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Diagnosing Oral Disease Bony Enlargement of the left Maxilla
D I A G N O S I N G ORAL D I S E A S E
Bony enlargement of the left maxilla Kevin J. O’Connell, DDS VA case coordinators Robert R. Rhyne, DDS, MS Ralph W. Correli, DDS Robert M. Craig, DDS
A
. 46-year-old w hite m an came to the dental clinic w ith an asym ptom a tic s w e llin g of th e left p o s te rio r m axilla (Fig 1). The area had been slowly enlarging since first noticed by the patient several years earlier. Clini cal e x a m in a tio n d isclo sed a firm , nonulcerated, poorly circum scribed e n la rg e m e n t of th e left p o s te rio r maxilla. The patient was edentulous and wore dentures that had been m od ified to accommodate the expanding mass in the left m axilla. No other sig nificant oral abnormalities were ob served. A panoram ic radiograph was taken (Fig 2). The patient smoked approxim ately one package of cigarettes every two days and did not drink alcohol. His medical history was noncontributory. An incisional biopsy was performed.
Diagnosis The biopsy specim en showed loss of n orm al b o n y a rc h ite c tu re an d re placem ent by cellular fibrous stroma w ith ran d o m ly arranged, irreg u lar bony trabeculae, consistent w ith a fibro-osseous process (Fig 3,4). Clini cal and radiographic correlation led to the diagnosis of fibrous dysplasia. Fibrous dysplasia is a benign, rela tively common disease distinct from other fibro-osseous lesions. In most cases, the disease affects only one bone (monostotic type); however, m ultiple bones can be involved (polyostotic type), and systemic com plications, in cluding abnormal skin pigm entation, 340 ■ JADA, Vol. 102, M a rc h 1981
prem ature sexual developm ent, and h y p erthyroidism m ay develop (Al bright’s syndrome). The etiology is unknow n and no general agreem ent exists as to the pathogenesis of the condition. It is c o m m o n ly th o u g h t th a t fib r o u s dysplasia is not neoplastic in origin, b u t re p re s e n ts a p e c u lia r d e v e l opm ental anom aly, as proposed by Lichtenstein.1
Characteristics Fibrous dysplasia of the facial bones most frequently affects the maxilla, w ith painless asymmetry as the most com m on in itial m anifestation. The condition u sually develops in late childhood, early adolescence, or occa sionally in adulthood. It is generally believed that fibrous dysplasia ceases to enlarge at skeletal maturity, but ex pansion may continue, as in this case. There is no apparent predilection for either sex. Batsakis2 reports that exten sive fibrous dysplasia involving the facial bones is more common in blacks. Radiographically, the disease shows a sp e c tru m of a p p e a ra n c e s, from purely radiolucent to radiopaque, with any variation or com bination of these changes. The radiographic appearance depends on the am ount of fibrous tis sue and bone w ithin the lesion, ac c o rd in g to w h ic h th e d is e a s e is categorized as one of three general ra diographic types. In one type, the le sion appears as a w ell-defined u n ilocular or m ultilocular radiolucent
area; in another, the lesion is similar but has mottled opacities. The third type is significantly more opaque and less w ell d e fin e d , an d resem b les ground glass or orange peel in appear ance. Root resorption is not a common m anifestation; how ever, separation and movement of roots w ithin the le sion is not uncommon. Significant facial deformity with se vere m alocclusion is frequently seen in patients with the disease, especially w hen the m axilla is involved. A l though fibrous dysplasia is usually slow to grow, some cases have been reported in w hich the disease has pro gressed rapidly, acting in an aggres sive m anner that clinically mimicked a m alignancy.3 The mucosa overlying the lesion is generally normal in ap pearance and nonulcerated. In both types of fibrous dysplasia (monostotic and polyostotic), the le sions consist essentially of localized regions of replacem ent of bony archi tecture by extensive proliferation of fibrous tissue. Poorly formed and ran domly arranged cancellous trabeculae of bone occur th ro u g h th is tissue. These trabeculae are usually coarse woven bone, having no internal lamel lar stru ctu re. V ariable am ounts of lam ellar bone may occasionally be present; this is not considered incom patible w ith a diagnosis of fibrous dysplasia.4 Rimming of the trabeculae by osteoblasts is an uncom m on fea ture. The dom inant pathologic m an ifestations are bone resorption, fibrosis of marrow spaces, and substitution of
D IA G N O S I N G
ORAL
D IS E A S E
Fig 2 ■ Radiograph shows poorly defined radiopacity with ground glass appearance.
*** Fig 1
II p Patient had asymptomatic swelling on left posterior maxilla.
Fig 4 ■ Small randomly arranged trabeculae are composed of woven bone.
Fig 3 ■ Normal bony architecture is replaced by fibrous stroma and irregu lar trabeculae.
p o o rly form ed an d d iso rd e rly a r ranged cancellous trabeculae for the original cancellous and cortical bone. W hen fibrous dysplasia appears as a well defined radiolucent area, it m ust be differentiated from other diseases w ith a sim ilar radiographic conforma tio n , in c lu d in g c e n tra l g ia n t cell g ran u lo m a , a m elo b lastic fibrom a, odontogenic cyst, and ameloblastoma. The m ixed-density lesion m ust be dif ferentiated from other radiopaque and ra d io lu c e n t lesions th a t affect th e bones of the jaw, for example, ossify in g fib ro m a , o s s e o u s d y s p la s ia ,
c h ro n ic s c le ro sin g o ste o m y e litis, am eloblastic fibro-odontoma, amelo blastic odontoma, and osteosarcoma. The densely sclerotic, ill-defined le sion w ith a ground glass appearance is m o re c h a r a c t e r i s t i c o f f ib r o u s dysplasia; however, exam ination of a biopsy specim en m ust confirm the clinicoradiologic impression. Conservative osseous contouring is recom m ended for functional or cosme tic reasons in the larger lesions with d iffu se , b le n d in g m a rg in s .5 T h is treatm ent should, if at all possible, be postponed until after lesional growth
subsides. If continued growth is expe rienced after conservative treatment, periodic contouring may be performed until a static phase is reached. Resec tion may be required for the aggres sive, rapidly growing lesion. Radia tion therapy is contraindicated be cause of the possibility of inducing malignant transform ation.6
Summary A swelling in the left m axilla of a 46year-old man, w hich had been enlarg ing for several years, was diagnosed as fibrous dysplasia. The disease usually affects only one bone and the lesion is benign; however, it can involve m ulti ple bones and systemic com plications may develop. A diagnosis of fibrous
O ’C onnell : BONY ENLARGEM ENT O F MAXILLA ■ 341
D I A G N O S IN G
ORAL
D IS E A S E
dysplasia, indicated by radiographic evaluation, should be confirm ed by biopsy examination.
The opinions and assertions contained herein are the private ones of the Oral Disease Project personnel and are not to be construed as official or reflecting the views of the Veterans Administra tion. This case was contributed by Dr. Kevin J. O’Connell, Dental Service, Veterans Administra tion Medical Center, Asheville, NC.
Dr. R hyne, d irecto r of the pro ject Oral Symptomatology and Its Relationship to Sys temic Disease, is also chief, Dental Service, Audie L. Murphy Memorial Veterans Hospital, San An tonio, Tex. Dr. Correll, project codirector, is pro ject registrar at the Veterans Administration Med ical Center, Long Beach, Calif. Dr. Craig is project oral diagnostician and coordinator of project field operations at the Veterans Administration Hospi tal, San Antonio, Tex, 78284. Address requests for reprints to Dr. Craig. 1. Lichtenstein, L., and Jaffe, H.L. Fibrous dysplasia of bone. Arch Pathol 33:777,1942.
Health science on stamps
2. Batsakis, J.G. Tumors of the head and neck (ed 2). Baltimore, Williams & Wilkins Co., 1979, pp 400-402. 3. Schofield, I.D. An aggressive fibrous dysplasia. Oral Surg 38:29-35, 1974. 4. Waldron, C.A., and Ciansanti, J.S. Benign fibro-osseous lesions of the jaws: a clinicalradiologic-histologic review of sixty-five cases. Oral Surg 35:190-201, 1973. 5. Eversole, L.R.; Sabes, W.R.; and Rovin, S. Fibrous dysplasia: a nosologic problem in the diagnosis of fibro-osseous lesions of the jaws. J Oral Pathol 1:189-220, 1972. 6. Schwartz, D.T., and Alpert, M. The malig nant transformation of fibrous dysplasia. Am J Med Sei 247:1-20,1964.
Commemorative cover
P
X enicillin is undoubtedly one of the m ost frequently used drugs in dentistry and medicine. It is not surprising, therefore, that a num ber of stam ps have been issued all over the world either honor ing Sir Alexander Fleming or publicizing penicillin. Sir Alexander Fleming was bom in Lochfield, Ayrshire, Scotland, on Aug 8,1881. He was educated at Kilmarnock and St. Mary’s Hos pital M edical School, U niversity of London. On graduation in m edicine in 1908, he became interested in bacteriology and did re search work under Sir Alm roth Wright. During World War I, Flem ing served in the Royal Army Medical Corps and discovered the an tiseptic properties of lysosyme. In 1928, w hile continuing his re search on antibacterial substances at St. M ary’s Hospital, Fleming noticed a m old, later to be identified as Pénicillium notatum, w hich interfered w ith and dissolved cultures of staphylococci. Fleming showed that this mold produced a substance that was capable of de stroying certain microorganism s. These experim ents were pub lished in the Journal of Experimental Pathology in 1929. Eleven years later, the biochemists Howard W alter Florey and Ernst Boris Chain were able to perfect the m ethods of production of penicillin. Fleming, Florey, and Chain shared the Nobel prize for M edicine in 1945, one year after Fleming and Florey had been knighted, Fleming died on March 11,1955, in London. Besides the stamps issued by M auritius and Benin in 1978 (JADA 99(3):534, 1979), several other countries have issued stamps honor ing Fleming and the discovery of penicillin. Great Britain issued one stamp in 1967, as did Togo in 1969; Nicaragua in 1973; Congo Re public in 1979; Mali in 1975; and the Comoro Islands in 1978, among others. On May 12, 1980, Mali issued a new 200F stamp com memorating Fleming, showing Fleming against a background of microbiology laboratory equipment. Hannelore T. Loevy, CD, PhD Aletha Kowitz, MA
342 ■ JADA, Vol. 102, M a rc h 1981
T
.JL he fiftieth anniversary of the establishm ent on July 1, 1930, of the Dental Branch of the Royal Air Force was being celebrated last year. For the occasion, a com memorative cover was prepared using an illustration based on a line drawing done in 1942 on the W estern Desert by the now Group C ap tain J. B. K arren. R egular covers, pilot signed covers, and covers flown by the Red Arrows and signed by the Director of the D ental B ranch of the RAF are available from Wing Commander J. C. Gray, Dental Centre, Royal Air Force, Cosford, W olverham pton WV7 3EX, England.
Bony enlargement of the left maxilla Kevin J. O’Connell, DDS VA case coordinators Robert R. Rhyne, DDS, MS Ralph W. Correli, DDS Robert M. Craig, DDS
A
. 46-year-old w hite m an came to the dental clinic w ith an asym ptom a tic s w e llin g of th e left p o s te rio r m axilla (Fig 1). The area had been slowly enlarging since first noticed by the patient several years earlier. Clini cal e x a m in a tio n d isclo sed a firm , nonulcerated, poorly circum scribed e n la rg e m e n t of th e left p o s te rio r maxilla. The patient was edentulous and wore dentures that had been m od ified to accommodate the expanding mass in the left m axilla. No other sig nificant oral abnormalities were ob served. A panoram ic radiograph was taken (Fig 2). The patient smoked approxim ately one package of cigarettes every two days and did not drink alcohol. His medical history was noncontributory. An incisional biopsy was performed.
Diagnosis The biopsy specim en showed loss of n orm al b o n y a rc h ite c tu re an d re placem ent by cellular fibrous stroma w ith ran d o m ly arranged, irreg u lar bony trabeculae, consistent w ith a fibro-osseous process (Fig 3,4). Clini cal and radiographic correlation led to the diagnosis of fibrous dysplasia. Fibrous dysplasia is a benign, rela tively common disease distinct from other fibro-osseous lesions. In most cases, the disease affects only one bone (monostotic type); however, m ultiple bones can be involved (polyostotic type), and systemic com plications, in cluding abnormal skin pigm entation, 340 ■ JADA, Vol. 102, M a rc h 1981
prem ature sexual developm ent, and h y p erthyroidism m ay develop (Al bright’s syndrome). The etiology is unknow n and no general agreem ent exists as to the pathogenesis of the condition. It is c o m m o n ly th o u g h t th a t fib r o u s dysplasia is not neoplastic in origin, b u t re p re s e n ts a p e c u lia r d e v e l opm ental anom aly, as proposed by Lichtenstein.1
Characteristics Fibrous dysplasia of the facial bones most frequently affects the maxilla, w ith painless asymmetry as the most com m on in itial m anifestation. The condition u sually develops in late childhood, early adolescence, or occa sionally in adulthood. It is generally believed that fibrous dysplasia ceases to enlarge at skeletal maturity, but ex pansion may continue, as in this case. There is no apparent predilection for either sex. Batsakis2 reports that exten sive fibrous dysplasia involving the facial bones is more common in blacks. Radiographically, the disease shows a sp e c tru m of a p p e a ra n c e s, from purely radiolucent to radiopaque, with any variation or com bination of these changes. The radiographic appearance depends on the am ount of fibrous tis sue and bone w ithin the lesion, ac c o rd in g to w h ic h th e d is e a s e is categorized as one of three general ra diographic types. In one type, the le sion appears as a w ell-defined u n ilocular or m ultilocular radiolucent
area; in another, the lesion is similar but has mottled opacities. The third type is significantly more opaque and less w ell d e fin e d , an d resem b les ground glass or orange peel in appear ance. Root resorption is not a common m anifestation; how ever, separation and movement of roots w ithin the le sion is not uncommon. Significant facial deformity with se vere m alocclusion is frequently seen in patients with the disease, especially w hen the m axilla is involved. A l though fibrous dysplasia is usually slow to grow, some cases have been reported in w hich the disease has pro gressed rapidly, acting in an aggres sive m anner that clinically mimicked a m alignancy.3 The mucosa overlying the lesion is generally normal in ap pearance and nonulcerated. In both types of fibrous dysplasia (monostotic and polyostotic), the le sions consist essentially of localized regions of replacem ent of bony archi tecture by extensive proliferation of fibrous tissue. Poorly formed and ran domly arranged cancellous trabeculae of bone occur th ro u g h th is tissue. These trabeculae are usually coarse woven bone, having no internal lamel lar stru ctu re. V ariable am ounts of lam ellar bone may occasionally be present; this is not considered incom patible w ith a diagnosis of fibrous dysplasia.4 Rimming of the trabeculae by osteoblasts is an uncom m on fea ture. The dom inant pathologic m an ifestations are bone resorption, fibrosis of marrow spaces, and substitution of
D IA G N O S I N G
ORAL
D IS E A S E
Fig 2 ■ Radiograph shows poorly defined radiopacity with ground glass appearance.
*** Fig 1
II p Patient had asymptomatic swelling on left posterior maxilla.
Fig 4 ■ Small randomly arranged trabeculae are composed of woven bone.
Fig 3 ■ Normal bony architecture is replaced by fibrous stroma and irregu lar trabeculae.
p o o rly form ed an d d iso rd e rly a r ranged cancellous trabeculae for the original cancellous and cortical bone. W hen fibrous dysplasia appears as a well defined radiolucent area, it m ust be differentiated from other diseases w ith a sim ilar radiographic conforma tio n , in c lu d in g c e n tra l g ia n t cell g ran u lo m a , a m elo b lastic fibrom a, odontogenic cyst, and ameloblastoma. The m ixed-density lesion m ust be dif ferentiated from other radiopaque and ra d io lu c e n t lesions th a t affect th e bones of the jaw, for example, ossify in g fib ro m a , o s s e o u s d y s p la s ia ,
c h ro n ic s c le ro sin g o ste o m y e litis, am eloblastic fibro-odontoma, amelo blastic odontoma, and osteosarcoma. The densely sclerotic, ill-defined le sion w ith a ground glass appearance is m o re c h a r a c t e r i s t i c o f f ib r o u s dysplasia; however, exam ination of a biopsy specim en m ust confirm the clinicoradiologic impression. Conservative osseous contouring is recom m ended for functional or cosme tic reasons in the larger lesions with d iffu se , b le n d in g m a rg in s .5 T h is treatm ent should, if at all possible, be postponed until after lesional growth
subsides. If continued growth is expe rienced after conservative treatment, periodic contouring may be performed until a static phase is reached. Resec tion may be required for the aggres sive, rapidly growing lesion. Radia tion therapy is contraindicated be cause of the possibility of inducing malignant transform ation.6
Summary A swelling in the left m axilla of a 46year-old man, w hich had been enlarg ing for several years, was diagnosed as fibrous dysplasia. The disease usually affects only one bone and the lesion is benign; however, it can involve m ulti ple bones and systemic com plications may develop. A diagnosis of fibrous
O ’C onnell : BONY ENLARGEM ENT O F MAXILLA ■ 341
D I A G N O S IN G
ORAL
D IS E A S E
dysplasia, indicated by radiographic evaluation, should be confirm ed by biopsy examination.
The opinions and assertions contained herein are the private ones of the Oral Disease Project personnel and are not to be construed as official or reflecting the views of the Veterans Administra tion. This case was contributed by Dr. Kevin J. O’Connell, Dental Service, Veterans Administra tion Medical Center, Asheville, NC.
Dr. R hyne, d irecto r of the pro ject Oral Symptomatology and Its Relationship to Sys temic Disease, is also chief, Dental Service, Audie L. Murphy Memorial Veterans Hospital, San An tonio, Tex. Dr. Correll, project codirector, is pro ject registrar at the Veterans Administration Med ical Center, Long Beach, Calif. Dr. Craig is project oral diagnostician and coordinator of project field operations at the Veterans Administration Hospi tal, San Antonio, Tex, 78284. Address requests for reprints to Dr. Craig. 1. Lichtenstein, L., and Jaffe, H.L. Fibrous dysplasia of bone. Arch Pathol 33:777,1942.
Health science on stamps
2. Batsakis, J.G. Tumors of the head and neck (ed 2). Baltimore, Williams & Wilkins Co., 1979, pp 400-402. 3. Schofield, I.D. An aggressive fibrous dysplasia. Oral Surg 38:29-35, 1974. 4. Waldron, C.A., and Ciansanti, J.S. Benign fibro-osseous lesions of the jaws: a clinicalradiologic-histologic review of sixty-five cases. Oral Surg 35:190-201, 1973. 5. Eversole, L.R.; Sabes, W.R.; and Rovin, S. Fibrous dysplasia: a nosologic problem in the diagnosis of fibro-osseous lesions of the jaws. J Oral Pathol 1:189-220, 1972. 6. Schwartz, D.T., and Alpert, M. The malig nant transformation of fibrous dysplasia. Am J Med Sei 247:1-20,1964.
Commemorative cover
P
X enicillin is undoubtedly one of the m ost frequently used drugs in dentistry and medicine. It is not surprising, therefore, that a num ber of stam ps have been issued all over the world either honor ing Sir Alexander Fleming or publicizing penicillin. Sir Alexander Fleming was bom in Lochfield, Ayrshire, Scotland, on Aug 8,1881. He was educated at Kilmarnock and St. Mary’s Hos pital M edical School, U niversity of London. On graduation in m edicine in 1908, he became interested in bacteriology and did re search work under Sir Alm roth Wright. During World War I, Flem ing served in the Royal Army Medical Corps and discovered the an tiseptic properties of lysosyme. In 1928, w hile continuing his re search on antibacterial substances at St. M ary’s Hospital, Fleming noticed a m old, later to be identified as Pénicillium notatum, w hich interfered w ith and dissolved cultures of staphylococci. Fleming showed that this mold produced a substance that was capable of de stroying certain microorganism s. These experim ents were pub lished in the Journal of Experimental Pathology in 1929. Eleven years later, the biochemists Howard W alter Florey and Ernst Boris Chain were able to perfect the m ethods of production of penicillin. Fleming, Florey, and Chain shared the Nobel prize for M edicine in 1945, one year after Fleming and Florey had been knighted, Fleming died on March 11,1955, in London. Besides the stamps issued by M auritius and Benin in 1978 (JADA 99(3):534, 1979), several other countries have issued stamps honor ing Fleming and the discovery of penicillin. Great Britain issued one stamp in 1967, as did Togo in 1969; Nicaragua in 1973; Congo Re public in 1979; Mali in 1975; and the Comoro Islands in 1978, among others. On May 12, 1980, Mali issued a new 200F stamp com memorating Fleming, showing Fleming against a background of microbiology laboratory equipment. Hannelore T. Loevy, CD, PhD Aletha Kowitz, MA
342 ■ JADA, Vol. 102, M a rc h 1981
T
.JL he fiftieth anniversary of the establishm ent on July 1, 1930, of the Dental Branch of the Royal Air Force was being celebrated last year. For the occasion, a com memorative cover was prepared using an illustration based on a line drawing done in 1942 on the W estern Desert by the now Group C ap tain J. B. K arren. R egular covers, pilot signed covers, and covers flown by the Red Arrows and signed by the Director of the D ental B ranch of the RAF are available from Wing Commander J. C. Gray, Dental Centre, Royal Air Force, Cosford, W olverham pton WV7 3EX, England.