Benign fibro-osseous lesions in Paget’s disease of the jaws Rosario Carrillo, MD,a Antonio Morales, IUD,~ Jose Luis Rodriguez-Peralto, Julio Lizama,d and Jo& M. Eslava.d Madrid, Spain HOSPITAL
RAM6N
Y CAJAL AND HOSPITAL
MD,c
12 DE OCTUBRE
A clinicopathologic study of five patients with Paget’s disease of the jaws is reported. All patients had well-documented cases of osteitis deformans with polyostotic involvement. Histologic studies of facial bones could be performed because surgery was necessary for either cosmetic or functional reasons. Three specimens consisted of maxillary bone, and two were mandibular. Microscopically, all cases showed multiple and irregular fibroproliferative lesions containing mineraked structures intermingled with bone characteristic of Paget’s disease. The purpose of this article is to present these fibro-osseous lesions in relation to Paget’s disease of the jaws and to emphasize their close resemblance to other benign fibro-osseous lesions of the jaws, especially cementifying and ossifying fibroma, and florid osseous dysplasia. (ORAL SURC ORAL MED ORAL PATHOL
1991;71:588-92)
P
aget’s disease of bone (osteitis deformans) is a chronic disorder of unknown etiology in which lesions are located in one or more sites of the skeleton.” 2 Ultrastructural studies have shown specific intranuclear inclusions in osteoclasts closely resembling viral nucleocapsids.3,4 Immunohistochemical techniques3,4 and in situ hybridization procedures5 strongly support the association of paramyxovirus with Paget’s diseaseof bone.6,7 High rates of bone resorption, bone formation, and marrow fibrosis are characteristic of Paget’s disease. This excessivebone turnover is reflected by increased urinary excretion of collagen breakdown products (oligopeptides of hydroxyproline and hydroxylysine glycosides) and an increase in serum alkaline phosphatase.* Histologically, abnormal osteoblastic and osteoelastic activity in the involved bone leads to a gradual
Supported by a grant from Fondo de Investigaciones de la Seguridad Social, Madrid, Spain. aAssociate Pathologist, Department of Pathology, Hospital Ramon y Cajal. bAssociate Rheumatologist, Department of Rheumatology, Hospital Rambn y Cajal. CAssociate Pathologist, Department of Pathology, Hospital 12 de Octubre. dAssociate Surgeon, Department of Maxillofacial Surgery, Hospital Ramon y Cajal. 7114126332 588
alteration of the histologic appearance and to a considerable disorganization of osseoustrabeculae. In the initial stagesof the disease,increased osteoclastic activity with destruction of the original bone is observed. Later, as a result of high osteoblastic activity, abnormal hyperplasia occurs with formation of woven bone followed by new lamellar bone. Intertrabecular spaces are occupied with fibrotic tissue that replacesthe bone marrow. Later, massive thick trabeculae with disjointed lamellar patterns occur. A mosaic of cement lines is formed by an extensive resorption and reapposition for many cellular generations.9 There are numerous recent publications on etiologic and therapeutic aspectsof Paget’s disease; however, there has been a dearth of histopathologic reports since the classic descriptions were published. The purpose of this report is to comment on some peculiarities that have been observedin five casesof Paget’s diseaseinvolving the jaw bones. To our knowledge these histologic features have not been previously analyzed in a seriesof cases.Thesestriking changeshave so far been overlooked, probably becauseof the small number of Paget’s diseasespecimensin jaw bonesthat are available for histopathologic study. MATERIAL
AND
METHODS
Five cases of Paget’s disease of bone with jaw involvement were selected from our files. All patients underwent surgical correction of their conditions for either cosmetic or functional reasons. A total of five
Fibro-osseous lesions in Paget’s disease of jaw
Volume 7 1 Number 5
589
Table 1.Clinical characteristics of patients Case No.
Age W/=x
Alkaline phosphatase (W
1 2
42/M 50/F
2349 2664
3
41/F
2800
4 5
40/F 55/F
1900 490
Skeletal distribution* Maxilla, pelvis Maxilla, mandible, thoracic spine, pelvis, femur Maxilla, mandible, skull, pelvis, Maxilla, pelvis, femur Mandible, pelvis
*Locations of pagetic sites determined by scintigraphic
bone scans
Fig. 1. Panoramic radiograph of patient with progressive enlargement of mandible as result of Paget’s disease of bone. Note extensive radiopaque, purely sclerotic lesion involving entire mandible and small highly sclerotic foci (arrows).
surgical specimens and one biopsy specimen were available, with 2 to 21 paraffin blocks in each case. Hematoxylin-eosin and Masson’s trichrome staining was performed on all specimens. In all patients radiologic studies confirmed the existence of typical osseouslesions of Paget’s disease. Jaw roentgenograms, including routine and panoramic views, were available in all cases.Radiologic changesof the jaws were similar to those found in the rest of the skeleton.’ The lesions were classified into lytic, sclerotic, and mixed categories. Furthermore, the presenceof hypercementosis and sclerotic, irregular masseseither surrounding dental roots or without a specific location have been examined. RESULTS Clinical and radiologic
findings
The clinical data are summarized in Table I. At presentation the patients’ agesranged between 41 and 55 years. Four were women, and one was a man. In all patients Paget’s diseasewas detected not only in the jaws but in at least one other bone. In four of the five casesthe serum alkaline phosphataselevels were extremely high (Table I), indicating an accelerated rate of bone turnover. Facial disfigurement was the most prominent clinical manifestation in all cases. Progressive enlargement of the jaws resulted in spreading of the teeth, leading to abnormal dental occlusion. Pain was a symptom in three of our cases. The radiologic patterns were purely sclerotic in three cases(Fig. 1) and mixed in two. Hypercementosis was not observed; however, sclerotic massesdiffusely distributed or surrounding dental roots were evident in both cases with mandibular involvement (Figs. 1 and 2). Histopathologic
findings
Every case of Paget’s diseaseof the jaws had similar findings, with the classic appearance of osteitis deformans. Irregular trabeculae with variable
Fig. 2. Panoramic radiograph of jaws. Diffuse mixed, sclerotic, and radiolucent pattern of Paget’s diseaseis seen over mandibular bone. Furthermore, large, densely mineralized massesare seensurrounding dental roots. Their appearance and distribution are very similar to florid cemento-osseousdysplasia.
amounts of osteoblastic and osteoclastic activity and prominent mosaic pattern were evident in all cases. Significantly, bone with typical Paget’s disease changes contained multiple, irregular, and variablesized foci that consisted of a combination of fibrous tissue and calcified material. These foci ranged from microscopic sizes to 2 to 3 cm in diameter (Fig. 3). The calcified material had a broad spectrum of appearancesincluding small spheric or irregular calcifications with some resemblanceto psammomatous bodies (Figs. 4 and 5), and spheroid or curvilinear basophilic structures, either acellular (Fig. 6) or cellular with lacunae (Fig. 7). In someareas these structures seemedto coalesceinto round calcified trabeculae or conglomerate masses(Fig. 8). In yet other areas sclerotic masses and irregular coarse calcifications were observed. Both cases with mandibular involvement showed densely sclerotic lesions, which enclosed dental roots in one of the cases(Fig. 2). Microscopically, these lesions were sclerotic, with few haversian spacesor lacuriae (Fig. 9).
ORhI
SURCi
ORAL.
Mm
ORAL.
~A1‘tV.N
May 1991
Fig. 3. Low-magnification view of small fibro-osseouslesion in specimen of maxillary pagetic bone. Focus, which measures a few millimeters, is composed of numerous rounded calcifications. Around them are trabeculae with characteristic pagetic appearance. Note sharp boundary between focus and trabeculae. (Hematoxylin-eosin stain; original magnification, X25.)
Fig.
4. Photomicrograph of pagetic bone (top right) and
fibro-osseouslesion (bottom left). Irregular trabeculae with pronounced osteoblastic and osteoclastic activity are seenin loose fibroconnective tissue. Fibro-osseous lesion shows small spheric calcifications with some resemblance to psammomatous bodies. Sharp boundary can be observed. (Hematoxylin-eosin stain; original magnification, X 100.)
The fibrous connective tissue, containing calcified structures, also had a variable histologic appearance. Richly cellular areas with densely packed fibroblasts that whorled around mineralized particles were encountered in some areas (Figs. 4 and 7) whereas densely collagenized, sparsely cellular tissue resided between the basophilic calcifications in other areas (Fig. 10). In general, a sharp, well-outlined boundary was observed around the fibro-osseouslesions (Figs. 3 to 5). DISCUSSION
From a clinical standpoint the most striking finding in our series is the early age of the patients when symptoms were noted. This fact is unique in osteitis
Fig. 5. Photomicrograph of junction between pagetic bone (right) and fibro-osseous lesion (left). Latter shows small rounded and irregular calcifications, enclosed in richly cellular fibroblastic tissue. Broad trabeculae with characteristic pagetic appearanceare evident (right). (Hematoxylin-eosin stain; original magnification, X 100.)
Fig. 6. Microscopic fields of lesion composedby fibrous cellular stroma containing spheroid or curvilinear cementurn-like structures. Note that they are strongly basophilic and devoid of nuclei. (Hematoxylin-eosin stain; original magnification, X200.)
deformans, because most cases are not discovered until much later in life. We have also noticed this feature in some isolated reports of Paget’s diseaseof the jaws. lo-l4 Indeed, patients with Paget’s disease in other locations, generally diagnosed in later life, may actually have been much younger at the time of onset. Because of the lack of symptoms, the condition was not diagnosed at that time. The facial disfigurement and disturbances of dental occlusion that occur in Paget’s diseaseof the jaws could have contributed to earlier diagnosis among these patients. Histopathologic studies of Paget’s disease have been based mainly on extragnathic skeleton specimens, with little information on histomorphologic features of the jaws. However, some peculiarities of this condition involving the facial boneshave been established. The occurrence of giant cell tumors or giant cell (reparative) granulomas complicating Paget’s
Volume7 1 Number 5
7. Fibro-osseouslesion showing cellular fibroblastic stroma containing mineralized structures with cellular lacunae and intermediate osteocemental features. (Hematoxylin-eosin stain; original magnification, X250.) Fig.
Fig. 8. Photomicrograph of fibro-osseouslesion showing coarse, densely mineralized, irregular trabeculae with intermediate osteocementalappearance.(Hematoxylin-eosin stain; original magnification, X 100.)
disease of facial bones is infrequent but well recognized.15-‘7On the other hand, radiologic evidence of hypercementosis has been reported in numerous isolated cases of Paget’s disease of the jaws “3 I33I4 and has been considered a characteristic radiologic finding. I23‘*, I9 Few studies have detailed the histologic appearance of these foci of hypercementosis.‘*+2oNone of our casesshowed radiologic hypercementosis,but in two casessclerotic masseswere radiologically seen and histologically large, densely mineralized acellular massesnot unlike those seenin florid osseousdysplasia were encountered.2’*22 Admittedly, the histologic lesions observed in our five casesof Paget’s diseaseof the jaws closely resemble those described in a group of conditions collectively designated as fibro-osseouslesions of the jaws.21 This term includes numerous disorders, of which the most characteristic are fibrous dysplasia, periapical cemental dysplasia, florid osseousdysplasia, ossifying and cementifying fibroma, and cementoblastoma. A variety of diagnostic terms have been used for these
Fibro-osseous lesions in Paget’s disease of jaw
591
Fig. 9. Photomicrograph from biopsy specimen of patient with Paget’s diseaseof mandible shows densely mineralized sclerotic acellular masses.Pattern is suggestive of cementum, although it could be considered sclerotic bone. (Hematoxylin-eosin stain; original magnification, x250.)
Fig. 10. Microscopic view of hypocellular area in fibroosseouslesion. Spheroid or curvilinear calcifications are scattered in densely collagenized, hypocellular stroma. (Hematoxylin-eosin stain; original magnification, X100.)
lesions in the literature.2” 23The replacement of normal bone by a tissue composedof collagen fibers and fibroblasts is a common characteristic in all of them. This collagenous tissue contains varying amounts of mineralized substance that has been described as having an osseousor cementum-like appearancea2’ We could find only two previous reports in which other fibro-osseous lesions have complicated Paget’s diseaseof the jaws. “3 I3 In one of these reports” the authors describe an “atypical case” of osteitis deformans of the maxilla in which unusual histopathologic features were recounted. Foci that “resembled disease processesthat some investigators might designate as cystic cementifying fibroma, ossifying fibroma, or fibrous dysplasia” were detailed. The other report13 referred to an instance of monostotic Paget’s disease of the mandible in which progressive root resorption of several teeth occurred. In several specimensthese authors described foci of collagenous connective tissue with scattered, irregular, rounded, basophilic,
592
Carrillo et al.
mineralized structures, suggestive of cementifying fibroma. 13 Alternatively, Waldron24 pointed out that the microscopic diagnosis in early Paget’s diseasecan be difficult becausethe observedpicture may simulate the features of fibrous dysplasia. Thus the fibro-osseouslesions reported here obfuscate the microscopic diagnosis of Paget’s disease of the jaws. In fact, in two of our casesthe original diagnoses were periapical dysplasia and gigantiform cementoma. Only when the clinical and radiologic data were analyzed and the histologic features carefully reviewed was the diagnosis of Paget’s diseaseestablished. In another caseproblems arose at the time of frozen section biopsy, because the specimen consisted exclusively of fibrocemental, highly cellular tissue resembling cementifying and ossifying fibroma. Paget’s disease is not the only condition involving the jaws that sometimes shows an atypical microscopic appearance. Identical structures to those observed in our cases,with so-called cementurn-like appearance, have been described in fibrous dysplasia of the jaws,21,25long bones, and sku11.26 Becauseeffective medical treatment is available in Paget’s disease,an accurate diagnosis is necessaryfor correct management and prevention of complications. In summary, we have reported histologic features in Paget’s disease of the jaws that resemble other “fibro-osseous lesions.” Similar findings have been reported in the literature, and in our opinion these lesions should not be considered exceptional findings in Paget’s diseaseof the jaw bones; rather, they may represent a feature that has not been properly stressed.
ORAL SURG ORAL Mm ORAL. PATHOI~ May 1991
6.
7. 8. 9.
10. 11. 12. 13.
14.
15.
16.
17.
18. 19. 20. 21. 22. 23.
REFERENCES 1, Russel RGG. Paget’s disease. In: Nordin BEC, ed. Metabolic bone and stone disease. New York: Churchill Livingstone, 1984:190-233. 2. Singer FR, Schiller AL, Pyle EB, Krane SM. Paget’s disease of bone. In: Avioli LV, Krane SM, eds. Metabolic bone disease; vol 2. New York: Academic Press, 1978:489-575. 3. Mills BG, Singer FR, Weiner LP, Holst PA. Immunohistological demonstration of respiratory syncytial virus antigens in Paget’s disease of bone. Proc Nat1 Acad Sci USA 1981;78: 1209-13. 4. Rebel A, Basle M, Pouplard A, Malkani K, Filmon R, Lepatezour A. Bone tissue of Paget’s disease of bone: ultrastructure and immunocytology. Arthritis Rheum 1980;23:1104-14. 5. Basle MF, Fournier JG, Rozenblatt S, Rebel A, Bouteille M.
24. 25.
26.
Measles virus RNA detected in Paget’s disease bone tissue by in situ hybridization. J Gen Viral 1986;67:907- 13. Basle MF, Rebel A, Fournier JG, Ruse11WC, Malkani K. On the trail of paramyxoviruses in Paget’s diseases of bone. Clin Orthop 1987;217:9-15. Singer FR, Mills BG. Evidence for a viral etiology of Paget’s disease of bone. Clin Orthop 1983;178:245-5 1. Krane SM, Simon LS. Metabolic consequences of bone turnover in Paget’s disease of bone. Clin Orthop 1987:217:26-36. Milgram JW. Radiographical and pathological assessment of the activity of Paget’s disease of bone. Clin Orthop 1977: 127: 43-54. Akin RK, Barton K, Walters PJ. Paget’s disease of bone: report of a case. ORAL SURG ORAL MED ORAL PATHOI. 1975; 39:707-12. Kirby JW, Robinson ME. Osteitis deformans of the maxilla: report of atypical case. J Oral Surg 1973;3 1:64-70. Rao VM, Karasick D. Hypercementosis: an important clue to Paget’s disease of the maxilla. Skeletal Radio1 1982;9:126-8. Smith NHH. Monostotic Paget’s disease of the mandible presenting with progressive resorption of the teeth. ORAL SURG ORAL MED ORAL PATHOL 1978;46:246-53. Wiesenfeld D, Cook RM, Bennet KR, Radden BG. The correction of mandibular deformity due to Paget’s disease of bone. J Oral Maxillofac Surg 1985;43:125-9. Goldstein BH, Laskin DM. Giant cell tumor of the maxilla complicating Paget’s disease of bone. J Oral Surg 1974;32: 209-13. Miller AS, Cuttino CL, Elzay RP, Levy WM, Hazwick RD. Giant cell tumor of the jaws associated with Paget’s disease of bone. Arch Otolaryngol 1974;100:233-6. Upchurch KS, Simon LS, Schiller Al, Rosenthal Dl, Campion EW. Krane SM. Giant cell renarative aranuloma of Parret’s disease of bone: a unique clinical entzy. Ann Intern Med 1983;98:35-40. Lucas RB. The jaws and teeth in Paget’s disease of bone. J Clin Path01 1955;8:195-200. Tillman HH. Paget’s disease of bone. ORAL SURG ORAL MED ORAL PATHOL 1962;15:1225-34. Barry HC. Paget’s disease of bone. Edinburgh: Churchill Livingstone, 1969. Waldron CA. Fibro-osseous lesions of the jaws. J Oral Maxillofac Surg 1985;43:249-62. Melrose R, Abrams A, Mills B. Florid osseous dysplasia. ORAL SURG ORAL MED ORAL PATHOL 1976;41:62-82. Makek MS. So-called “tibro-osseous lesions” of tumorous origin. J Craniomaxillofac Surg 1987; 15: 154-67. Waldron CA. In: Tiecke RW. Oral pathology. New York: McGraw-Hill, 1965:563. Eversole LR, Sabes WR, Rovin S. Fibrous dysplasia: a nosologic problem in the diagnosis of fibro-osseous lesions of the jaws. J Oral Path01 1972;1:189-220. Dahlin DC. Bone tumors. Springfield, Ill: Charles C Thomas, 1978.
Reprint requests to: Rosario Carrillo, MD Servicio de Anatomia Patologica Hospital Ramon y Cajal Carretera de Colmenar Km 9,100 28043 Madrid, Spain
RAM6N
Y CAJAL AND HOSPITAL
MD,c
12 DE OCTUBRE
A clinicopathologic study of five patients with Paget’s disease of the jaws is reported. All patients had well-documented cases of osteitis deformans with polyostotic involvement. Histologic studies of facial bones could be performed because surgery was necessary for either cosmetic or functional reasons. Three specimens consisted of maxillary bone, and two were mandibular. Microscopically, all cases showed multiple and irregular fibroproliferative lesions containing mineraked structures intermingled with bone characteristic of Paget’s disease. The purpose of this article is to present these fibro-osseous lesions in relation to Paget’s disease of the jaws and to emphasize their close resemblance to other benign fibro-osseous lesions of the jaws, especially cementifying and ossifying fibroma, and florid osseous dysplasia. (ORAL SURC ORAL MED ORAL PATHOL
1991;71:588-92)
P
aget’s disease of bone (osteitis deformans) is a chronic disorder of unknown etiology in which lesions are located in one or more sites of the skeleton.” 2 Ultrastructural studies have shown specific intranuclear inclusions in osteoclasts closely resembling viral nucleocapsids.3,4 Immunohistochemical techniques3,4 and in situ hybridization procedures5 strongly support the association of paramyxovirus with Paget’s diseaseof bone.6,7 High rates of bone resorption, bone formation, and marrow fibrosis are characteristic of Paget’s disease. This excessivebone turnover is reflected by increased urinary excretion of collagen breakdown products (oligopeptides of hydroxyproline and hydroxylysine glycosides) and an increase in serum alkaline phosphatase.* Histologically, abnormal osteoblastic and osteoelastic activity in the involved bone leads to a gradual
Supported by a grant from Fondo de Investigaciones de la Seguridad Social, Madrid, Spain. aAssociate Pathologist, Department of Pathology, Hospital Ramon y Cajal. bAssociate Rheumatologist, Department of Rheumatology, Hospital Rambn y Cajal. CAssociate Pathologist, Department of Pathology, Hospital 12 de Octubre. dAssociate Surgeon, Department of Maxillofacial Surgery, Hospital Ramon y Cajal. 7114126332 588
alteration of the histologic appearance and to a considerable disorganization of osseoustrabeculae. In the initial stagesof the disease,increased osteoclastic activity with destruction of the original bone is observed. Later, as a result of high osteoblastic activity, abnormal hyperplasia occurs with formation of woven bone followed by new lamellar bone. Intertrabecular spaces are occupied with fibrotic tissue that replacesthe bone marrow. Later, massive thick trabeculae with disjointed lamellar patterns occur. A mosaic of cement lines is formed by an extensive resorption and reapposition for many cellular generations.9 There are numerous recent publications on etiologic and therapeutic aspectsof Paget’s disease; however, there has been a dearth of histopathologic reports since the classic descriptions were published. The purpose of this report is to comment on some peculiarities that have been observedin five casesof Paget’s diseaseinvolving the jaw bones. To our knowledge these histologic features have not been previously analyzed in a seriesof cases.Thesestriking changeshave so far been overlooked, probably becauseof the small number of Paget’s diseasespecimensin jaw bonesthat are available for histopathologic study. MATERIAL
AND
METHODS
Five cases of Paget’s disease of bone with jaw involvement were selected from our files. All patients underwent surgical correction of their conditions for either cosmetic or functional reasons. A total of five
Fibro-osseous lesions in Paget’s disease of jaw
Volume 7 1 Number 5
589
Table 1.Clinical characteristics of patients Case No.
Age W/=x
Alkaline phosphatase (W
1 2
42/M 50/F
2349 2664
3
41/F
2800
4 5
40/F 55/F
1900 490
Skeletal distribution* Maxilla, pelvis Maxilla, mandible, thoracic spine, pelvis, femur Maxilla, mandible, skull, pelvis, Maxilla, pelvis, femur Mandible, pelvis
*Locations of pagetic sites determined by scintigraphic
bone scans
Fig. 1. Panoramic radiograph of patient with progressive enlargement of mandible as result of Paget’s disease of bone. Note extensive radiopaque, purely sclerotic lesion involving entire mandible and small highly sclerotic foci (arrows).
surgical specimens and one biopsy specimen were available, with 2 to 21 paraffin blocks in each case. Hematoxylin-eosin and Masson’s trichrome staining was performed on all specimens. In all patients radiologic studies confirmed the existence of typical osseouslesions of Paget’s disease. Jaw roentgenograms, including routine and panoramic views, were available in all cases.Radiologic changesof the jaws were similar to those found in the rest of the skeleton.’ The lesions were classified into lytic, sclerotic, and mixed categories. Furthermore, the presenceof hypercementosis and sclerotic, irregular masseseither surrounding dental roots or without a specific location have been examined. RESULTS Clinical and radiologic
findings
The clinical data are summarized in Table I. At presentation the patients’ agesranged between 41 and 55 years. Four were women, and one was a man. In all patients Paget’s diseasewas detected not only in the jaws but in at least one other bone. In four of the five casesthe serum alkaline phosphataselevels were extremely high (Table I), indicating an accelerated rate of bone turnover. Facial disfigurement was the most prominent clinical manifestation in all cases. Progressive enlargement of the jaws resulted in spreading of the teeth, leading to abnormal dental occlusion. Pain was a symptom in three of our cases. The radiologic patterns were purely sclerotic in three cases(Fig. 1) and mixed in two. Hypercementosis was not observed; however, sclerotic massesdiffusely distributed or surrounding dental roots were evident in both cases with mandibular involvement (Figs. 1 and 2). Histopathologic
findings
Every case of Paget’s diseaseof the jaws had similar findings, with the classic appearance of osteitis deformans. Irregular trabeculae with variable
Fig. 2. Panoramic radiograph of jaws. Diffuse mixed, sclerotic, and radiolucent pattern of Paget’s diseaseis seen over mandibular bone. Furthermore, large, densely mineralized massesare seensurrounding dental roots. Their appearance and distribution are very similar to florid cemento-osseousdysplasia.
amounts of osteoblastic and osteoclastic activity and prominent mosaic pattern were evident in all cases. Significantly, bone with typical Paget’s disease changes contained multiple, irregular, and variablesized foci that consisted of a combination of fibrous tissue and calcified material. These foci ranged from microscopic sizes to 2 to 3 cm in diameter (Fig. 3). The calcified material had a broad spectrum of appearancesincluding small spheric or irregular calcifications with some resemblanceto psammomatous bodies (Figs. 4 and 5), and spheroid or curvilinear basophilic structures, either acellular (Fig. 6) or cellular with lacunae (Fig. 7). In someareas these structures seemedto coalesceinto round calcified trabeculae or conglomerate masses(Fig. 8). In yet other areas sclerotic masses and irregular coarse calcifications were observed. Both cases with mandibular involvement showed densely sclerotic lesions, which enclosed dental roots in one of the cases(Fig. 2). Microscopically, these lesions were sclerotic, with few haversian spacesor lacuriae (Fig. 9).
ORhI
SURCi
ORAL.
Mm
ORAL.
~A1‘tV.N
May 1991
Fig. 3. Low-magnification view of small fibro-osseouslesion in specimen of maxillary pagetic bone. Focus, which measures a few millimeters, is composed of numerous rounded calcifications. Around them are trabeculae with characteristic pagetic appearance. Note sharp boundary between focus and trabeculae. (Hematoxylin-eosin stain; original magnification, X25.)
Fig.
4. Photomicrograph of pagetic bone (top right) and
fibro-osseouslesion (bottom left). Irregular trabeculae with pronounced osteoblastic and osteoclastic activity are seenin loose fibroconnective tissue. Fibro-osseous lesion shows small spheric calcifications with some resemblance to psammomatous bodies. Sharp boundary can be observed. (Hematoxylin-eosin stain; original magnification, X 100.)
The fibrous connective tissue, containing calcified structures, also had a variable histologic appearance. Richly cellular areas with densely packed fibroblasts that whorled around mineralized particles were encountered in some areas (Figs. 4 and 7) whereas densely collagenized, sparsely cellular tissue resided between the basophilic calcifications in other areas (Fig. 10). In general, a sharp, well-outlined boundary was observed around the fibro-osseouslesions (Figs. 3 to 5). DISCUSSION
From a clinical standpoint the most striking finding in our series is the early age of the patients when symptoms were noted. This fact is unique in osteitis
Fig. 5. Photomicrograph of junction between pagetic bone (right) and fibro-osseous lesion (left). Latter shows small rounded and irregular calcifications, enclosed in richly cellular fibroblastic tissue. Broad trabeculae with characteristic pagetic appearanceare evident (right). (Hematoxylin-eosin stain; original magnification, X 100.)
Fig. 6. Microscopic fields of lesion composedby fibrous cellular stroma containing spheroid or curvilinear cementurn-like structures. Note that they are strongly basophilic and devoid of nuclei. (Hematoxylin-eosin stain; original magnification, X200.)
deformans, because most cases are not discovered until much later in life. We have also noticed this feature in some isolated reports of Paget’s diseaseof the jaws. lo-l4 Indeed, patients with Paget’s disease in other locations, generally diagnosed in later life, may actually have been much younger at the time of onset. Because of the lack of symptoms, the condition was not diagnosed at that time. The facial disfigurement and disturbances of dental occlusion that occur in Paget’s diseaseof the jaws could have contributed to earlier diagnosis among these patients. Histopathologic studies of Paget’s disease have been based mainly on extragnathic skeleton specimens, with little information on histomorphologic features of the jaws. However, some peculiarities of this condition involving the facial boneshave been established. The occurrence of giant cell tumors or giant cell (reparative) granulomas complicating Paget’s
Volume7 1 Number 5
7. Fibro-osseouslesion showing cellular fibroblastic stroma containing mineralized structures with cellular lacunae and intermediate osteocemental features. (Hematoxylin-eosin stain; original magnification, X250.) Fig.
Fig. 8. Photomicrograph of fibro-osseouslesion showing coarse, densely mineralized, irregular trabeculae with intermediate osteocementalappearance.(Hematoxylin-eosin stain; original magnification, X 100.)
disease of facial bones is infrequent but well recognized.15-‘7On the other hand, radiologic evidence of hypercementosis has been reported in numerous isolated cases of Paget’s disease of the jaws “3 I33I4 and has been considered a characteristic radiologic finding. I23‘*, I9 Few studies have detailed the histologic appearance of these foci of hypercementosis.‘*+2oNone of our casesshowed radiologic hypercementosis,but in two casessclerotic masseswere radiologically seen and histologically large, densely mineralized acellular massesnot unlike those seenin florid osseousdysplasia were encountered.2’*22 Admittedly, the histologic lesions observed in our five casesof Paget’s diseaseof the jaws closely resemble those described in a group of conditions collectively designated as fibro-osseouslesions of the jaws.21 This term includes numerous disorders, of which the most characteristic are fibrous dysplasia, periapical cemental dysplasia, florid osseousdysplasia, ossifying and cementifying fibroma, and cementoblastoma. A variety of diagnostic terms have been used for these
Fibro-osseous lesions in Paget’s disease of jaw
591
Fig. 9. Photomicrograph from biopsy specimen of patient with Paget’s diseaseof mandible shows densely mineralized sclerotic acellular masses.Pattern is suggestive of cementum, although it could be considered sclerotic bone. (Hematoxylin-eosin stain; original magnification, x250.)
Fig. 10. Microscopic view of hypocellular area in fibroosseouslesion. Spheroid or curvilinear calcifications are scattered in densely collagenized, hypocellular stroma. (Hematoxylin-eosin stain; original magnification, X100.)
lesions in the literature.2” 23The replacement of normal bone by a tissue composedof collagen fibers and fibroblasts is a common characteristic in all of them. This collagenous tissue contains varying amounts of mineralized substance that has been described as having an osseousor cementum-like appearancea2’ We could find only two previous reports in which other fibro-osseous lesions have complicated Paget’s diseaseof the jaws. “3 I3 In one of these reports” the authors describe an “atypical case” of osteitis deformans of the maxilla in which unusual histopathologic features were recounted. Foci that “resembled disease processesthat some investigators might designate as cystic cementifying fibroma, ossifying fibroma, or fibrous dysplasia” were detailed. The other report13 referred to an instance of monostotic Paget’s disease of the mandible in which progressive root resorption of several teeth occurred. In several specimensthese authors described foci of collagenous connective tissue with scattered, irregular, rounded, basophilic,
592
Carrillo et al.
mineralized structures, suggestive of cementifying fibroma. 13 Alternatively, Waldron24 pointed out that the microscopic diagnosis in early Paget’s diseasecan be difficult becausethe observedpicture may simulate the features of fibrous dysplasia. Thus the fibro-osseouslesions reported here obfuscate the microscopic diagnosis of Paget’s disease of the jaws. In fact, in two of our casesthe original diagnoses were periapical dysplasia and gigantiform cementoma. Only when the clinical and radiologic data were analyzed and the histologic features carefully reviewed was the diagnosis of Paget’s diseaseestablished. In another caseproblems arose at the time of frozen section biopsy, because the specimen consisted exclusively of fibrocemental, highly cellular tissue resembling cementifying and ossifying fibroma. Paget’s disease is not the only condition involving the jaws that sometimes shows an atypical microscopic appearance. Identical structures to those observed in our cases,with so-called cementurn-like appearance, have been described in fibrous dysplasia of the jaws,21,25long bones, and sku11.26 Becauseeffective medical treatment is available in Paget’s disease,an accurate diagnosis is necessaryfor correct management and prevention of complications. In summary, we have reported histologic features in Paget’s disease of the jaws that resemble other “fibro-osseous lesions.” Similar findings have been reported in the literature, and in our opinion these lesions should not be considered exceptional findings in Paget’s diseaseof the jaw bones; rather, they may represent a feature that has not been properly stressed.
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Reprint requests to: Rosario Carrillo, MD Servicio de Anatomia Patologica Hospital Ramon y Cajal Carretera de Colmenar Km 9,100 28043 Madrid, Spain
