Brirish 0
lournal
1991 The
of Oral and Maxi//ofana/ Surgery (IWI) British
Association
Gigantiform Kyiisti Oikarinen,
29, 194-197 Surgeons
of Oral and Maxillofacial
cementoma Mikko Altonen,
affecting a Caucasian Risto-Pekka
family
Happonen
Departments of Oral and Maxillofacial Surgery, Institute of Dentistry, Institute of Dentistry, University of Turku, Finland
University of Oulu, and
A Caucasian family is presented in which two out of three children showed lesions resembling SUMMARY. gigantiform cementoma clinically, radiologically and histopathologically. Both affected children had swellings of the alveolar crest, caused by large, lobular, radio-opaque masses in all four quadrants of the jaws. Histologically, cementum-like masses were surrounded by inflamed fibrous connective tissue. There was evidence indicating that the late father had similar lesions which became malignant following radiation therapy.
1987). Dense, cementurn-like acellular hard tissue surrounded by connective tissue is seen histologically (Van der Waal & Van der Kwast, 1974; Dominquez et al., 1987; Young et al., 1989). The condition has been considered familial, being found mostly in blacks (Van der Waal & Van der Kwast, 1974; Lyons & Babajews, 1986), although opposite opinions have also been presented (Punniamoorthy, 1980). Only few reports exist in which the hereditary nature of the lesion has been proven (Agazzi & Belloni, 1953; Young et al., 1989). This study reports a Caucasian family in which gigantiform cementoma was diagnosed in two out of three children as well as in the late father, whose lesions had been treated as mandibular malignancy several decades previously.
INTRODUCTION
In the classification of fibro-osseous lesions by Waldron (1985), gigantiform cementoma is placed among those arising in the periodontal ligament. Classification of cementomas introduced by Pindborg et al. (1971) differentiates between benign cementoblastoma, cementifying fibroma, periapical cemental dysplasia and gigantiform cementoma. Gigantiform cementoma, also called familial multiple cementoma and florid cemento-osseous dysplasia, has been often misdiagnosed as sclerosing osteomyelitis, osseous dysplasia and bony enostosis (Bhaskar & Cutright, 1968; Waldron et al., 1975; Wolf et al., 1989) due to lack of specific diagnostic methods and the rarity of this lesion. Gigantiform cementoma is reported to have a racial and sex predilection affecting mainly middle-aged Negro women (Gorlin & Goldman, 1970). It is essentially a benign condition (Winer et al., 1972) and must therefore be differentiated from Paget’s disease which may sometimes have radiological similarities but can undergo malignant transformation (Rosenmertz & Schare, 1969). Gigantiform cementoma has been speculated as originating from remnants of cementum left within the bone after tooth extraction (Punniamoorthy, 1980). Deposition of cementum can impair the vascularity of the bone which can predispose to osteomyelitis (Van der Waal & Van der Kwast, 1974). Even though it is difficult to differentiate the origin of the hard tissue in various fibro-osteocemental lesions (Burkhardt, 1989), the diagnosis of gigantiform cementoma can be confirmed on the basis of clinical, radiological and histological findings (Young et al., 1989). Swelling of the jaw bones can cause facial deformity (Young et al., 1989) which may be the only complaint of the patient (Lucas, 1984). Irregular, often lobular radio-opacities are seen symmetrically in the mandible and maxilla, most typically in the molar regions (Punniamoorthy, 1980; Lyons & Babajews, 1986; Dominquez et al.,
Case reports V.S. (The father):
In 1933 at the age of 17 years an expansive, radiologically sclerotic tumour in the left mandible was found, excised
Fig. I- Radiograph of the father’s mandible taken in connection with radiation therapy for tonsillary adenocarcinoma at the age of 64 years (the metallic pins are seen). Lesions resembling gigantiform cementoma affect most of the body of the mandible (Arrows). M = mandibular angle. 194
Gieantiform and irradiated because malignancy was suspected. No histopathological specimens or radiographs are available fro? the operation, but an X-ray taken shortly before his death show lesions resembling gigantiform cementoma (Fig. 1). Nine years after the primiry treatment, osteosarcoma arose at the site of the original tumour. The lesion was treated by mandibular resection and bone grafting. Eighteen years after the radiation a squamous cell carcinoma of the left ear was found and treated by irradiation. Basal cell carcinoma of the chin, carcinoma in situ of the glottis and tonsillar adenocarcinoma were diagnosed 31, 37 and 48 years after the first radiation. A brain tumour (detailed diagnosis unknown) was the final cause of death at the age of 65 years. E. H. (The first daughter): A 2%year-old woman suffering from insulin dependent diabetes since the age of 12 years had her first operation because of a firm swelling in the maxilla. Histologically, cementurn-like masses were found within connective tissue. Three years later a recurrence was excised from the same region. The patient was referred to the Department of Oral and Maxillofacial Surgery, Institute of Dentistry, University of Oulu, 7 years after the first operation. Clinically, an alveolar swelling was found on the buccal side of the left maxillary molar region. A radiograph revealed a lobular area of radio-opacity around the left maxillary molars. Advanced periodontitis and a periapical radiolucency were seen around the non-vital left mandibular second molar (Fig. 2A). The tooth was extracted under local anaesthesia. Four years later at the age of 40 years, the patient returned to the department because of swelling of the left side of the mandible and maxilla. The lesion involved all maxillary molars. In the left mandibular molar region it extended to the lower border of the mandible. Clinically and radiologically distal displacement of the upper left second molar was evident. At review 1 year later the patient was still symptom-free apart from the swelling and refused any further surgery because of a recent diagnosis of a brain tumour (Schwannoma). Further ossification of the mandibular lesion was evident on the orthopantomogram (Fig. 2B). A biopsy taken from the periapical lesion around the lower left second molar supported a cemental origin of the lesion. Cementurn-like masses with rounded margins were surrounded by fibrous connective tissue containing mononuclear inflammatory cells, active fibroblasts and immature collagen (Fig. 3).
cementoma
affecting a Caucasian familv
195
Fig. 2B - Elongation and distal displacement of maxillary left molars as well as bilateral enlargement of the lesions in the maxilla can be seen in the orthopantomograph taken five years after the radiograph in Figure 2A. Note also further ossification of the mandibular lesion in the left mandible.
Fig. 3 - Photomicrograph of the biopsy from the periapical area of the mandibular second molar of the first daughter showing cellular connective tissue with spherical hard tissue particles resembling cementum. (H & E. Original magnification x320).
Fig. 4 - Orthopantomograph
of the son with edentulous maxilla and partially dentate mandible affected by large lesions resembling gigantiform cementoma.
R.S. (The son):
Orthopantomograph of the first daughter demonstrating lobular radio-opaque areas in the maxillary left molar region and
Fig. 2A -
periapical molar.
radiolucency
affecting the left mandibular
second
At the age of 14 years, the son had his first operation performed in the left mandibular molar region. The histopathological diagnosis was fibrous dysplasia. Ten years later, a recurrence was operated upon, and the histopathological diagnosis was cementifying fibroma. At the age of 27 years, a third operation was performed at the same site and the excised tissue was reported histologically
196
British
Journal
of Oral
and Maxillofacial
Surgery
Fig. 5 - Scintigraphy of the son in postero-anterior uptake of ~~ Tc bilaterally
(A) and lateral projections from the right (B) and left (C) side demonstrate in the maxilla and mandible, especially in the left side. (Dex = right, sin = left).
Fig. 6 - PhotuwLLug mdible of the son s
rounded by IGIll
as fibrous dysplasia. Ten years after the third operation the patient was referred to a maxillofacial surgeon. The patient indicated that the lesion had been growing slowly and had caused only slight discomfort. A painless swelling was noted in the left mandibular molar region. Radiographs showed that the edentulous maxilla and partially dentate mandible were affected by large sclerotic lesions (Fig. 4). Scintigraphy with WmTc showed increased uptake bilaterally in the maxilla and mandible especially in the left side (Fig. 5). The shape of the mandible was corrected surgically under local anaesthesia. Histopathological findings were essentially similar to those found in the biopsy of the sister (Fig. 6). M.M. (The second daughter):
The third child of the family, a daughter, was examined clinically and radiographically at the age of 42 years, but she was found to be free of any jaw diseases.
DISCUSSION Gigantiform cementoma is a rare benign lesion affecting the maxilla and mandible. The familial
increased
nature of the disease has been suspected (Van der Waal & Van der Kwast, 1974; Lyons & Babajews, 1980), although genetic transmission of the disease has not been confirmed. A study by Agazzi and Belloni (1953) reported families with members suffering from hard tissue tumours which histologically and radiographically resembled gigantiform Young et al. (1989) presented an cementoma. American family in which gigantiform cementoma could be confirmed over five generations. This report presents a Caucasian family in which the hereditary pattern of the lesion is evident. Even though detailed information concerning the diagnosis of the lesion of the father was not available, it is likely that his disease had been originally misdiagnosed as a malignant lesion because of lack of information on this condition at that time. It is possible that numerous malignancies found during the following years were related to primary radiation of the benign lesion. Osteosarcoma of the jaws can arise even several decades after the irradiation (Tucker et al., 1987). Clinical, radiological and histopathological features found in the son were similar to those described in the earlier literature supporting the diagnosis of gigantiform cementoma (Lyons & Babajews, 1986; Dominquez et al., 1987; Wolf et al., 1989; Young et al., 1989). Alternative diagnoses for similar changes include terms such as benign fibroosteo-cemental lesion (Burkhardt, 1989) and familial ossifying dysplasia (Melrose et al., 1976; Wolf et al., 1989; Fun-thee & Jinn-fei, 1989). A biopsy specimen was taken from the periapical lesion of the daughter at an earlier stage of the disease than that of the son. Even though no radiological evidence of hard tissue formation was noted periapically in the left mandibular second molar in her first orthopantomograph (Fig. 2A), deposition of cementurn-like masses were found on histopathological examination (Fig. 3). A later radiograph of the daughter revealed hard tissue formations similar to those of her brother, and were
considered to be typical of gigantiform cementoma. It was impossible to obtain a biopsy from the later
Gigantiform
stages of the lesions because the patient refused surgery. Radiologically, the lesions of both the daughter and the son were lobular radiopacies as described in the literature (Van der Waal & Van der Kwast, 1974; Punniamoorthy, 1980; Lyons & Babajews, 1986; Young et al., 1989) and the scintiscan of the son showed uptake of radioactive Technetium by the affected jaws similar to that reported by Millet (1990). The histological demonstration of dense cementum-like structures surrounded by cell-rich connective tissue is a typical finding in gigantiform cementoma (Van der Waal & Van der Kwast, 1974; Punniamoorthy, 1980; Lyons & Babajews, 1986; Wolf et al., 1989; Young et al., 1989). However, the histological appearance varies in different stages of the disease, and the final diagnosis should be based on the histopathological, radiological and clinical findings taken together. Usually gigantiform cementoma does not require any treatment. However, if jaw deformities develop, the jaws can be surgically reshaped (Van der Waal & Van der Kwast, 1974). Asymptomatic lesions should be left untreated (Waldron et al., 1975). The son of the family had been operated on four times and the daughter three times. Both patients will be kept under regular follow-up. It can be concluded that gigantiform cementoma is a rare and benign fibro-osseous lesion of the jaws. There are only a few reports in the literature dealing with the hereditary nature of the disease. Members of the family described in this report must have a familial tendency for fibro-osseous lesions, and were diagnosed as having familial gigantiform cementoma which according to a recent study by Young et al. (1989) should be recognised as a separate entity. References Agazzi, C. & Belloni, L. (1953). Gli odontomi duri dei mascellari. Archieve ftaliano Odontological Rinologica Laringologica, 64,5. Bhaskar, S. N. & Cutright, D. E. (1968). Multiple enostosis: Report of 16 cases. Journal of Oral Surgery, 26,321. Burkhardt, A. (1989). Dentin formation in so-called ‘fibro-osteocemental’ lesions of the jaws: Histological, electron microscopic and immunohistochemical investigations. Oral Surgery, Oral Medicine, Oral Pathology, 68,729. Dominquez, F. V., Espinal, E. G. & Ferraria. J. L. (1987). Gigantiform cementoma, Report of 2 cases. Dentomaxillofacial Radiology, 16, 113. Fun-thee, L. & Jinn-fei, Y. (1989) Florid osseous dysplasia in Orientals. Oral Surgery, Oral Medicine, Oral Pathology, 68. 748. Gorlin, R. J. &Goldman, H. M. (1970). Thoma’s Oral Pathology, 6th Edition, Vol. I. p. 506. St. Louis: The C.V. Mosby Co. Lucas, A. B. (1984). Pathology of Tumours of the Oral Tissues, 4th Edition, pp. 104-105. London: Churchill Livingstone. Lyons, A. J. & Babajews, A. V. (1986). Gigantiform cementoma -an unusual incidental finding. British Journal of Radiology, 59,277.
cementoma
affecting
a Caucasian
family
197
Melrose, R. J., Abrams, A. M. & Mills, B. G. (1976). Florid osseous dysplasia: a clinical-pathological study of thirty-four cases. Oral Surgery, Oral Pathology, Oral Medicine. 41,62. Millet, D. T. (1990). Gigantiform cementoma showing apparent activity on a bone scan. Dentomaxillofacial Radiology. 19. 137. Pindborg. J. J., Kramer. I. R. H. & Toroni. H. (1971). Histological Typing of Odontogenic Turnouts, Jaw Cysts and Allied Lesions, International Classification of Turnouts. No. 5, pp. 31-34. Geneva: World Health Organisation. Punniamoorthy. A. (1980). Gigantiform cementoma. Review of the literature and a case report. British Journal of Oral Surgery. 18,221. Rosenmertzt, S. K. & Schare, H. J. (1969). Osteogenicsarcoma arising in Paget’s disease of the mandible. Review of the literature and report of a case. Oral Surgery, Oral Medicine, Oral Pathology, 28,304. Tucker, M. A., Angio, G. J. & Boice, J. D. Jr. (1987). Bone sarcomas linked to radiotherapy and chemotherapy in children. New England Journalof Medicine, 28,304. Van der Waal, I. & Van der Kwast, W. A. M. (1974). A case of gigantiform cementoma. International Journalof Oral Surgery. 3,440. Waldron, C. A., Giansanti. J. S. & Brownard, B. C. (1975). Sclerotic cemental masses of the jaws (so called chronic sclerosing osteomyelitis, sclerosing osteitis, multiple enostosis and gigantiform cementoma). Oral Surgery, Oral Medicine, Oral Pathology, 39,590: Waldron, C. A. (1985). Fibro-osseous lesions of the jaws. Journal 1 of Oral and Maxitlofacial Surgery, 43,249. Winer. H. J.. Goepp. Gieantiform . . R. A. & Olsen. R. E. (1972). _ cementoma resembling Paget’s disease. Report of a case. Journal of Oral Surgery. 30,5 17. Wolf. J.. Hietanen, J. & Sane, J. (1989). Florid cemento-osseous dysplasia (gigantiform cementoma) in a Caucasian woman. British Journal of Oral and Ma.xill~facial Surgery, 27,46. Young, S. K., Markowitz. R., Sullivan. S.. Seale, T. W. & Hirshi, R. (1989). Familial gigantiform cementoma: Classification and presentation of a large pedigree. Oral Surgery, Oral Medicine, Oral Pathology. 68,140. I
”
The Authors Kyi3sti Oikarinen DDS, PhD, DMD Senior Lecturer Department of Oral and Maxillofacial Institute of Dentistry University of Oulu Oulu Finland Risto-Pekka Happonen DDS, PhD Senior Lecturer Department of Oral and Maxillofacial Institute of Dentistry University of Turku Turku Finland Mikko Altonen MD, DDS, PhD Professor Department of Oral and Maxillofacial Institute of Dentistry University of Oulu Oulu Finland
Surgery
Surgery
Surgery
Correspondence and requests for offprints to Kyosti Oikarinen, Institute of Dentistry, Aapistie 3. SF-90220 Oulu, Finland Paper received 6 November Accepted 9 January 1991
1990
lournal
1991 The
of Oral and Maxi//ofana/ Surgery (IWI) British
Association
Gigantiform Kyiisti Oikarinen,
29, 194-197 Surgeons
of Oral and Maxillofacial
cementoma Mikko Altonen,
affecting a Caucasian Risto-Pekka
family
Happonen
Departments of Oral and Maxillofacial Surgery, Institute of Dentistry, Institute of Dentistry, University of Turku, Finland
University of Oulu, and
A Caucasian family is presented in which two out of three children showed lesions resembling SUMMARY. gigantiform cementoma clinically, radiologically and histopathologically. Both affected children had swellings of the alveolar crest, caused by large, lobular, radio-opaque masses in all four quadrants of the jaws. Histologically, cementum-like masses were surrounded by inflamed fibrous connective tissue. There was evidence indicating that the late father had similar lesions which became malignant following radiation therapy.
1987). Dense, cementurn-like acellular hard tissue surrounded by connective tissue is seen histologically (Van der Waal & Van der Kwast, 1974; Dominquez et al., 1987; Young et al., 1989). The condition has been considered familial, being found mostly in blacks (Van der Waal & Van der Kwast, 1974; Lyons & Babajews, 1986), although opposite opinions have also been presented (Punniamoorthy, 1980). Only few reports exist in which the hereditary nature of the lesion has been proven (Agazzi & Belloni, 1953; Young et al., 1989). This study reports a Caucasian family in which gigantiform cementoma was diagnosed in two out of three children as well as in the late father, whose lesions had been treated as mandibular malignancy several decades previously.
INTRODUCTION
In the classification of fibro-osseous lesions by Waldron (1985), gigantiform cementoma is placed among those arising in the periodontal ligament. Classification of cementomas introduced by Pindborg et al. (1971) differentiates between benign cementoblastoma, cementifying fibroma, periapical cemental dysplasia and gigantiform cementoma. Gigantiform cementoma, also called familial multiple cementoma and florid cemento-osseous dysplasia, has been often misdiagnosed as sclerosing osteomyelitis, osseous dysplasia and bony enostosis (Bhaskar & Cutright, 1968; Waldron et al., 1975; Wolf et al., 1989) due to lack of specific diagnostic methods and the rarity of this lesion. Gigantiform cementoma is reported to have a racial and sex predilection affecting mainly middle-aged Negro women (Gorlin & Goldman, 1970). It is essentially a benign condition (Winer et al., 1972) and must therefore be differentiated from Paget’s disease which may sometimes have radiological similarities but can undergo malignant transformation (Rosenmertz & Schare, 1969). Gigantiform cementoma has been speculated as originating from remnants of cementum left within the bone after tooth extraction (Punniamoorthy, 1980). Deposition of cementum can impair the vascularity of the bone which can predispose to osteomyelitis (Van der Waal & Van der Kwast, 1974). Even though it is difficult to differentiate the origin of the hard tissue in various fibro-osteocemental lesions (Burkhardt, 1989), the diagnosis of gigantiform cementoma can be confirmed on the basis of clinical, radiological and histological findings (Young et al., 1989). Swelling of the jaw bones can cause facial deformity (Young et al., 1989) which may be the only complaint of the patient (Lucas, 1984). Irregular, often lobular radio-opacities are seen symmetrically in the mandible and maxilla, most typically in the molar regions (Punniamoorthy, 1980; Lyons & Babajews, 1986; Dominquez et al.,
Case reports V.S. (The father):
In 1933 at the age of 17 years an expansive, radiologically sclerotic tumour in the left mandible was found, excised
Fig. I- Radiograph of the father’s mandible taken in connection with radiation therapy for tonsillary adenocarcinoma at the age of 64 years (the metallic pins are seen). Lesions resembling gigantiform cementoma affect most of the body of the mandible (Arrows). M = mandibular angle. 194
Gieantiform and irradiated because malignancy was suspected. No histopathological specimens or radiographs are available fro? the operation, but an X-ray taken shortly before his death show lesions resembling gigantiform cementoma (Fig. 1). Nine years after the primiry treatment, osteosarcoma arose at the site of the original tumour. The lesion was treated by mandibular resection and bone grafting. Eighteen years after the radiation a squamous cell carcinoma of the left ear was found and treated by irradiation. Basal cell carcinoma of the chin, carcinoma in situ of the glottis and tonsillar adenocarcinoma were diagnosed 31, 37 and 48 years after the first radiation. A brain tumour (detailed diagnosis unknown) was the final cause of death at the age of 65 years. E. H. (The first daughter): A 2%year-old woman suffering from insulin dependent diabetes since the age of 12 years had her first operation because of a firm swelling in the maxilla. Histologically, cementurn-like masses were found within connective tissue. Three years later a recurrence was excised from the same region. The patient was referred to the Department of Oral and Maxillofacial Surgery, Institute of Dentistry, University of Oulu, 7 years after the first operation. Clinically, an alveolar swelling was found on the buccal side of the left maxillary molar region. A radiograph revealed a lobular area of radio-opacity around the left maxillary molars. Advanced periodontitis and a periapical radiolucency were seen around the non-vital left mandibular second molar (Fig. 2A). The tooth was extracted under local anaesthesia. Four years later at the age of 40 years, the patient returned to the department because of swelling of the left side of the mandible and maxilla. The lesion involved all maxillary molars. In the left mandibular molar region it extended to the lower border of the mandible. Clinically and radiologically distal displacement of the upper left second molar was evident. At review 1 year later the patient was still symptom-free apart from the swelling and refused any further surgery because of a recent diagnosis of a brain tumour (Schwannoma). Further ossification of the mandibular lesion was evident on the orthopantomogram (Fig. 2B). A biopsy taken from the periapical lesion around the lower left second molar supported a cemental origin of the lesion. Cementurn-like masses with rounded margins were surrounded by fibrous connective tissue containing mononuclear inflammatory cells, active fibroblasts and immature collagen (Fig. 3).
cementoma
affecting a Caucasian familv
195
Fig. 2B - Elongation and distal displacement of maxillary left molars as well as bilateral enlargement of the lesions in the maxilla can be seen in the orthopantomograph taken five years after the radiograph in Figure 2A. Note also further ossification of the mandibular lesion in the left mandible.
Fig. 3 - Photomicrograph of the biopsy from the periapical area of the mandibular second molar of the first daughter showing cellular connective tissue with spherical hard tissue particles resembling cementum. (H & E. Original magnification x320).
Fig. 4 - Orthopantomograph
of the son with edentulous maxilla and partially dentate mandible affected by large lesions resembling gigantiform cementoma.
R.S. (The son):
Orthopantomograph of the first daughter demonstrating lobular radio-opaque areas in the maxillary left molar region and
Fig. 2A -
periapical molar.
radiolucency
affecting the left mandibular
second
At the age of 14 years, the son had his first operation performed in the left mandibular molar region. The histopathological diagnosis was fibrous dysplasia. Ten years later, a recurrence was operated upon, and the histopathological diagnosis was cementifying fibroma. At the age of 27 years, a third operation was performed at the same site and the excised tissue was reported histologically
196
British
Journal
of Oral
and Maxillofacial
Surgery
Fig. 5 - Scintigraphy of the son in postero-anterior uptake of ~~ Tc bilaterally
(A) and lateral projections from the right (B) and left (C) side demonstrate in the maxilla and mandible, especially in the left side. (Dex = right, sin = left).
Fig. 6 - PhotuwLLug mdible of the son s
rounded by IGIll
as fibrous dysplasia. Ten years after the third operation the patient was referred to a maxillofacial surgeon. The patient indicated that the lesion had been growing slowly and had caused only slight discomfort. A painless swelling was noted in the left mandibular molar region. Radiographs showed that the edentulous maxilla and partially dentate mandible were affected by large sclerotic lesions (Fig. 4). Scintigraphy with WmTc showed increased uptake bilaterally in the maxilla and mandible especially in the left side (Fig. 5). The shape of the mandible was corrected surgically under local anaesthesia. Histopathological findings were essentially similar to those found in the biopsy of the sister (Fig. 6). M.M. (The second daughter):
The third child of the family, a daughter, was examined clinically and radiographically at the age of 42 years, but she was found to be free of any jaw diseases.
DISCUSSION Gigantiform cementoma is a rare benign lesion affecting the maxilla and mandible. The familial
increased
nature of the disease has been suspected (Van der Waal & Van der Kwast, 1974; Lyons & Babajews, 1980), although genetic transmission of the disease has not been confirmed. A study by Agazzi and Belloni (1953) reported families with members suffering from hard tissue tumours which histologically and radiographically resembled gigantiform Young et al. (1989) presented an cementoma. American family in which gigantiform cementoma could be confirmed over five generations. This report presents a Caucasian family in which the hereditary pattern of the lesion is evident. Even though detailed information concerning the diagnosis of the lesion of the father was not available, it is likely that his disease had been originally misdiagnosed as a malignant lesion because of lack of information on this condition at that time. It is possible that numerous malignancies found during the following years were related to primary radiation of the benign lesion. Osteosarcoma of the jaws can arise even several decades after the irradiation (Tucker et al., 1987). Clinical, radiological and histopathological features found in the son were similar to those described in the earlier literature supporting the diagnosis of gigantiform cementoma (Lyons & Babajews, 1986; Dominquez et al., 1987; Wolf et al., 1989; Young et al., 1989). Alternative diagnoses for similar changes include terms such as benign fibroosteo-cemental lesion (Burkhardt, 1989) and familial ossifying dysplasia (Melrose et al., 1976; Wolf et al., 1989; Fun-thee & Jinn-fei, 1989). A biopsy specimen was taken from the periapical lesion of the daughter at an earlier stage of the disease than that of the son. Even though no radiological evidence of hard tissue formation was noted periapically in the left mandibular second molar in her first orthopantomograph (Fig. 2A), deposition of cementurn-like masses were found on histopathological examination (Fig. 3). A later radiograph of the daughter revealed hard tissue formations similar to those of her brother, and were
considered to be typical of gigantiform cementoma. It was impossible to obtain a biopsy from the later
Gigantiform
stages of the lesions because the patient refused surgery. Radiologically, the lesions of both the daughter and the son were lobular radiopacies as described in the literature (Van der Waal & Van der Kwast, 1974; Punniamoorthy, 1980; Lyons & Babajews, 1986; Young et al., 1989) and the scintiscan of the son showed uptake of radioactive Technetium by the affected jaws similar to that reported by Millet (1990). The histological demonstration of dense cementum-like structures surrounded by cell-rich connective tissue is a typical finding in gigantiform cementoma (Van der Waal & Van der Kwast, 1974; Punniamoorthy, 1980; Lyons & Babajews, 1986; Wolf et al., 1989; Young et al., 1989). However, the histological appearance varies in different stages of the disease, and the final diagnosis should be based on the histopathological, radiological and clinical findings taken together. Usually gigantiform cementoma does not require any treatment. However, if jaw deformities develop, the jaws can be surgically reshaped (Van der Waal & Van der Kwast, 1974). Asymptomatic lesions should be left untreated (Waldron et al., 1975). The son of the family had been operated on four times and the daughter three times. Both patients will be kept under regular follow-up. It can be concluded that gigantiform cementoma is a rare and benign fibro-osseous lesion of the jaws. There are only a few reports in the literature dealing with the hereditary nature of the disease. Members of the family described in this report must have a familial tendency for fibro-osseous lesions, and were diagnosed as having familial gigantiform cementoma which according to a recent study by Young et al. (1989) should be recognised as a separate entity. References Agazzi, C. & Belloni, L. (1953). Gli odontomi duri dei mascellari. Archieve ftaliano Odontological Rinologica Laringologica, 64,5. Bhaskar, S. N. & Cutright, D. E. (1968). Multiple enostosis: Report of 16 cases. Journal of Oral Surgery, 26,321. Burkhardt, A. (1989). Dentin formation in so-called ‘fibro-osteocemental’ lesions of the jaws: Histological, electron microscopic and immunohistochemical investigations. Oral Surgery, Oral Medicine, Oral Pathology, 68,729. Dominquez, F. V., Espinal, E. G. & Ferraria. J. L. (1987). Gigantiform cementoma, Report of 2 cases. Dentomaxillofacial Radiology, 16, 113. Fun-thee, L. & Jinn-fei, Y. (1989) Florid osseous dysplasia in Orientals. Oral Surgery, Oral Medicine, Oral Pathology, 68. 748. Gorlin, R. J. &Goldman, H. M. (1970). Thoma’s Oral Pathology, 6th Edition, Vol. I. p. 506. St. Louis: The C.V. Mosby Co. Lucas, A. B. (1984). Pathology of Tumours of the Oral Tissues, 4th Edition, pp. 104-105. London: Churchill Livingstone. Lyons, A. J. & Babajews, A. V. (1986). Gigantiform cementoma -an unusual incidental finding. British Journal of Radiology, 59,277.
cementoma
affecting
a Caucasian
family
197
Melrose, R. J., Abrams, A. M. & Mills, B. G. (1976). Florid osseous dysplasia: a clinical-pathological study of thirty-four cases. Oral Surgery, Oral Pathology, Oral Medicine. 41,62. Millet, D. T. (1990). Gigantiform cementoma showing apparent activity on a bone scan. Dentomaxillofacial Radiology. 19. 137. Pindborg. J. J., Kramer. I. R. H. & Toroni. H. (1971). Histological Typing of Odontogenic Turnouts, Jaw Cysts and Allied Lesions, International Classification of Turnouts. No. 5, pp. 31-34. Geneva: World Health Organisation. Punniamoorthy. A. (1980). Gigantiform cementoma. Review of the literature and a case report. British Journal of Oral Surgery. 18,221. Rosenmertzt, S. K. & Schare, H. J. (1969). Osteogenicsarcoma arising in Paget’s disease of the mandible. Review of the literature and report of a case. Oral Surgery, Oral Medicine, Oral Pathology, 28,304. Tucker, M. A., Angio, G. J. & Boice, J. D. Jr. (1987). Bone sarcomas linked to radiotherapy and chemotherapy in children. New England Journalof Medicine, 28,304. Van der Waal, I. & Van der Kwast, W. A. M. (1974). A case of gigantiform cementoma. International Journalof Oral Surgery. 3,440. Waldron, C. A., Giansanti. J. S. & Brownard, B. C. (1975). Sclerotic cemental masses of the jaws (so called chronic sclerosing osteomyelitis, sclerosing osteitis, multiple enostosis and gigantiform cementoma). Oral Surgery, Oral Medicine, Oral Pathology, 39,590: Waldron, C. A. (1985). Fibro-osseous lesions of the jaws. Journal 1 of Oral and Maxitlofacial Surgery, 43,249. Winer. H. J.. Goepp. Gieantiform . . R. A. & Olsen. R. E. (1972). _ cementoma resembling Paget’s disease. Report of a case. Journal of Oral Surgery. 30,5 17. Wolf. J.. Hietanen, J. & Sane, J. (1989). Florid cemento-osseous dysplasia (gigantiform cementoma) in a Caucasian woman. British Journal of Oral and Ma.xill~facial Surgery, 27,46. Young, S. K., Markowitz. R., Sullivan. S.. Seale, T. W. & Hirshi, R. (1989). Familial gigantiform cementoma: Classification and presentation of a large pedigree. Oral Surgery, Oral Medicine, Oral Pathology. 68,140. I
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The Authors Kyi3sti Oikarinen DDS, PhD, DMD Senior Lecturer Department of Oral and Maxillofacial Institute of Dentistry University of Oulu Oulu Finland Risto-Pekka Happonen DDS, PhD Senior Lecturer Department of Oral and Maxillofacial Institute of Dentistry University of Turku Turku Finland Mikko Altonen MD, DDS, PhD Professor Department of Oral and Maxillofacial Institute of Dentistry University of Oulu Oulu Finland
Surgery
Surgery
Surgery
Correspondence and requests for offprints to Kyosti Oikarinen, Institute of Dentistry, Aapistie 3. SF-90220 Oulu, Finland Paper received 6 November Accepted 9 January 1991
1990
