florid osseous dysplasia

Florid osseous dysplasia A clinical-pathologic study of thirty-four cases

Raymond J. Melrose, D.D.S.,* Albert M. Abrams, D.D.S., M.S.,#* and Barbara G. Mills, M.D.,*** Los Angeles, Calif.

SCHOOL OF DENTISTRY, UNIVERSITY OF SOUTHERN CALIFORNIA

Thirty-four patients with florid osseous dysplasia were studied. The majority were asymptomatic Negro women: Seventeen biopsy-proven simple bone cysts were found in affected quadrants of fourteen patients. Radiographs displayed a spectrum of sclerotic and ground-glass opacities limited to alveolar processes but not to root apices. Biopsy material was studied in all cases, and biochemical analyses of serum and cyst fluid were performed on some patients. Test results and skeletal radiographs indicate that the disease is limited to the jaws. Patients have remained asymptomatic with little alteration of radiographic patterns. Three cysts failed to heal following treatment, others filled with radiographically abnormal tissue. Chronic osteomyelitis may infrequently complicate the disease. These eases appear to represent the most exuberant manifestation of this reactive fibro-osseous jaw disease.

0 sseous dysplasia is a disease with a spectrum ranging from localized periapical involvement ( cementoma,l periapical cemental dysplasia,2 periapical fibrous dysplasia,” periapical osteofibrosis4) to the exuberant multiquadrant process designated in this article as florid osseous dysplasia (FOD). Florid osseous dysplasia is probably not a rare condition, and what appear to be additional cases have been reported under an array of terms, including multiple cemento-ossifying fibromas,6 sclerosing osteitis,7 sclerosing osteomyelitis,* multiple enOstosis,g multiple osteomas,‘* periapical cementoblastoma,ll Paget’s disease of the mandible,l’ gigantiform cementoma,2~ 13-15 chronic sclerosing Osteomyelitis,lG-lg sclerotic cemental masses of the jaws,20 and multiple periapical osteofibromatosis.*l Simple bone cysts have been described in adults, 22-24 but the concomitant occurrence of

This work was supported in part by United States Public Health Service Grant 5-TI2- CA08023 from the National Cancer Institute.

*Associate Professor, Department of Pathology. **Professor and Chairman, Department of Pathology. ***Assistant Professor, Department of Physiology.

62

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Florid osseous dysplasia 63

florid osseous dysplasia and simple bone cysts is distinctly uncommon. In a search of the English-language literature, we found only two cases which seemed to be valid examples.2”, 2~ One of these is included in the present study. A third case, also included here, was reported by us under the term “multiple osseous dysplasia with concomitant simple bone cyst.“2i

This study was undertaken to document and better define this peculiar condition, to help clarify its position in the scheme of fibro-osseous diseases, and to es- tablish diagnostic parameters.

MATERIALS AND METHODS

Cases were selected from files of the Oral Pathology Registry, Oral Tumor Board, and Diagnostic Oral Pathology Laboratory at the IJniversity of Southern California School of Dentistry. Criteria for inclusion in the study were (1) ade- quate historical data ; (2) radiographs (or copies) displaying diffuse alveolar process involvement affecting more than one quadrant and with disease not limited to the apices ; (3) microscopic slides showing features described in detail later. On the basis of these criteria, thirty-four cases were found to be acceptable examples.

When possible, patients were recalled for examination. Otherwise, the referring dentist was contacted for follow-up information. Regrettably, this information was not obtainable in many instances. An attempt was made to secure fresh biopsy material and serum samples from each recalled patient. For the past several years serum samples have also been obtained from new patients suspected of having FOD seen at the School of Dentistry. In those patients with concomitant simple bone cysts, an attempt was also made to acquire “cyst” fluid prior to exploration. Referring clinicians were requested to obtain serum calcium, phosphorus, and alkaline phosphatase values for their recalled patients.

CLINICAL FEATURES

Age at diagnosis ranged from 26 to 59 years, with a mean of 42 years. Thirty- three of thirty-four patients were women. There were thirty-two Negroes and one oriental, and race was unknown in one instance. Duration was unknown in twenty-eight cases and varied from 6 months to over 29 years in the remainder. Twenty-three patients were asymptomatic; the condition was discovered on rou- tine radiographs. One asymptomatic patient stated that a “severe infection” had occurred in an affected area 1 year previously. Eight patients complained of a vague, intermittent, dull aching sensation in the mandibular molar area, while two patients complained of toothache but no symptoms referable to their bone disease. One patient reported intermittent pain and swelling in the posterior mandible for 3 months prior to diagnosis, and at surgery several loose scquestra were removed along with viable tissue from two asymptomatic affected quadrants.

Of six patients on whom pulp vitality tests were performed, only three mandibular molars of one patient failed to respond. These teeth were neither carious nor restored.

Hard buccal and/or lingual cortical expansion was present in sixteen patients and tended to correlate with the radiographically determined extent of involve-

64 Melrose, Abram, and Mills Oral Surg. January, 1976

EXTENT OF INVOLVEMENT

FOD with SBC

3M 2M 1M 28 1B C L C - C L C 1B 28 1M 2M 3M

‘iv

I ; ---___---- @

-----_ -‘ii:

_-______ 8 I 12

- - -------------------------, c---l 13

--------mm------- ,-------- 12

left Mandible right

Chart 1. Broken line denotes extent of simple bone cyst. Solid line represents extent of FOD.

ment. Externally visible facial asymmetry was not commented upon, and none was observed in any patient examined by us.

A total of seventeen simple bone cysts were found in fourteen patients. Over a prolonged interval, one patient developed bilateral mandibular cysts and a cyst in the posterior maxilla. A second patient had simultaneous bilateral mandibular cysts. All cysts were proved by surgical exploration and biopsy. Aspiration yielded small quantities of thin yellow to serosanguineous fluid in twelve instances. One surgeon reported finding only a “hole,” and there was no record con- cerning contents of four cysts. Most surgeons reported that cysts had defined boundaries, but in one instance (personally witnessed by one of us) the surgeon

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EXTENT OFINVOLVEMENT FODWithOUtSBC

Maxilla

I I 0

3M 2M 1M 2B 16 C L C - C L C 1B 28 1M 2M 3M

I I 1 left

w I 2

I Mandible

-@ riqht

Chart 2’.

was unable to delineate cyst limits despite the creation of a large window in the expanded lingual cortex in the incisor region.

RADIOGRAPHIC FEATURE5

In twenty patients, FOD was present in four quadrants, while in two there was bilateral mandibular and unilateral maxillary involvement. In twelve patients, disease was present in the mandible bilaterally (Charts 1 and 2).

The spectrum was considerable ; radiographs usually displayed diffuse distri-

66 Me&rose, Abranzs, and Mills Ornl Surg. Janunry, 1976

Fig. 1. Full-mouth radiographs of usymptomatie 43.year-old Negro woman, showing extensive irregular opacities involving both edcrltulous and tooth-bearing areas.

bution of lobular, irregularly shaped radiopacities throughout the alveolar process (Figs. 1 to 3). These were often enmeshed within poorly defined zones of de- creased radiodensity having a “ground-glass” appearance. Only occasionally was a normal trabecular pattern visible in an affected quadrant. This was especially true in the mandible. In panographic, lateral jaw, and anteroposterior projections the appearance was often strikingly “pagetoid” (Fig. l), but only the alveolar processes were affected. In some patients, there were zones of limited involvement consisting of “typical” osseous dysplasia at root apices. In a few instances teeth showed hypercementosis or fusion of irregular masses of mineralized tissue to the apices. Progressive external resorption of a molar root occurred over a 6-year period in one patient (Fig. 4). Occlusal films displayed intact, thinned cortices overlying areas of expansion.

Concomitant simple bone cysts mere well-defined and, when large, extended toward the inferior border. In three cases mineralized tissue attached to a root apex projected into the radiolucent cavity.

Cysts varied from 0.5 cm. to 3.5 cm. in greatest dimension, except in one notable instance when the entire mandible was progressively “hollowed out” (Fig. 5). Four cysts mere found in edentulous areas, and three were located between the roots of mandibular molars. Smaller lesions were well demarcated and uniloc- ulated, while larger cysts tended to be multiloculated and suggested other odontogenic or fibro-osseous lesions, Larger cysts were associated with jaw expansion in four patients.

Skeletal radiographs were obtained of three patients with extensive four- quadrant involvement, but no skeletal evidence of osseous disease was found.

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Fig. d. This 59-year-old asymptomatic Negro woman has extensive mandibular involvement by lobular sclerotic masses. Localized disease can be seen in the posterior maxilla.

Fig. 3. The “ground-glass” pattern is well illustrated in this asymptomatic 27-year-old Negro woman with four-quadrant disease.

MICROSCOPIC APPEARANCE

Most cases were composed of a mixture of cementum-like material and irregular trabeculae of bone (Fig. 7). Some bone trabeculae were rimmed by plump osteoblasts with large vesicular nuclei and abundant, sometimes vacuolated cyto- plasm (Fig. 8). Active resorption associated with numerous multinucleated osteo- clasts could be seen. This mineralized tissue was in an abundant and cellular fibrous connective tissue stroma. In some areas spindle-shaped fibroblasts were ar- ranged in a swirling pattern about small mineralized deposits (Fig. 9). Mitoses were not seen. Inflammation was found in only one case, and this was associated with a sequestrum. Irregular and rounded deposits of metaplastically formed “cementoid” had dark purple boundaries and often seemed to fuse, creating large globular masses with rounded contours sometimes manifesting a narrow “feather edge.” Coalescence occasionally imparted a “pagetoid” appearance because of the presence of basophilic staining lines resembling “reversal” lines (Figs. 10 and 11).

The stroma often contained a rich capillary network. Many capillaries were

60 Melrose, Abram, a<nnd Mills Oral Slug. January, 1976

Fig. 4. A, Radiograph of 48-year-old Negro woman in 1967 shows fusion of large and small sclerotic masses to the tooth roots, surgically confirmed SBC in the right posterior mandible, and partial root resorption of the mandibular right first molar. B, The 1971 radiograph of the same patient shows filling-in of the SIX on the right by abnormal-appearing bone. Note significant alteration in bony pattern in the left mandible and more extensive maxillary involvement as compared to the 1967 film. There has been no opportunity to explore the left mandible.

almost aneurysmally dilated (Fig. 12). This degree of vascularity was not limited to those patients in whom simple bone cysts were present.

The simple bone cysts were generally lined with flattened, elongated spindle cells, often supported by a narrow band of fibrous connective tissue (Fig. 14). Oc- casionally, cyst linings featured a layer of plump cells resembling osteoblasts.

In several cysts, portions of the wall lacked bands of fibrous connective tissue but, instead, were characterized by formation of a condensed eosinophilic matrix resembling osteoid into which calcium salts were deposited in a fuzzy dystrophic pattern (Fig. 15).

Examination under polarized light disclosed a variety of birefringent patterns similar to those described by Waldron and associates.Zo “Droplet cementum” displayed little birefringence except at the “feather-edge” periphery, which was often brightly birefringent. Fused globular cementum-like masses presented a mixed pattern of swirls and of intersecting groups of short, thick fibers. Occasion-

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Florid osseous dysplnsia 69

F ‘ig. 5. Pronounced, progressive involvement by surgically proven simple bone cysts lustra ted in this series of radiographs from an asymptomatic 44-year-old Negro morn2 1955; B and C, 1967; D, 1973. Beginning maxillary involvement apparent in 1967 (C more extensive m 1973 (II).

is il- tn. A, ) and

70 Melrose, Abram, and Mills Oral Burg. January, 1976

Fig. 5 (Cont’d). For legend see p. 69.

ally, lamellar bone fragments could be readily distinguished enmeshed within tissue resembling cementum. Zones of dystrophic mineralization showed fairly diffuse birefringence in which long, slender parallel lines could sometimes be seen.

Analysis of fluid removed from three cysts in patients with concomitant FOD and from three teen-agers with typical simple bone cysts showed consistent elevations of alkaline phosphatase and acid phosphatase (Tables I and II). Alkaline phosphatase levels were higher in those patients with FOD, but acid phosphatase levels were similar for both groups. In both groups the enzyme levels were higher than normal serum values. Calcium and phosphorus levels in serum and/or cyst fluid were also obtained when sample size permitted. With one exception, values for both minerals in cyst fluid were slightly lower than the measured or expected serum values.

Three patients had borderline elevations of serum calcium. In one instance parathyroid hormone assay was obtained; this showed slight elevation beyond the upper limit of normal. Unfortunately, these patients were uncooperative and have been lost to follow-up. Parathyroid hormone radioimmunoassays were performed on two normocalcemic patients with widely divergent results which the laboratory claimed were within normal limits and represented alteration in laboratory standard.

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Plorid osseous dysplasia 71

frc wa pa

Fig. 6. Radiographs of 42-year-old Negro woman show alteration in trabecular pat ,m 1967 (A) to 1971 (B). In 1967 the mandibular left third molar was extracted and an i s explored below the second molar. It failed to fill in completely by 1971. This patient in in the region of the SBC; this resolved following surgical intervention.

tern 3BC had

72 Melrose, Abrams, and Mills Oral Surg. January, 1976

Fig. 7. Composite photomicrograph displaying various types of mineralized material that were seen in FOD. A, Woven bone with cellular fibrous tissue. R, Coalescing masses of cemen- turn-like material in cellular fibrous stroma. C, Globular masses of cementum-like material supported by sparsely cellular fibrous tissue. (Hematoxylin and eosin. Original magnification, x40.)

FOLLOW-UP

Follow-up information and/or recent radiographs were obtained for five of twenty patients with FOD without concomitant simple bone cysts. Duration ranged from 1 to 3 years. Follow-up duration in nine of fourteen patients with concomitant simple bone cysts ranged from 1 to 29 years.

None of these patients reported symptoms during their respective follow-up periods, and biopsy sites had healed without complication. In two patients, con- tinued slow mandibular cortical expansion was noted.

Sequential radiographs showed little alteration of the basic mixed radiolucent/radiopaque pattern unique to each patient. In several patients, however, progressive mineralization of radiolucent zones occurred, with creation of irregular radiopaque masses. Except in cases in which there were concomitant simple bone cysts, radiographic evidence of progressive demineralization was not seen.

In six of these nine patients with simple bone cysts complete resolution of the radiolucency was noted following surgical intervention. However, the area filled with radiographically abnormal tissue similar to the adjacent bone. In three patients, only partial radiographic resolution of the cyst occurred, and the lesions either persisted or enlarged in another direction (Fig. 6).

In one notable patient progressive involvement of both horizontal rami and symphysis occurred between 1967 and 1974 despite three surgical procedures in

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Florid OSSEOUS dysplasia 73

Fig. 8. This ease features trabeculae of woven bone bordered by plump osteoblasts. (Hema- toxylin and eosin stain. Original magnification, x100.)

Fig. 9. This example of FOD is characterized by cellular fibrous connective tissue containing small mineralized deposits resembling cementicles. (Hematoxylin and eosin stain. Original magnification, x100.)

1967, 19’73, and 1974 in the right, left, and anterior portions of the mandible, re- spectively. Most recent radiographs show slight evidence of filling in the posterior mandible bilaterally, but the anterior mandible is unchanged and the maxilla now displays radiographic evidence of what appears to be a simple bone cyst extending from cuspid to cuspid (Fig. 5, B, C, and D). Radiographs of this patient taken in 1955 document the presence of “typical” FOD preceding development of the cysts (Fig. 5, A). Another patient developed three separate lesions over a period of 22 years, but each healed after exploration and biopsy.

74 Melrose, Abrams, ad Mills Oral Hurg. January, 1976

Pig. 10. It is difficult to tell whether the ovoid mineralized masses in this cellular area of FOD represent hone or cementum. (Hematoxylin and eosin stain. Original magnification, x100.)

Pig. 11. Large cementnl masses in FOD apparently develop by coalescence of globular masses similar to those illustrated in Fig. 7. Note the markedly dilated capillaries and the scat- tered reversal lines. (Hematoxylin and eosin stain. Original magnification, x100.)

DISCUSSION

Age range, predilection for females, and marked preponderance of Negro patients are similar to data reported for “typical” osseous dysplasia.‘! 2 The absence of Caucasians is unusual and is at slight variance with the study reported by Waldron, Giansanti, and Browand20 in which Negroes represented 87.5 per cent (12/14) of proven florid cases. Only 62 per cent of their “isolated” cases were Negro patients, and this might suggest that some of these are truly isolated lesions and do not represent a manifestation of the florid form of the disease.

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Plorid osseous dysplasia 75

Fig. 1.3. Aneurysmally dilated capillary bordered by bone similar to that seen adjacent to SBC’s and may represent early development of a cyst. (Hematoxylin and eosin stain. Original magnification, x100.)

Fig. 13. SBC characterized by a membranous lining supported by fibrous tissue in which dystrophic calcification and bone may be seen. (Hematoxylin and eosin stain. Original magnification, x40.)

This is not known because full-mouth radiographs were unavailable for study. Since the majority of our cases were submitted by community oral surgeons and the greatest proportion of specimens obtained by these individuals is from the Caucasian population, it seems unlikely that sample bias played a significant role in the data reported here.

Laband and Leacock? reported nineteen cases which they interpreted as “sclerosing osteitis” and which appear to be valid examples of FOD. All occurred in adult, Negro females ; however, their patient population was predominantly

76 Melrose, Abrams, and Mills Oral Surg. January, 1976

Fig. 24. High-power view of a cyst wall showing the lumen bordered by a narrow band of fibrous connective tissue supported by a broad bone trabecula. (Hematoxylin and eosin stain. Original magnification, x250.)

FQ. 15. The wall of this SBC features a band of osteoid-like material in which diffuse mineralization has occurred. The lumen is lined with both plump and flattened cells. (Hematoxylin and eosin stain. Original magnification, x100.)

Negro. On the other hand, Bhaskar and Cutrights reported a slight preponderance of Caucasians in their sixteen cases of “multiple enostosis” which also appear to be valid examples of FOD. The World Health Organization (WHO) “Histolog- ical Typing of Odontogenic Tumours, Jaw Cysts and Allied Lesions” uses the designation gigantiform cementoma to categorize what appears to be FOD and states that the disease is most common in middle-aged Negro women.2 The same classification also lists the term familial multiple cementomas as a synonym for

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Table I. Florid osseous dysplasia

Patient Ca P Alkaline Acid

phosphatase phospharase

M.C. (1973) S (1974)

: M.M. T.S. :

(1973) B.R. c

(1974) ;

CM?. :

T.T. S

9.4

lo*5 11:2 9.7 7.7

iI 11:3 10.1

8.8

With simpfObone cysrs

; 1.90 0.13 2.10 0.54

:*; 2.20 0.56

316 1.95 0.48 2.55 0.50

27.20 3.36 2.7 * 3.3 2520

1:95 Et 21.40 2:68

3.6 114t

L.MCC. Without bone

cysts C.H. : 1:::

sim&e 3:2

1.70 1.75

A.A. 3.7 16 K.A.U. M.J. I;;;;;

; E

10:2 2.70

s” 2.8 2.35 E.M. 8.1 * * I.W.

: 11.2

i.3” 1.1 B.U.

W.B. 10.4 B.M. S 9.6 2:6

6.0 K.A.U. 1.35

9 = Serum. c = cyst. *Inadequate sample tUnits unknown; normal range stated 19-74. Normal values: Ca = 9-11 mg. per cent. P = 3-4.5 mg. per cent. Alkaline phosphatase = 0.80-3.00 sigma units.

3.0 -14 King-Armstrong units. 2.0 -4.5 Bodansky units.

Acid phosphatase = Female 0.01-0.56 sigma units. Male 0.13-0.63 sigma units.

All enzymes reported as sigma units except where noted.

0.49

1.25 0.55

*

1.35

gigantiform cementoma, as do Shafer, Hine, and Levy.‘28 Apparently Agazzi and Belloni reported several cases accepted by Shafer and associates as examples of “gigantiform cementoma” in which a familial incidence was manifest. Although we have not been able to review this paper, we also have not been able to find additional familial instances in our review of the literature. One of our patients stated that “several family members had similar jaw problems,” but they were unavailable for examination. Recalled patients were questioned, and none were aware of affected family members, but this information cannot be considered reliable. The children (ages 4 and 7 years) of one patient demonstrated no ab- normalities. This is to be expected since florid osseous dysplasia has yet to be reported in a child. Insufficient data are available for conclusions on the familial aspects of this disease.

Two patients who had symptoms of osteomyelitis were edentulous in the affected regions and wore removable prostheses. The development of low-grade osteomyelitis with sequestra in edentulous areas seems to be the principal complication of FODzO and has resulted in a number of case reports of “chronic scleros-

78 Melrose, Abram, and Mills Oral Burg. January, 1976

Table II. “Control” patients (simple bone cysts only)

Alkaline Acid Patient Ca P phosphatmef phosphatasef

L.M. : 8.1 ::; 5.80 1.09 12.7 15.00 3.56

E.H. s: ;8 2:9 3.50 0.12 9.00 4.24

B.E. c ; * 10.00 2.48

*Inadequate sample. tNorma1 (child), 2.80-6.70 sigma units. iNorma (child) is unknown.

ing osteomyelitis” and “sclerosing osteitis.” Cases reported by Laband and Lea- cock,7 three cases reported by Be11,8 as well as cases reported by Lyons,‘O El- Mofty, I6 Keen and associates,1’ Shafer,ls and Towns,‘” are representative of this situation. The vast majority of these patients were edentulous, and most were asymptomatic until the development of osteomyelitis. The case reported by El- MoftylG is particularly instructive since the patient was asymptomatic over 30 years of follow-up but developed low-grade osteomyelitis shortly after she began wearing full dentures. Radiographs stated to have been taken in 1941 show the typical picture of FOD.

As suggested by Waldron and colleagues, *O the pathogenesis of this complication of FOD appears to be related to the presence of fused masses of cementum or dysplastic bone which contain few lacunae and no marrow spaces. This tissue has little or no capacity for physiologic resorption beneath prostheses, with resultant mucosal ulceration and exposure of readily sequestrated dense yellow, mineralized fragments. Healing then occurs rapidly, usually without the aid of antibiotics.

Although none of our patients experienced complications directly related to tooth extractions, it would seem prudent to advise retention of teeth if possible and to recommend the use of fixed rather than removable prostheses.

Simple bone cysts in these patients did not differ in most respects from typical simple bone cysts. The fact that all patients were adults is unusual when compared to the studies of Huebner and Turlington,22 Hansen and associates,‘3 and Howe.24

Pain or a dull aching sensation was experienced at the site of a simple bone cyst in five of our fourteen patients. In Huebner and Turlington’P review, 18 per cent complained of pain and 3 per cent experienced some form of paresthesia. In the series of Hansen and colleagues,2” pain or sensitive teeth was recorded in eight of sixty-six patients (12 per cent). In Howe’s study,24 pain was a feature in six of sixty cases.

In these articles no mention is made of whether symptoms were relieved following therapy and resolution. Our material added little information, since follow-up data were available on only one patient who had presented with symptoms and that patient had complete relief of symptoms in the operative area, even though the cyst did not entirely resolve and slowly expanded anteriorly.

There continues to be speculation regarding the etiology of simple bone cysts. Cohenz9 compared sodium, chloride, sugar, nonprotein nitrogen, and total protein

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levels and performed electrophoresis on serum and cyst fluid samples obtained from six patients with simple bone cysts of long bones. In four patients the values obtained resembled serum, while in two they resembled blood. Cohen postulated that the etiology of simple bone cysts was principally related to the blockage of drainage of interstitial fluid in a rapidly growing and remodeling area of cancel- lous bone. He later used radiopaque media injection methods to demonstrate absence of drainage from the proximal portion of cyst walls in two patients-a feature interpreted to be a sign of obstruction.30 On the basis of his combined data, he further postulated that the primary cause of simple bone cysts is venous obstruction caused by a developmental vascular anomaly.

Our laboratory findings do not entirely support Cohen’s contention that cyst fluid represents serum (Tables I and II). However, the fluid might represent a transudate of interstitial fluid regardless of the etiology of accumulation. Ele- vated alkaline phosphatase levels are known to be associated with osteoblastic activity, while elevated acid phosphatase levels are associated with osteoclastic activity.31, 32 Given the marked degree of bone activity in patients with FOD, the high levels of both enzymes in cyst fluid is t,o be expected. While it is conceivable that the elevated acid phosphatase level of some cystic contents might be second- ary to hemolysis, that does not explain the consistent elevation found in associa- tion with all cysts. It is of interest to note that alkaline phosphatase levels were lower in the “control” group of teen-agers who did not have concomitant FOD, while acid phosphatase levels were comparable. Biopsies from our FOD patients often showed increased numbers of dilated capillary vessels regardless of whethel concomitant cysts were present, and it is tempting to speculate that disorderly bone production might result in obstruction to drainage, with the resultant chain of events described by Cohen 3o leading to cyst formation. It is well known that large cystic spaces are often seen in fibrous dysplasia and are reported to contain serumlike fluids.33 On the other hand, in Paget’s disease, where bone activity is greatest and dilated capillaries are most numerous, one almost never finds cystic spaces.

There was no historical, gross, or microscopic evidence in our material to sub- stantiate intramedullary hemorrhage as an etiologic factor in cyst production, as thought by some.24’ 34

The development of multiple cysts and the failure of some to resolve following procedures which normally promote healing strongly suggest that, at least in those patients with concomitant FOD, the process of cyst formation is an active one. Although our data do not shed new light on the etiology of simple bone cysts! they do suggest that research into the hemodynamics of venous blood flow in normal mandibles as well as in those with FOD, simple bone cysts, fibrous dysplasia, and Paget’s disease might be fruitful.

Differential diagnosis of FOD must include Paget’s disease as well as sclerosing osteomyelitis. The absence of clinical signs of infection, multiple quadrant involvement, consistent radiographic features, and lack of osseous inflammation in biopsy specimens readily rule out the latter. However, it must be remembered that patients with FOD seem to have a predisposition to low-grade chronic osteomyelitis when they wear tissue-borne prosthetic appliances.

80 Melrose, Abram, and Mills Oral Surg. January, 1976

Paget’s disease can be excluded by biopsy and evaluation of serum alkaline phosphatase. Although we were unable to measure this enzyme in every patient, only one of thirteen (Table I) had significant elevation. The clinician in this instance thought that the elevation might be more likely due to liver disease, but additional follow-up information was unavailable. Nonetheless, it is suggested that in patients with proven or suspected FOD serum calcium, phosphorus, alkaline phosphatase, acid phosphatase should be evaluated and skeletal radiographs should be made if possible. Accumulation and recording of such data is the only method of determining whether our information on limited numbers of patients is accurate.

We believe this disease to be a distinct clinical pathologic entity representing an exuberant variant of osseous dysplasia, defined by Robinsons to be an abnormal reaction of bone to irritation or stimulation, Although the biology of this disease remains relatively obscure, it is capable of displaying a broad spectrum of clinical features, microscopic patterns, and complications. This spectrum seems to extend from the classic limited periapical involvement of lower incisors (periapical osteofibrosis, periapical cemental dysplasia, cementoma) or other localized condensations (cemental masses) to the florid involvement described in this article. Patients with florid disease may also display a combination of patterns to include diffuse multiple quadrant involvement, sclerotic masses, classic localized disease, and simple bone cysts.

There is evidence to support such an interpretation : 1. The disease is self-limiting, affects only the alveolar processes, and seems

to be independent of teeth. 2. Multiple quadrants are affected, and often in a symmetrical pattern. 3. There is constant and marked predisposition for occurrence in Negro

women. 4. The disease may be present for many years without symptoms. 5. The radiographic patterns may change without surgical intervention, usu-

ally to a more radiopaquc character. 6. There is an unusually high incidence of simple bone cysts in patients with

FOD compared to patients of the same age range without FOD. 7. Simple bone cysts in these patients often manifest active enlargement and

do not always respond to the usual therapeutic methods. 8. When simple bone cysts “heal,” the radiographic appearance of the

mineralized tissue is often abnormal. 9. Biopsies show disorderly unencapsulated fibroblastic proliferation and

mixed types of mineralized tissue distributed in a nonfunctional pattern and in- distinguishable from that seen in classic localized osseous dysplasia (periapical cemental dysplasia).

10. In asymptomatic patients biopsies generally fail to show evidence of in- flammatory disease.

Although some patients have localized osteomyelitis, we believe this to be a complication of the basic disease process and recommend that such terms as chrok diffuse sclerosi?lg osteomyelitis and sclerosi~~g osteitis not be used when FOD is evident. The term gigantifornt cementoma seems especially inappropriate

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because it implies a “giant” solitary lesion rather than a diffuse, multi-quadrant, nonneoplastic process. Familial multiple ce?l2erl tomas is a partially accurate term in the sense that there is more than one lesion. At present, there is minimal evidence that FOD is familial, and more investigation is required before this is established or disproven. Use of the word cementoma is inappropriate because the evidence that all of the abnormal mineralized material actually represents cemen- turn is far from convincing. Identical calcifications may be found in fibro-osseous lesions affecting bones other than the jaws. 35 Indeed, on the basis of our study US-

ing plain and polarized light, mineralized tissues resembling both bone and cementum can be found in the same specimen.

SUMMARY

Clinical, radiographic, and microscopic features of thirty-four patients with florid osseous dysplasia were studied. Data indicate that the condition is self- limiting and restricted to alveolar processes. There is a tendency for simple bone cysts to develop in affected regions. Patients are usually asymptomatic except when the disease is complicated by chronic osteomyelitis. This condition probably represents the most extensive manifestation of the reactive fibro-osseous process commonly known as “cementoma.” The term florid osseous dysplasia is considered to be preferable to other designations found in the literature.

Our appreciation is expressed to the following for contributing case material to this study: Dr. James Bullard, Dr. Vince Castaldo, Dr. Richard Delo, Dr. Lewis Fendell, Dr. James Heid, Dr. Francis Howell, Dr. Paul Jacobs, Dr. Gordon Lambson, Dr. R. W. Lofton, Dr. Kenneth Neal, Dr. Marsh Robinson, Dr. Max Schoen, and Dr. James Vamvas.

REFERENCES 1. Zegarelli, E. V., Kutscher, A. H., Napoli, N., Iurono, F., and Hoffman, P.: The Cementoma

-A Study of 230 Patients With 435 Cementomas. ORAL SURG. 17: 219-224, 1964. 2. Pindborg, J. J., Kramer, I. R. H., and Torloni, H.: Histologic Typing of Odontogenic

Turnours. Jaw Cvsts and Allied Lesions. International Histological Classification of Turnours: No. 5, Geneva, 1971, World Health Organization, pp. 32-3<

3. Chaudhry, A. P., Spink, J. H., and Gorlin, R’. J.: Periapieal Fibrous Dysplasia (Cemen- toma), J. Oral Surg. 16: 483-488, 1958.

4. Ogilvie, A. L.: Periapical Pathosis. In Ingle, J. I.: Endodontics, Philadelphia, 1965, Lea & Febiger, p. 378.

5. Robinson, H. B. G.: Osseous Dysplasia-Reaction of Bone to Injury, J. Oral Surg. 14: 3-14, 1956.

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Oral surg. January, 1976

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the Mantlible, .J. Am. 1)ent. Assoc. 76: 597-599, 1968. 18. Shafer. W. G. : Chronic Hclcrosine Osteomvelitis. J. Oral Surp. 15: 138-l-12. 1957. 19. Tow& T. L.: Chronic Diff’use &lerosingwOstedmyelitis of t%e Maxilla an>1 Mnndil)lc, J.

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27. Melrose. R. J.. Abramx. A. M.. and Nedleman. G.: Clinical PatholoPic Conferrncr No. 1X- Multipl;! Osseous Dyspiasia W&h Concomitant Simple Bone Cysts, ?. S. Calif. Dent. Assoc. 39: 386-389, 1971.

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O”, I

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Dysplasia of Bone, Cancrr 10: 1157-1161, 1957. 32. Jeffree, G. M.: Enzymes in Fihroblastie Lesions, J. Bone Joint Sure. 54B: 535-546. 1972. 33. Jaffe, ‘H. L.: Tumors and Tumorous Conditions of the Bones anvd Joints, Philadelphia,

1964. Lea & Febiger. n. 126. 34. Waldron, C. A.: goli&ry (Hemorrhagic) Cyst of the Mandihle, O~ar, SURG. 7: 88-95, 1954. 35. Friedman, N. B., and Goldman, R. L.: Cementoma of Long Bones-An Extraenathic Odon-

togenic Tumor, Clin. Orthop. 6?: 243-248, 1969.

Reprilzt requests to : Dr. Raymond J. Melrose School of Dentistry University of Southern California 925 West 34th St. Los Angeles, Calif. 90007

florid osseous dysplasia

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