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Journal of Peking University (Health Sciences) ›› 2022, Vol. 54 ›› Issue (1): 54-61. doi: 10.19723/j.issn.1671-167X.2022.01.009

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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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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

CLC Number: 

  • R739.8

Figure 1

A case of McCune-Albright syndrome A and B, a typical Café-au-lait skin pigmentation on the back of the neck (A), and lower back (B) of a 37-year-old woman with McCune-Albright syndrome which demonstrates jagged “coast of Maine” borders; C-E, CT images demonstrate a ground-glass ill-defined pattern in the cranio-maxillofacial region; F, bilateral clavicle and scapula are involved with chest deformity; G and H, bilateral tibia and femur are involved with bone deformity."

Table 1

Clinical characteristics and follow-up data of the different types of FD patients"

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)

Figure 2

The age, gender and clinical characteristics of the different types of FD patients A, the differential onset age distribution of four types of FD; B, CFD was more present in men than in women; C, the median onset age of CFD was significantly lower than MFD; D, the proportion of CFD patients with elevated serum ALP before operation was significantly higher than MFD; E, the average course of CFD was significantly shorter than PFD; F, the median number of operations of CFD was significantly less than PFD. * P<0.05, ** P<0.01, *** P<0.001. Abbreviations as in Table 1."

Figure 3

Radiologic characteristics of FD Single bone was involved in monostotic fibrous dysplasia in A (left mandible) and C (right maxilla), and clear zygomaticomaxillary suture was shown in C and D (white arrow). Multiple bones were involved in B (right mandible and left maxilla). The right maxilla and zygoma were involved crossing the zygomaticomaxillary suture (white arrow) in CFD (D, case 5 in Table 2), while the bone sutures were indistinct in E (case 20 in Table 2). FD, fibrous dysplasia; CFD, craniofacial fibrous dysplasia."

Table 2

Clinical characteristics and follow-up data of CFD patients"

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

Figure 4

Histopathologic characteristics of FD A, the lesion is ill-defined in continuity with normal bone (HE ×40); B, typical histological features of FD show thin, irregular, and disconnected trabeculae and fibrous tissue replaces the normal bone structures (HE ×40, HE ×400 in the upper right box); C, the predominant of fibrous tissue (HE ×40); D, the low expression of Ki-67 in FD lesions (HE ×400). FD, fibrous dysplasia."

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