raised blood alkaline phosphatase and the bone mineral content decreased in this stage. During growth suppression phase, the lesions showed no apparent sign of increased expansion. CT scans revealed large radiolucent swellings. The blood alkaline phosphatase returned to normal range and the bone mineral content increased. CONCLUSIONS : In different phases, patients with FGC have special clinical characteristics. These features are extremely helpful in assessing the growth potential of FGC, as well as in formulating the treatment strategies.

Key words: Familial gigantiform cementoma, Clinical phase, Clinical feature

中图分类号:

R739.8

王宏伟，杨嵘，贺光，张陈平*，秦兴军*. 家族性巨大型牙骨质瘤患者各时期临床特点分析[J]. 中国口腔颌面外科杂志, 2015, 13(6): 534-538.

WANG Hong-wei, YANG Rong, HE Guang, ZHANG Chen-ping, QIN Xing-jun.. Clinical features of patients with familial gigantiform cementoma[J]. China J Oral Maxillofac Surg, 2015, 13(6): 534-538.

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