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北京大学学报（医学版） ›› 2022, Vol. 54 ›› Issue (1): 54-61. doi: 10.19723/j.issn.1671-167X.2022.01.009

• 论著 • 上一篇    下一篇

105例口腔颅颌面部纤维性结构不良的临床病理分析

薛江1,张建运1,时瑞瑞2,谢晓艳3,白嘉英1,李铁军1,△()   

  1. 1.北京大学口腔医学院·口腔医院 口腔病理科, 北京 100081
    2.北京大学口腔医学院·口腔医院 中心实验室, 北京 100081
    3.北京大学口腔医学院·口腔医院 口腔颌面医学影像科,国家口腔医学中心,国家口腔疾病临床医学研究中心,口腔数字化医疗技术和材料国家工程实验室,口腔数字医学北京市重点实验室, 北京 100081
  • 收稿日期:2021-09-03 出版日期:2022-02-18 发布日期:2022-02-21
  • 通讯作者: 李铁军 E-mail:litiejun22@vip.sina.com
  • 基金资助:
    中国医学科学院口腔颌面部肿瘤精准病理诊断创新单元(2019RU034);北京大学口腔医学院教育教学研究项目(YS030120)

Clinicopathological analysis of 105 patients with fibrous dysplasia of cranio-maxillofacial region

XUE Jiang1,ZHANG Jian-yun1,SHI Rui-rui2,XIE Xiao-yan3,BAI Jia-ying1,LI Tie-jun1,△()   

  1. 1. Department of Oral Pathology, Peking University School and Hospital of Stomatology, Beijing 100081, China
    2. Central Laboratory, Peking University School and Hospital of Stomatology, Beijing 100081, China
    3. Department of Oral and Maxillofacial Radiology, Peking University School and Hospital of Stomatology & National Center of Stomatology & National Clinical Research Center for Oral Diseases & National Engineering Laboratory for Digital and Material Technology of Stomatology & Beijing Key Laboratory of Digital Stomatology, Beijing 100081, China
  • Received:2021-09-03 Online:2022-02-18 Published:2022-02-21
  • Contact: Tie-jun LI E-mail:litiejun22@vip.sina.com
  • Supported by:
    Research Unit of Precision Pathologic Diagnosis in Tumors of the Oral and Maxillofacial Regions, Chinese Academy of Medical Sciences(2019RU034);Education and Teaching Research Project Fund of Peking University School of Stomatology(YS030120)

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摘要：

目的: 比较不同类型口腔颅颌面部纤维性结构不良(fibrous dysplasia,FD)的临床病理特点,为临床诊治及预后判断提供依据。方法: 收集2013年1月至2020年12月于北京大学口腔医院确诊为FD或McCune-Albright综合征(McCune-Albright syndrome, MAS)的患者105例,分析其临床资料、影像学和病理学特点,分别归类为单骨型、多骨型、MAS型及口腔颅颌面部特有的口腔颅面型纤维性结构不良(craniofacial fibrous dysplasia,CFD)四型,对各型的临床病理特征、治疗及随访资料等进行分析。结果: 105例患者的男女比为1 ∶1.3,发病年龄0~56岁(中位数为12岁)。本组病例中,单骨型43例(40.95%), 其中上颌骨29例,下颌骨12例,颧骨2例;多骨型32例(30.48%);MAS型7例(6.67%);发生在颅上颌部位的FD,经影像学分析确诊CFD型23例(21.90%)。CFD在患者性别、术前血清碱性磷酸酶水平等方面有别于其他类型的FD。病理学上,各型FD的形态特点类似,但多骨型及MAS型患者可能出现以纤维成分增生为主的特征。结论: 口腔颅颌面部FD的临床和病理特点有别于身体其他部位发生的FD病变,CFD的临床和病理特点与发生于颅颌面部的单骨型或多骨型FD也有显著差异,应注意区分,以进一步明确CFD在临床处置及预后等方面的特殊性。

关键词: 骨纤维发育不良, 面骨, 病理学, 临床, 碱性磷酸酶

Abstract:

Objective: To compare the clinicopathologic features and prognosis of the different types of fibrous dysplasia (FD) of cranio-maxillofacial region, so as to provide a new reference for clinicians to treat these patients and make prognostic judgement. Methods: Clinical records, radiographic data and pathological information of 105 patients diagnosed with FD or McCune-Albright syndrome (MAS) at the Department of Oral Pathology, Peking University Hospital of Stomatology from January 2013 to December 2020 were collected. The patients were divided into 4 groups: monostotic FDs, polyostotic FDs, MAS and a specific type called craniofacial fibrous dysplasia (CFD) limited in the craniofacial region. The clinicopathological characteristics, treatment and follow-up data of each type were analyzed. Results: Of all the 105 patients, 46 were males and 59 were females, with a male-to-female ratio of 1 ∶1.3. The onset age ranged from 0 to 56 years and the median age was 12 years. On the basis of different involvement conditions, 4 types were divided. The most common type was monostotic FDs (43 cases, 40.95%), including maxilla (29 cases), mandibular (12 cases) and zygoma (2 cases). 32 cases (30.48%) were diagnosed with polyostotic FDs, 7 cases (6.67%) were MAS, and 23 cases (21.90%) were CFDs confirmed by computed tomography (CT) analysis. CFD was clearly distinct from other types of FD, such as the patient gender and the serum alkaline phosphatase level in peripheral blood before operative surgery. The pathologic findings of various types FD were quite similar, whilst the predominant fibrous tissue hyperplasia could be observed in polyostotic FDs and MAS types. Conclusion: The clinicopathologic features of FD in the cranio-maxillofacial region are different from the FD lesions in other parts of the body. The clinicopathological features of CFD are significantly different from those of monostotic and polyostotic FDs in the cranio-maxillofacial region. Therefore, the clinicians should pay attention to distinguish CFD in clinic, imaging and pathology aspects, so as to further clarify its features in clinic management and prognosis.

Key words: Fibrous dysplasia of bone, Facial bones, Pathology, clinical, Alkaline phosphatase

中图分类号: 

  • R739.8

图1

McCune-Albright 综合征1例"

表1

各型FD患者的临床及随访资料"

Items MFD (n=43) PFD (n=32) MAS (n=7) CFD (n=23) Total (n=105)
Onset age/years 15.00 (13.00, 30.00) 9.50 (5.50, 12.75) 8.57±2.37 10.00 (6.00, 14.00) 12.00 (8.00, 17.00)
Onset age group/years
≤10 7 (16.28) 21 (65.63) 5 (71.43) 13 (56.52) 46 (43.81)
11-20 23 (53.49) 7 (21.88) 2 (28.57) 7 (30.43) 39 (37.14)
21-30 4 (9.30) 2 (6.25) 0 (0) 2 (8.70) 8 (7.62)
31-40 6 (13.95) 1 (3.13) 0 (0) 1 (4.35) 8 (7.62)
41-50 1 (2.32) 1 (3.13) 0 (0) 0 (0) 2 (1.90)
51-60 2 (4.65) 0 (0) 0 (0) 0 (0) 2 (1.90)
Gender
Male 20 (46.51) 12 (37.50) 1 (14.29) 13 (56.52) 46 (43.81)
Female 23 (53.49) 20 (62.50) 6 (85.71) 10 (43.48) 59 (56.19)
Clinical sign
Facial deformity 39 (90.70) 32 (100.00) 7 (100.00) 23 (100.00) 101 (96.19)
Pain 8 (18.60) 2 (6.25) 1 (14.29) 1 (4.35) 12 (11.43)
Treatment
Conservative surgery 41 (95.35) 30 (93.75) 6 (85.71) 21 (91.30) 98 (93.33)
Radical surgery with reconstruction 2 (4.65) 2 (6.25) 1 (14.29) 2 (8.70) 7 (6.67)
Number of conservative operations 1.0 (1.0, 1.5) 2.0 (1.0, 3.0) 2.0 (1.0, 3.5) 1.0 (1.0, 1.5) 1.0 (1.0, 2.0)
Course 8.78±7.86 17.09±10.27 18.86±9.56 10.39±6.77 12.34±9.34
Serum ALP concentration
Elevation 5 (11.63) 22 (68.75) 6 (85.71) 11 (47.83) 44 (41.90)
Non-elevation 36 (83.72) 10 (31.25) 1 (14.29) 11 (47.83) 58 (55.24)
NA 2 (4.65) 0 (0) 0 (0) 1 (4.35) 3 (2.86)
Follow-up
No regrowth 28 (65.12) 18 (56.25) 3 (42.86) 16 (69.57) 65 (61.90)
Slow regrowth 3 (6.98) 3 (9.38) 1 (14.29) 1 (4.35) 8 (7.62)
Marked regrowth 2 (4.65) 3 (9.38) 0 (0) 2 (8.70) 7 (6.67)
Death 0 (0) 0 (0) 1 (14.29) 0 (0) 1 (0.95)
Loss to follow-up 10 (23.26) 8 (25.00) 2 (28.57) 4 (17.39) 24 (22.86)

图2

各型FD患者的年龄、性别及临床特征"

图3

FD的影像学特征"

表2

CFD患者的临床及随访资料"

No. Gender Last treatment Onset age/
years		 Number of the
operation		 Age at
operation/
years		 Age at
follow-up/
years		 Bone involved Current status
1 Male Conservative surgery 11 1 19 20 Left zygoma and left sphenoid bone Loss to follow-up
2 Female Conservative surgery 6 1 16 17 Left maxilla, left ethmoid bone, left nasal bone, left inferior nasal concha, left temporal bone, left parietal bone, bilateral frontal bone and bilateral sphenoid bone No regrowth
3 Female Conservative surgery 23 1 26 28 Left sphenoid bone, left ethmoid bone, left nasal bone, left temporal bone, bilateral frontal bone No regrowth
4 Male Conservative surgery 11 1 17 20 Bilateral occipital bone, bilateral frontal bone, right temporal bone, right maxilla, right sphenoid bone, right zygoma, right ethmoid bone Marked regrowth
5 Female Conservative surgery 10 2* 10, 17 20 Right maxilla, right sphenoid bone, right zygoma, right temporal bone No regrowth
6 Female Extended resection with
flap reconstruction		 6 2& 9, 10 13 Right maxilla, right sphenoid bone Loss to follow-up
7 Female Conservative surgery 16 2* 16, 31 33 Left maxilla, left sphenoid bone No regrowth
8 Female Conservative surgery 11 1 18 21 Left maxilla, left sphenoid bone, left zygoma, left ethmoid bone, left occipital bone, left frontal bone No regrowth
9 Female Conservative surgery 35 1 35 38 Left maxilla, left sphenoid bone, left zygoma, left ethmoid bone, left occipital bone, left frontal bone No regrowth
10 Female Conservative surgery 10 1 25 28 Right maxilla, right ethmoid bone, right zygoma Loss to follow-up
11 Male Conservative surgery 6 1 7 11 Right maxilla, right sphenoid bone, right temporal bone No regrowth
12 Male Conservative surgery 13 1 43 47 Right maxilla, right zygoma No regrowth
13 Male Conservative surgery 9 1 19 23 Left maxilla, left sphenoid bone, left zygoma Slow regrowth
14 Male Conservative surgery 15 1 21 25 Right maxilla, right sphenoid bone No regrowth
15 Female Conservative surgery 9 1 15 18 Left maxilla, left sphenoid bone, left zygoma No regrowth
16 Male Conservative surgery 14 1 21 25 Left maxilla, left sphenoid bone, left temporal bone No regrowth
17 Male Conservative surgery 4 2* 14, 18 22 Right maxilla, right sphenoid bone, right zygoma Loss to follow-up
18 Male Conservative surgery 6 2* 12, 23 27 Left maxilla, left sphenoid bone, left temporal bone Marked regrowth
19 Male Conservative surgery 7 2* 7, 18 22 Right maxilla, right sphenoid bone, right zygoma, right temporal bone, right ethmoid bone No regrowth
20 Male Extended resection with
flap reconstruction		 22 4# 22, 25,
40, 47		 53 Left zygoma, bilateral maxilla No regrowth
21 Male Conservative surgery 9 1 22 27 Left maxilla, left sphenoid bone, left zygoma No regrowth
22 Female Conservative surgery 8 1 22 28 Left maxilla, left zygoma No regrowth
23 Male Conservative surgery 4 1 19 27 Right maxilla, right sphenoid bone, right zygoma, right temporal bone No regrowth

图4

FD的病理学特征"

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ISSN 1671-167X
CN 11-4691/R
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