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Australian Dental Journal 1998;43:(6):390-4

Fibrous dysplasia of the jaw bone: A review of 15 new


cases and two cases of recurrence in Jamaica together
with a case report
C. Ogunsalu, BDS(Ib), MSc(Lond), FRACDS*
N. J. D. Smith, BDS, MSc, MPhil, DDRRCR
A. Lewis, BchD, FDS, RCS(Eng&Edin), LDS, MBA

Abstract seven cases involving the maxilla and two cases


The authors reviewed 15 new cases of fibrous involving the mandible. In the present series of 15
dysplasia of the jaw bone and two cases of new cases in Jamaica, ten cases involved the maxilla
recurrence seen in Jamaica between 1980 and and five involved the mandible.
1995. Only cases which had a histological
confirmation of fibrous dysplasia were included. The diagnosis of fibrous dysplasia is often made in
The clinical behaviour and radiological findings of infancy and childhood. The maxilla or mandible
these cases were studied from the case files, either may be involved but a predominance of the maxilla
at the Cornwall Regional Hospital or the Kingston has been documented.2 Males are less often affected
Public Hospital in Jamaica.
than females. The deformity of the jaw results from
Key words: Fibrous dysplasia, atypical clinical a progressively slow-growing painless swelling, but
presentations, case report. growth often slows or become arrested at a time
(Received for publication November 1996. Accepted coinciding with the onset of puberty.2
December 1996.)

Materials and methods


Introduction All cases histologically confirmed to be fibrous
Fibrous dysplasia is a fairly common localized dysplasia were studied for findings such as age of the
misdifferentiation of the bone-forming mesenchyme patient, sex, radiographic appearance and clinical
affecting a single or many bones in the skeletal behaviour of the tumour (fibrous dysplasia). This
system. Skeletal aberration represents the cardinal study was conducted by using the information
feature but certain endocrinopathies, abnormal documented for each patient in the case files at both
pigmentation of the skin and mucous membrane, the Cornwall Regional Hospital and Kingston
and occasionally other abnormalities form part of Public Hospital in Jamaica. Based on the existing
the entire disease process. When the finding of legislation3 on the retention of medical records in
fibrous dysplasia coincides with the finding of Jamaica, records were only readily available from
abnormal pigmentation of the skin, the condition is 1980 to April 1995, a period of 15 years. No records
called McCune-Albright syndrome. pertaining to a patient were recorded twice, and
Monostotic fibrous dysplasia, though less serious cases of recurrence were recorded separately. A total
than polyostotic fibrous dysplasia, is of greater of 15 new cases of fibrous dysplasia and two cases of
concern to the dentist because of the frequency in recurrence were found.
which the jaws are affected. In a series of 67 cases of
monostotic fibrous dysplasia, Schlumberger1 found Results
The present series of 15 new cases and two cases
of recurrent fibrous dysplasia of the jaw bones is the
*Oral and Maxillofacial Surgeon, Cornwall Dental Centre, Montego
Bay, Jamaica. first to be documented in Jamaica. Between 1980
Head of Department of Dental Radiology, Dental Institute, Kings and April 1995, 15 new cases of monostotic fibrous
College Medical and Dental School, United Kingdom.
Consultant and Head of Department of Oral and Maxillofacial dysplasia and two cases of recurrent fibrous
Surgery, Kingston Public Hospital, Jamaica. dysplasia of the jaw bone were recorded. The cases
390 Australian Dental Journal 1998;43:6.
Table 1. Summary of cases of fibro-osseous
lesions
Fibro-osseous lesions No of cases %
Monostotic fibrous dysplasia 15 47.0
Ossifying fibroma 10 31.2
Gigantiform cementoma 3 9.4
Recurrent fibrous dysplasia 2 6.2
Cementoma (cementoblastoma) 1 3.1
Cemental dysplasia 1 3.1
Cementifying fibroma 0
Total number of fibro-osseous lesions 32 100

10
2

Fig. 2. Extra-oral appearance of a patient (Case 14, Table 2) with


fibrous dysplasia.
15

K Monostotic fibrous dysplasia; K Ossifying fibroma;


K Gigantiform cementoma; K Recurrent fibrous dysplasia D;
K Cementoblastoma; K Cemental dysplasia.
Fig. 1. Pie chart showing the distribution of fibro-osseous lesions in
Jamaica (1990 to 1995).

of new monostotic fibrous dysplasia and recurrent


fibrous dysplasia accounted for 5.2 per cent and
0.68 per cent respectively of jaw bone tumours in
Jamaica. Fibrous dysplasia (excluding recurrent
fibrous dysplasia) accounted for 47 per cent of all
cases of fibro-osseous lesions (WHO classification)
seen in Jamaica during this period (Table 1, Fig. 1).
Recurrent fibrous dysplasia accounted for 6.2 per
Fig. 3. Intra-oral appearance of the patient shown in Fig. 2.
cent of all cases of fibro-osseous lesions during this
period.
years (Table 2) with a mean age of 25.7 years. Of
Clinical features these, five (33.3 per cent) were 20 years old and
The presenting symptom in all cases was an below, and 12 (80 per cent) were 30 years old and
increasing swelling of the jaw bone that either below. Two cases were patients aged 47 years at the
interfered with function or was disfiguring (Fig. 2, time of presentation, which has been previously
3). Case 14 (Table 2) presented with a painful, recorded as the average age at presentation. Figure 4
suppurative swelling with ulceration, which is very shows the age distribution of fibrous dysplasia in
atypical of fibrous dysplasia (Fig. 3). Jamaica. The male:female ratio of the patients was
For the new cases of monostotic fibrous dysplasia, 2:3. Five lesions were located in the mandible and
the patients were between the ages of 10 and 47 ten in the maxilla, thus giving a site ratio of 1:2 for
Australian Dental Journal 1998;43:6. 391
Table 2. Summary of cases of fibrous dysplasia in Jamaica
Case Sex Age (years) Site Radiographic appearance
1 F 16 Ant maxilla (R) Not located
2 F 27 Ant maxilla (R) Orange peel
3 F 23 Ant maxilla Orange peel
4 F 29 Ant and post mandible (R) Radiolucent lesion between 48 and 44
5 F 47 Ant and post mandible (R) Evidence of excessive bone production*
6 M 25 Ramus (post) mandible (L) Varying degree of opacification
7 M 10 Post mandible (R) Orange peel
8 F 31 Ant maxilla (R) Not located
9 M 16 Post maxilla (L) Ground glass appearance
10 F 47 Post mandible (R) Radiolucent lesion
11 F 11 Post mandible (R) Ground glass appearance
12 M 23 Ant and post maxilla (R) Large opacification of maxilla
13 F 28 Ant and post maxilla (R) Opacification of lower part of R antrum
14 M 34 Post maxilla (R) Radiopacity of R antrum
15 M 20 Post maxilla (L) Radiopacity of the L antrum
*Particularly involving the alveolar process.

8- 4. Varying degree of opacification (not ground


K Female glass and not orange peel).
K Male Figures 5 and 6 show the radiographic appearance
6- of two cases of fibrous dysplasia.
Surprisingly, Cases 4 and 10, female patients aged
29 and 47 respectively, presented as radiolucent
4- lesions of the mandible, thus signifying early lesions
of fibrous dysplasia in patients over the age of 25
years.
2-
Recurrent fibrous dysplasia
Two separate cases of recurrent fibrous dysplasia
were also documented during this period (Table 3).
0-
Because so few attempts at total removal of the
10-19 20-29 30-39 40-49
lesion have been reported (for obvious reasons), any
Age band (years)
discussion of recurrence rate of fibrous dysplasia
Fig. 4. Age and sex distribution of 15 cases of fibrous dysplasia in
Jamaica (1990-1995). may not be practicable.

mandible and maxilla respectively. Table 2 shows the Case repor t


exact location of the tumours as recorded in the A 34 year old male, otherwise healthy looking,
patients case files. All cases for the mandible were presented at the Maxillofacial Clinic of the Cornwall
located in the posterior mandible while, interestingly, Regional Hospital in Jamaica with a rapidly growing,
Case 6 involved the ascending ramus of the left painful swelling of the right maxilla of two months
mandible. Four cases were located in the anterior duration. Epiphoria, nasal discharge and anaesthesia
maxilla, three cases in the posterior maxilla, while in the distribution of the infra-orbital nerve on the
three cases involved both the posterior and anterior right side of the jaw were associated findings of
maxilla. clinical significance.
Extra-oral examination revealed a bony, hard
Radiologic appearance swelling of the right maxilla, extending from the
The radiographic appearance of fibrous dysplasia right infra-orbital region above, to the area just
in Jamaica is shown in the appropriate column in above the right angle of the mouth below, and
Table 2. The radiographs or radiographic report for extending to the zygomatic region. The nasolabial
Cases 1 and 8 could not be located, hence only 13 fold on this side had been obliterated (Fig. 2).
radiographic reports were available. For simplicity, it Intra-oral examination revealed an extensive,
was decided to categorize the radiographic ulcerated lesion extending from the distal aspect of
appearance as follows: 13 to the region of 18. The lesion measured 50 mm
1. Orange peel/ground glass appearance. at its largest diameter. Ulceration and suppuration
2. Opacification of the antrum. of this swelling were the confusing clinical findings
3. Radiolucent lesion of the mandible. (Fig. 3).
392 Australian Dental Journal 1998;43:6.
Fig. 5. Occipitomental view radiograph of a patient (Case 14, Table Fig. 6. Occlusal radiograph of the maxilla of a 31 year old black
2) with fibrous dysplasia. Note complete opacification of the right Jamaican female showing what may be called ground glass appearance
maxillary antrum. of the palate on the left side.

The gross intra-oral appearance was disfiguring The excision of this lesion was followed by
and masticatory activity and phonation were recurrence to the original size in less than six months
impaired. The clinical impression was that of a (Case 1, Table 3). For a 34 year old patient with
malignant neoplasm such as a fibrosarcoma or fibrous dysplasia, recurrence following excision of
carcinoma of the maxillary antrum. the lesion is not expected, as has always been
An occipitomental radiograph revealed complete previously reported.
opacification of the right maxillary antrum (Fig. 5),
suggestive of a space-occupying lesion in the right Discussion
maxillary sinus. Because of the similarity between fibrous
Histologically, the gross appearance of the dysplasia and ossifying fibroma at a histological
submitted specimen consisted of five irregular level, the diagnosis of each of these fibro-osseous
portions of firm, tan tissue, some of which were lesions continues to be a problem. For this reason
partially covered by mucosa. On sectioning, each one can correctly say that the X-ray studies are
revealed a firm, cream cut surface. indispensable for the interpretation of fibro-osseous
Sections showed a portion of a lesion consisting of lesions of the jaw, and more particularly in the
irregularly shaped spicules of woven bone dispersed differentiation between fibrous dysplasia and ossifying
in a background of compact, collagenous fibrous fibroma, which is virtually impossible on the basis of
tissue (Fig. 7a, b). Osteoblastic rimming of the bony histopathology alone.
spicules was not evident. Some sections were Monostotic fibrous dysplasia is characterized by a
partially covered by stratified squamous, non- swelling resulting from a poorly circumscribed area
keratinizing epithelium, which was focally ulcerated of fibro-osseous proliferation. Prior to 1970, fibrous
and associated with areas of haemorrhage and a dysplasia was used as an all-inclusive term that
heavy chronic inflammatory cell infiltrate within the encompassed both monostotic and polyostotic
subjacent fibrous stroma. The overall histologic forms of fibrous dysplasia and a variety of other
features were those of fibrous dysplasia with fibro-osseous lesions, notably ossifying fibroma,
ulceration and infection. fibrous osteoma and osteoblastoma.4 Recently, the

Table 3. Summary of cases of recurrent fibrous dysplasia in Jamaica


Case Sex Age (years) Site Radiographic appearance
1 M 35 Post maxilla (R) Radiopacity of the R antrum
2 F 13 Post mandible (R) Ground glass appearance of the mandible in the molar region

Australian Dental Journal 1998;43:6. 393


a b
Fig. 7. a, b, Photomicrographs (380 and 3500 respectively) showing irregularly shaped spicules of woven bone dispersed on a background of
compact, collagenous fibrous tissues.

term fibro-osseous lesion has been frequently used There is clinical evidence which indicates that local
for disorders ranging from fibrous dysplasia to the infection or trauma may eventuate in the disease
circumscribed lesions of ossifying or cementifying under yet unrecognized conditions,1 and this may be
fibroma and cemental dysplasia. More recently, the the cause in Case 14. Despite the similarity in
WHO classification of fibro-osseous lesion has nomenclature and histologic appearance, the mono-
recognized these conditions as distinct entities. stotic fibrous dysplasia is not necessarily related to
Monostotic fibrous dysplasia starts in childhood the polyostatic form and the former does not
but typically undergoes increasing ossification and necessarily progress into the latter.
subsequent arrest in adulthood. It is clinically seen
mainly in young persons, usually in their 20s, as a Conclusion
painless, smoothly rounded swelling, usually in the The clinicopathological aspect of the 15 new cases
maxilla. In this study of 15 new cases in Jamaica, of fibrous dysplasia in this series differs from what
this was found not to be so, as the majority (10 has been previously documented. It is also very
cases) presented initially after the age of 20 years surprising to find recurrence at the age of 34 years.
and, more surprisingly, two cases presented at the For these reasons, there is a need for further study of
age of 47 years. The authors are very certain that new cases in Jamaica.
pain from a rapidly growing swelling which was not
previously present (confirmed by the referring References
dentist) was the presenting complaint of Case 14 1. Schlumberger HG. Fibrous dysplasia (ossifying fibroma) of the
who was seen in late 1994. Because of the atypical maxilla and mandible. Am J Orthodont Oral Surg 1946;32:579-87.
clinical behaviour of fibrous dysplasia in Jamaica, as 2. Neville BW, Damn DD, Allen CM, Bunquot JE. Oral and
maxillofacial pathology. 1st edn. Philadelphia: WB Saunders,
documented by findings from this series of 15 new 1995:460-9.
cases, further study on the clinicopathologic aspect 3. Ogunsalu CO. A study of tumours of the jaw bones in Jamaica with
of the disease is necessary. special emphasis on radiology. London: University of London,
1995. MSc thesis.
The mean age of occurrence in the 69 patients 4. Lynch MA, Brightman VJ, Greenberg MS, eds. Burkets oral
reported by Zimmerman and his associates5 was 27 medicine: diagnosis and treatment. 8th edn. Philadelphia:
years, while in the 53 patients with craniofacial Lippincott, 1984:327-9.
fibrous dysplasia reported by Gardner and Halpert, 5. Zimmerman DC, Dahlin DC, Stafne EC. Fibrous dysplasia of the
mandible and maxilla. Oral Surg Oral Med Oral Pathol
the mean age was 34 years,6 both of which are higher 1958;11:55-68.
than the mean age of 25.7 years for 15 patients 6. Gardner AF, Halpert L. Fibrous dysplasia of the skull with special
reported in the present study. reference to the oral regions. D Practitioner D Record
1963;13:337-40.
The aetiology of monostotic fibrous dysplasia is
unknown. Although a variety of possible factors has
been suggested, none has found general acceptance. Address for correspondence/reprints:
Mr Christopher Ogunsalu,
Cornwall Dental Centre,
The word new has been used for the 15 cases of fibrous dysplasia to 42 Market Street,
distinguish them from the two cases of recurrent fibrous dysplasia also
recorded. Montego Bay, Jamaica.

394 Australian Dental Journal 1998;43:6.

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