Cystic degeneration in fibrous dysplasia of the jaws

A case report
Carlo Ferretti, BDS, FCD(SA),a Hedley Coleman, BDS, BChD(Hons), M Dent,b and Mario
Altini, BDS, M Dent,b Johannesburg, South Africa
UNIVERSITY OF THE WITWATERSRAND

A case of nonspecific cystic degeneration complicating fibrous dysplasia of the mandible is presented. This condition
is extremely rare in the jaw bones. The patient had a painless swelling of the right face measuring 10 cm in diameter, and
there was no history of trauma. Radiographs showed a poorly defined, ground glass radiopaque lesion; a central welldefined cyst was confirmed by means of computed tomography. During surgery, a large cystic cavity with surrounding, soft
fibrous bone that merged with the cortex was observed. Histologic examination showed a large nonepithelial-lined cystic
cavity with a surrounding fibro-osseous lesion, which was consistent with a diagnosis of fibrous dysplasia. (Oral Surg Oral

Med Oral Pathol Oral Radiol Endod 1999;88:337-42)

Nonepithelial-lined cysts occasionally occur in association with various benign and malignant bone lesions,
including fibrous dysplasia,1-8 giant cell tumor,2,8 chondroblastoma,2,8 ossifying fibroma,9,10 benign osteoblastoma,8,11 cemento-osseous dysplasia,12-15 fibrous histiocytoma,2 fibrosarcoma,2 and osteosarcoma.2 These
cysts vary in nature; some are aneurysmal bone
cysts,2,3,6,7,11,16 some are simple bone cysts,1,12-15 and
others are nonspecific cystic degenerations.4,5,17
In a review of 639 extragnathic bone tumors and
tumorlike lesions, the frequency of secondary
aneurysmal bone cyst varied from 2% in fibrosarcoma
and osteosarcoma to 14.6% in chondroblastoma and
giant cell tumors; in addition, nonspecific cysts were
found in many of the cases.2 A review of 66 cases of
aneurysmal bone cysts found that 32% occurred as
secondary phenomena in preexisting bone lesions.8
In the jaws this association has not been as well documented, but nonepithelial-lined cysts have been
described in association with fibrous dysplasia,3,6,9,16,18
ossifying fibroma,9,10 and, more frequently, cementoosseous dysplasia.13-15 The frequency of aneurysmal
bone cyst occurring in fibro-osseous jaw lesions has
been reported as 21%.3
The aim of this article is to report a case of fibrous
dysplasia of the mandible complicated by nonspecific
cystic degeneration.
CASE REPORT
The patient, a 12-year-old boy, complained of a painless
aDivision

of Maxillofacial and Oral Surgery, Department of Surgery.

of Oral Pathology, Department of Anatomical Pathology.
Received for publication Dec 17, 1998; returned for revision Jan 15,
1999; accepted for publication Apr 14, 1999.
Copyright 1999 by Mosby, Inc.
1079-2104/99/$8.00 + 0 7/14/99710
bDivision

lump on the right side of his face. The mass had first been
noted approximately 1 year earlier and was slowly increasing
in size. Medical examination was noncontributory, and there
was no history of trauma. Extraoral examination showed a
diffuse swelling approximately 10 cm in diameter centered
on the right angle of the mandible (Fig 1). On palpation the
mass was bony-hard and nontender, and the margins of the
lesion blended imperceptibly with the surrounding bone. The
overlying skin was normal in color, texture, and temperature,
and there was no motor nor sensory deficit. Mouth opening
was limited to 10 mm interincisally, but no mandibular deviation was noted (Fig 1).
Intraoral examination revealed a normal dentition and occlusion with expansion of the buccal cortex, anterior border of the
ramus, right coronoid process, and lingual cortical plate.
Obliteration of the buccal sulcus opposite the mandibular right
first molar was evident. Palpation of the anterior border of the
ramus elicited fluctuation and egg-shell crackling. The overlying mucosa was normal in color and texture.
Radiographic examination of the jaws showed a poorly
defined, ground-glass radiopaque lesion in the right ramus
and posterior body of the mandible; more anteriorly the
lesion was radiolucent. The right coronoid process was
massively expanded and extended into the infratemporal
fossa. The mandibular right second and third molars were
unerupted and displaced anteriorly by the lesion (Fig 2).
Computed tomography (CT) confirmed the presence of a
large, central, well-defined cyst surrounded by a layer of
poorly mineralized bone of variable thickness that in some
areas had replaced the cortex and in others merged with host
bone. The lesion extended to involve the coronoid, ramus,
and posterior aspects of the body of the right mandible. The
expanded coronoid process encroached into the infratemporal fossa with displacement and erosion of the right zygomatic arch (Figs 3 and 4).
At biopsy, the affected mandible was found to be soft and
fibrous in texture. There was no plane of demarcation
between the lesion and surrounding bone. The lesion
consisted principally of a unilocular cyst that measured

337

338 Ferretti, Coleman, and Altini

ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY

September 1999

Fig 2. Panoramic radiograph shows ill-defined, diffuse,

ground glass, radiopaque lesion occupying ramus, angle,
and body of mandible and causing massive expansion of coronoid process (arrows).

Fig 1. Frontal view shows large, diffuse swelling of right side

of face and limited mouth opening.

approximately 5 cm in diameter and was lined by a thick,

fibrous tissue layer.
Histologic examination of the decalcified sections showed
a thin layer of cortical and cancellous bone, which merged
with a fibro-osseous lesion consisting of hypercellular
fibrous connective tissue containing scattered irregular foci
of osteoid and thin trabeculae of woven bone; some of this
woven bone showed osteoblastic rimming (Fig 5). The
fibrous connective tissue was composed of plump,
ovaltospindle-shaped fibroblasts with vesicular nuclei.
Scattered multinucleated, osteoclastlike giant cells were
present. On the innermost aspect of the biopsy specimen, an
irregular cystlike cavity was present; it was lined by a thick
layer of fibrous connective tissue and in places by inflamed
granulation tissue. There was no epithelial lining (Fig 6). The
histologic features were not pathognomonic; they were
essentially those of a benign fibro-osseous lesion. The differential diagnosis included a reparative/reactive process,
fibrous dysplasia, juvenile cemento-ossifying fibroma, and
osteoblastoma.
In view of the poor circumscription of the lesion radiographically, the intraoperative finding of a soft, fibrous
lesion with no plane of cleavage, and the absence of any
history of trauma (although it should be noted that in approximately 50% of cases subsequently diagnosed as traumatic
bone cyst, there has been no previous trauma to the area), the
lesion was signed out as fibrous dysplasia with a secondary
degenerative cyst. The case was referred for consultation to a

pathologist, who subsequently agreed with our diagnosis

(personal communication, Dr K. Unni, Rochester, Minn).
A coronoidectomy and enucleation of the cyst were
performed through use of a combined submandibular and
intraoral approach. Lack of circumscription of the lesion was
again confirmed at this time. No attempt was made to remove
the entire lesion. Postoperative healing was uneventful, and
the patient was maintained on a rigorous home physiotherapy
program. Six months postoperatively, the patient maintained
a mouth opening of 30 mm, and there was no increase in the
size of the lesion. The patient has subsequently been lost to
follow-up, and all attempts to recall him have failed (he lives
in a rural community). However, his father has reported that
2 years after the operation there has been no increase in the
size of the lesion and the patient has not been experiencing
any functional problems.

DISCUSSION
Aneurysmal and simple bone cysts (the latter also
referred to as a unicameral bone cyst, solitary bone
cyst, and traumatic bone cyst) are well-defined clinicopathologic entities that sometimes occur as secondary
phenomena in many benign and malignant bone
tumors and tumorlike lesions. In addition, secondary
cystic lesions of bone are encountered that fail to meet
the histologic criteria for a diagnosis of either
aneurysmal or simple bone cyst.19,20 These cysts
consist of blood-filled cavities in bone that are lined by
a thick layer of fibrous tissue; they have been referred
to as nonspecific cystic degenerations.4,17 They do not
appear to represent yet another distinct pathologic
lesion and have not been classified as such by various
authorities.21-23 More probably, they form part of the
clinicopathologic spectrum of nonepithelial-lined
cysts of bone.
The pathogenesis of nonepithelial-lined bone cysts

ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY

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Fig 3. Coronal CT scan shows buccal and lingual expansion

of coronoid process of right mandible extending into
infratemporal fossa. At center of lesion is a large, wellcircumscribed unilocular cyst.

remains unknown. However, there is growing acceptance of the postulate that aneurysmal and simple bone
cysts are 2 histologic expressions of a related process.24
It has been proposed that these cystic lesions arise from
an intrabony vascular defect, such as an arteriovenous
malformation that results in intramedullary hemorrhage.8,13,15,25 Direct circulatory connection with the
hematoma may lead to the formation of an aneurysmal
bone cyst, whereas complete interruption of the blood
supply may lead to simple bone cyst formation. It is an
attractive concept to include nonspecific cystic degenerations in this spectrum and to consider them as representing another manifestation of this pathogenetic
process. The clinical findings of a cavity filled with
blood and lined by a vascular connective tissue provide
support for this proposal. Support for the origin of
nonepithelial-lined bone cysts from vascular defects
has been provided by the finding that aneurysmal bone
cysts had elevated intracystic pressure consistent with
an arteriovenous communication.8
It remains difficult, however, to explain why certain
of these nonepithelial-lined bone cysts occur more
frequently in some fibro-osseous lesions than in
others,13-15 and it should be pointed out that unlike the
aneurysmal and simple bone cysts, nonspecific cystic
degeneration does not appear to occur as a primary
phenomenon. This suggests that other factors may be
involved in the pathogenesis.

Ferretti, Coleman, and Altini 339

Fig 4. Axial CT scan shows expanded ramus of right mandible

causing lateral displacement and thinning of right zygomatic
arch. Central cyst is surrounded by a layer of poorly mineralized bone, which in parts has replaced cortex. In condylar
region, lesional tissue merges with surrounding bone.

The first reports of fibrous dysplasia complicated

by nonspecific cystic degeneration have been attributed to Jaffe26 and Schlesinger, Keats, and Ruoff.27
Their cases occurred in the rib and proximal tibia,
respectively. A comprehensive review of 42 cases of
extragnathic fibrous dysplasia2 revealed 13 examples
of nonspecific macrocystic and microcystic degeneration. An additional 3 cases of nonspecific cystic
degeneration occurring in fibrous dysplasia of the
ribs, vertebra, and tibia have been described.3 In these
instances, the rapid swelling associated with the
cystic degeneration raised concerns about possible
malignant transformation.
Nonspecific cystic degeneration occurring in
fibrous dysplasia of the jaws has rarely been reported
in the literature. Obwegeser, Freihofer, and Horejs5
reported 2 cases of fibrous dysplasia that demonstrated radiographic and clinical evidence of cyst
formation. In one of these cases, which presented as a
unilocular radiolucency of the mandible associated
with several impacted teeth, the cyst wall was
composed of highly cellular connective tissue with no
epithelial lining. The cyst recurred after treatment; in
addition, the patient subsequently developed several
similar cystic lesions in the maxilla. Fisher17 reported
2 cases of bone cavities in fibro-osseous lesions in the
maxillofacial skeleton. One of these lesions, which
presented as a nontender expansion of the mandible
and radiographically as a well-defined radiolucency,
was characterized by a cystic cavity lined by dense
fibrous tissue.

340 Ferretti, Coleman, and Altini

ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY

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Fig 5. Fibrous dysplasia consists of cellular fibrous connective tissue stroma with scattered irregular trabeculae and osteoid (hematoxylin-eosin, original magnification 112).

A single case of fibrous dysplasia of the mandible

complicated by a large simple bone cyst was published
by Hara et al.1 Their report, however, shows the cystic
cavity to have been lined by a thick fibrous tissue layer;
in our opinion, the case should be regarded as an additional example of nonspecific cystic degeneration. The
cyst did not resolve after marsupialization.
The occurrence of aneurysmal bone cyst in fibrous
dysplasia of the jaws is more frequently reported.3,6,16
A review of 53 cases of fibro-osseous lesions of the
jaws found associated aneurysmal bone cysts in 21%.3
In a later study of 212 bone tumors and tumorlike
lesions of the jaws, microcysts and blood-filled spaces
similar to those seen in aneurysmal bone cyst were
observed in 10% of the fibrous dysplasias.18
The development of cystic degeneration in fibrous
dysplasia can pose a diagnostic and therapeutic
dilemma. It may present clinically as a rapidly
enlarging mass that can be alarming to both patient and
physician. In this regard, sarcomatous transformation
with and without radiation therapy has been
reported.28,29 Such transformation should be borne in
mind when cases of fibrous dysplasia characterized by

sudden rapid enlargement are being assessed. The

swellings may be bony-hard or fluctuant; the latter
finding is not usually associated with fibrous dysplasia
and can be misleading. Moreover, the radiographic
picture may fail to reflect the cystic nature of the
lesion. CT is thus an essential aid in the diagnosis.
It is a generally accepted principle in the management of fibrous dysplasia that surgery should be postponed until late adolescence to avoid the possibility of
postsurgical recurrence and that it should be limited to
cosmetic debulking of the lesion.30 The development
of cystic degeneration may cause considerable expansion and concurrent functional and cosmetic problems
that may compel surgery at a younger age.
In the case reported, surgery had to be performed early
to relieve the obstruction to mouth opening caused by
the expanding lesion. In addition, the surgery was more
extensive to ensure complete removal of the cystic mass
and the expanded coronoid. However, no attempt was
made to remove the affected bone in its entirety.
Finally, it is interesting to note that several authors
have reported recurrences after surgical removal of
nonepithelial-lined bone cysts occurring in fibro-

Ferretti, Coleman, and Altini 341

ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY

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Fig 6. High-power view shows cyst consisting of dense, hyalinized fibrous tissue without any epithelial lining
(hematoxylin-eosin, original magnification 40).

osseous jaw lesions.6,17 Careful follow-up is therefore advised.

We thank Dr Krishnan Unni of the Mayo Clinic, Rochester,
Minn, for reviewing the case.

9.
10.
11.

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Reprint requests:
Hedley Coleman, BDS, BChD(Hons), M Dent
Division of Oral Pathology
Private Bag 3
WITS 2050
South Africa

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