国际口腔医学杂志

国际口腔医学杂志 ›› 2023, Vol. 50 ›› Issue (4): 445-451.doi: 10.7518/gjkq.2023051

• 病例报告 • 上一篇    下一篇

颌骨Gorham-Stout病1例及文献复习

吴一凡(),卢浩,刘胜文,徐万林(),杨雯君   

  1. 上海交通大学医学院附属第九人民医院口腔颌面-头颈肿瘤科 上海交通大学口腔医学院国家口腔医学中心 国家口腔疾病临床医学研究中心 上海市口腔医学重点实验室 上海 200011
  • 收稿日期:2022-11-29 修回日期:2023-04-06 出版日期:2023-07-01 发布日期:2023-06-21
  • 通讯作者: 徐万林
  • 作者简介:吴一凡，住院医师，硕士，Email：Asher0713@163.com
  • 基金资助:
    上海市青年科技英才扬帆计划(21YF1423500);上海第九人民医院生物样本库项目(YBKB202214)

Gorham-Stout disease in maxillofacial bone: a case report and literature review

Wu Yifan(),Lu Hao,Liu Shengwen,Xu Wanlin(),Yang Wenjun.   

  1. Dept. of Oral and Maxillofacial-Head and Neck Oncology, Shanghai Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine, College of Stomatology, Shanghai Jiao Tong University, National Center for Stomatology, National Clinical Research Center for Oral Diseases, Shanghai Key Laboratory of Stomatology, Shanghai 200011, China
  • Received:2022-11-29 Revised:2023-04-06 Online:2023-07-01 Published:2023-06-21
  • Contact: Wanlin Xu
  • Supported by:
    Shanghai Sailing Program(21YF1423500);the Biobank Project of Shanghai Ninth People’s Hospital(YBKB202214)

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摘要：

Gorham-Stout病（GSD）又名大块骨溶解症或消失性骨病（VBD），是一种以淋巴组织及血管增生为主要病理特点的自发性、进行性的骨吸收疾病。该病没有年龄、性别及种族差异，可累及全身单个或多个骨骼，罕发于颌面部。目前，GSD的发病原因尚存争议，其治疗方案及疗效的评价仍未达成统一的标准。本文通过报道1例上海交通大学医学院附属第九人民医院口腔颌面-头颈肿瘤科收治的骨溶解症病例，结合国内外相关文献，对GSD的病因、临床病理特点、诊断要点及治疗作一综述，以进一步提高对发生于颌骨的GSD的认识。

关键词: Gorham-Stout病, 骨溶解症, 消失性骨病, 颌面骨

Abstract:

Gorham-Stout disease (GSD), also known as massive osteolysis or vanishing bone disease (VBD), is a spontaneous, progressive disease of bone resorption characterized by lymphoid tissue and vascular hyperplasia. GSD does not differ in age, sex, or race and can involve single or multiple bones, but rarely occurs in the maxillofacial region. However, its cause remains controversial, and there is still no unified standard for the evaluation of treatment options and efficacy. In this paper, we report a case of GSD with recurrence in the Department of Oral and Maxillofacial-Head and Neck Onco-logy, Shanghai Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine. In addition, we briefly review the etiology, clinicopathological features, diagnostic points, and treatment of GSD to further enhance our understan-ding of GSD that occurs in the maxillofacial bone.

Key words: Gorham-Stout disease, massive osteolysis, vanishing bone disease, maxillofacial bone

中图分类号: 

  • R 782

图 1

术前影像学照A：全景片示右侧下颌骨体部及升支处骨吸收显著；B：CT横断位显示右侧下颌骨骨质吸收破坏；C：CT冠状位显示右下颌骨骨质缺失；D：CT三维重建显示右下颌骨体部及升支骨质吸收；E：CT三维重建显示左侧下颌骨骨质完整，未见明显骨缺失。"

图 2

组织学HE染色见血管增生 光学显微镜 × 100A：骨组织内血管增生，黄色箭头示增生的血管；黑色箭头示骨；星号示骨髓腔内的神经组织； B：软组织内血管增生，黄色箭头示增生的血管。"

图 3

术后随访全景片"

表 1

文献报道的颌面部GSD汇总"

时间/年参考文献

年龄/岁

（性别）

部位组织学表现治疗随访时间/预后
2015[12]29（女）下颌骨致密纤维组织，血管左下颌骨节段性切除术6个月/无进展
2015[13]18（男）下颌骨致密胶原纤维组织手术+二期修复2.5年/无进展
2016[14]15（女）下颌骨、上颌骨、颧骨血管淋巴样增生，炎细胞浸润化学治疗（顺铂+5-氟尿嘧啶）10年/无进展
2016[15]

7（男）

8（男）

39（男）

颅颌面多骨

颞骨

颅颌面多骨

———
2016[16]6（女）下颌骨浆细胞淋巴细胞浸润观察2年/无进展
2016[17]54（女）上颌骨———
2017[18]14（女）下颌骨血管瘤样血管增生——
2017[19]8（女）下颌骨血管内皮细胞增生观察—
2017[20]29（男）下颌骨血管瘤样血管增生地诺单抗—
2017[21]7（女）下颌骨纤维胶原，淋巴细胞浸润，血管增生放射治疗（共计40 Gy）9个月/无进展
2017[22]38（男）下颌骨—阿仑膦酸—
2017[23]13（男）下颌骨纤维组织及血管增生帕米膦酸二钠1年/进展
2017[24]38（男）下颌骨致密纤维组织及血管观察—
2018[25]

6（男），6（男）

9（女），7（男）

下颌骨，颞下颌关节窝—其中3例行双膦酸盐治疗无进展
2018[26]25（男）下颌骨血管瘤样病变阿仑膦酸钠（每周70 mg）14个月/进展
2018[27]51（男）颞骨、颞下颌关节—观察—
2018[28]20~51/2例（男），2例（女）2例下颌骨，1例颌骨和颞骨，1例下颌骨、颞骨和颧骨—阿仑膦酸钠（每周70 mg）—/无进展
2018[29]14（男）下颌骨—干扰素和类固醇6个月/进展
2019[30]34（男），16（男）下颌骨—下颌骨节段性切除术+游离腓骨瓣重建—/进展
2020[31]3（男）下颌骨骨吸收伴淋巴细胞浸润干扰素、唑来膦酸盐、雷帕霉素4年/进展
2020[32]61（女）下颌骨致密结缔组织及扩张血管增生下颌骨病损切除术4个月/无进展
2021[3]60（男）上颌骨慢性炎细胞浸润加巴喷丁6个月/无进展
2021[33]—（女）下颌骨胶原纤维、炎细胞、血管增生——
2022[34]3（女）颞骨炎细胞浸润、血管扩张增生——
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