cystic degeneration in fibrous dysplasia of the jaws.pdf

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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 hypercellularfibrous 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 differ-

ential diagnosis included a reparative/reactive process,

fibrous dysplasia, juvenile cemento-ossifying fibroma, and

osteoblastoma.

In view of the poor circumscription of the lesion radi-

ographically, 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 approx-

imately 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 experiencingany functional problems.

DISCUSSIONAneurysmal and simple bone cysts (the latter also

referred to as a unicameral bone cyst, solitary bone

cyst, and traumatic bone cyst) are well-defined clinico-

pathologic 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

338 Ferretti, Coleman, and Altini ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGYSeptember 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 coro-

noid process (arrows).

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

of face and limited mouth opening.


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remains unknown. However, there is growing accep-

tance 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 hemor-

rhage.8,13,15,25 Direct circulatory connection with thehematoma 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 degener-

ations in this spectrum and to consider them as repre-

senting 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.

The first reports of fibrous dysplasia complicated

by nonspecific cystic degeneration have been attrib-

uted 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 degenera-

tion. An additional 3 cases of nonspecific cystic

degeneration occurring in fibrous dysplasia of the

ribs, vertebra, and tibia have been described.3 In theseinstances, 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 demon-

strated 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.

ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY Ferretti, Coleman, and Altini 339Volume 88, Number 3

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, well-

circumscribed unilocular cyst.

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 mineral-

ized bone, which in parts has replaced cortex. In condylarregion, lesional tissue merges with surrounding bone.


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

fore advised.

We thank Dr Krishnan Unni of the Mayo Clinic, Rochester,

Minn, for reviewing the case.

REFERENCES1. Hara H, Ohishi M, Higuchi Y. Fibrous dysplasia of the mandible

associated with large solitary bone cyst. J Oral Maxillofac Surg1990;48:88-91.

2. Martinez V, Sissons HA. Aneurysmal bone cyst: a review of 123cases including primary lesions and those secondary to otherbone pathology. Cancer 1988;61:2291-304.

3. El-Deeb M, Sedano HO, Waite DE. Aneurysmal bone cyst of thejaws: report of a case associated with fibrous dysplasia andreview of the literature. Int J Oral Surg 1980;9:301-11.

4. Simpson AHRW, Creasy TS, Williamson DM, et al. Cystic

degeneration of fibrous dysplasia masquerading as sarcoma. JBone Joint Surg 1989;71B:434-6.

5. Obwegeser HL, Freihofer HPM, Horejs J. Variations of fibrousdysplasia in the jaws. J Maxillofac Surg 1973;1:161-71.

6. Oliver LP. Aneurysmal bone cyst: report of a case. Oral SurgOral Med Oral Pathol 1973;35:67-76.

7. Diercks RL, Sauter AJM, Mallens WMC. Aneurysmal bone cystin association with fibrous dysplasia. J Bone Joint Surg1986;68B:144-6.

8. Biesecker JL, Marcove RC, Huvos AG, et al. Aneurysmal bone

cysts: a clinicopathologic study of 66 cases. Cancer 1970;26;615-25.

9. Waldron CA. Fibro-osseous lesions of the jaws. J Oral

Maxillofac Surg 1985;43:249-62.10. Makek MS. So-called fibro-osseous lesions of tumorous

origin. Biology confronts terminology. J Craniomaxillofac Surg1987;15:154-68.

11. Svensson B, Isacsson G. Benign osteoblastoma associated withan aneurysmal bone cyst of the mandibular ramus and condyle.Oral Surg Oral Med Oral Pathol 1993;76:433-6.

12. Saito Y, Hoshina Y, Nagamine T, et al. Simple bone cyst: a clin-ical and histopathological study of fifteen cases. Oral Surg OralMed Oral Pathol 1992;74:487-91.

13. Higuchi Y, Nakamura N, Tashiro H. Clinicopathologic study ofcemento-osseous dysplasia producing cysts of the mandible.Oral Surg Oral Med Oral Pathol 1988;65:339-42.

14. Ackerman GL, Altini M. The cementomas: a clinico-patholog-ical reappraisal. Journal of the Dental Association of SouthAfrica 1992;47:187-94.

15. Melrose RJ,Abrams AM, Mills BG. Florid osseous dysplasia: a

clinicopathologic study of thirty-four cases. Oral Surg Oral MedOral Pathol 1976;41:62-82.

16. Buraczewski J, Dabska M. Pathogenesis of aneurysmal bonecyst: relationship between the aneurysmal bone cyst and fibrousdysplasia of bone. Cancer 1971;28:597-604.

17. Fisher AD. Bone cavities in fibro-osseous lesions. Br J Oral Surg1976;14:120-7.

18. Struthers PJ, Shear M. Aneurysmal bone cyst of the jaws: patho-genesis. Int J Oral Surg 1984;13:92-100.

19. Rushton MA. Solitary bone cysts in the mandible. Br Dent J1946;81:37-49.

ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY Ferretti, Coleman, and Altini 341Volume 88, Number 3

Fig 6. High-power view shows cyst consisting of dense, hyalinized fibrous tissue without any epithelial lining

(hematoxylin-eosin, original magnification 40).


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20. Jaffe HL, Liechtenstein L. Solitary unicameral bone cyst withemphasis on the roentgen picture, the pathologic appearance andthe pathogenesis. Arch Surg 1942;44:1004-25.

21. Shear M. Cysts of the oral regions 3rd ed. Oxford: Wright; 1992.p 171-86.

22. Unni KK. Dahlins bone tumours: general aspects and data on11087 cases 5th ed. Philadelphia: Lippincott-Raven; 1996. p382-93.

23. Kramer IRH, Pindborg JJ, Shear M. The WHO histologicaltyping of odontogenic tumours. Cancer 1992;70:2988-94.

24. Hillerup S, Hjorting-Hansen E. Aneurysmal bone cystsimplebone cyst: two aspects of the same pathological entity? Int J OralSurg 1978;7:16-22.

25. Jaffe HL. Giant-cell reparative granuloma, traumatic bone cyst,

and fibrous (fibro-osseous) dysplasia of the jaw bones. OralSurg Oral Med Oral Pathol 1953;6:159-75.

26. Jaffe HL. Fibrous dysplasia of bone. Bulletin of the New YorkAcademy of Medicine 1946;22:588-604.

27. Schlesinger PT, Keats S, Ruoff AC III. Fibrous dysplasia: reportof a case. J Bone Joint Surg Am 1949;31A:187-91.

28. Schwartz DT, Alpert M. The malignant transformation of fibrousdysplasia. Am J Med Sci 1964;247:1-20.

29. Eversole LR, Sabes WR, Rovin S. Fibrous dysplasia: a noso-logic problem in the diagnosis of fibro-osseous lesions of the

jaws. J Oral Pathol 1972;1:189-220.30. Waldron CA. Fibro-osseous lesions of the jaws. J Oral

Maxillofac Surg 1993;51:828-35.

Reprint requests:

Hedley Coleman, BDS, BChD(Hons), M DentDivision of Oral PathologyPrivate Bag 3WITS 2050South Africa

342 Ferretti, Coleman, and Altini ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGYSeptember 1999

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