THE CEMENTOMA. A STUDY OF 230 PATIENTS WITH 435 CEMENTOMAS.
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Familial gigantiform cementoma: classification and presentation of a large pedigree.
Very few cases of gigantiform cementoma have been reported, and those associated with a positive family history are especially rare. Confusion exists about the relationship of gigantiform cementoma to florid osseous dysplasia, cementifying fibroma, and diffuse chronic sclerosing osteomyelitis. It has been unclear whether gigantiform cementoma should be accorded the status of a separate entity. In this article, we report our findings on a family that, over five generations, has exhibited clinical, radiographic, and/or histologic findings consistent with the designation familial gigantiform cementoma. This pedigree consists of 55 members. Significant heterogeneity in expression of this trait was noted. The pattern of occurrence of the trait is consistent with an autosomal dominant mode of inheritance with variable expressivity of the phenotype. We suggest that familial gigantiform cementoma should be recognized as a separate entity.
Cementoma↗
Cementomas--aggressive or innocuous neoplasms?
No class of tumor is so inadequately discussed in the otolaryngology literature as the "cementomas". Until recent years, four distinctly different benign fibro-osseouslesions containing cementum were clumped together under the heading of cementoma. The separate entities in this tumor class are benign cementoblastoma (true cementoma), cementifying fibroma, peripical cemental dysplasia, and gigantiform cementoma (florid osseous dysplasia). Only an occasional case of the gigantiform variant or an unusually aggressive cementifying fibroma requires an extensive operation for complete excision.
Adolescent↗
Gigantiform cementoma: clinicopathologic presentation of 3 cases.
Gigantiform cementoma is a rare, benign fibro-cemento-osseous disease of the jaws, seen most frequently in young girls. Radiographically, it typically presents as multiquadrant, expansile, mixed radiolucent-opaque lesions that cross the midlines of the jaws. Although cases with a familial pattern are noted in a few publications, sporadic cases have been reported without a family history. The term gigantiform cementoma has been used interchangeably with designations of other fibro-osseous entities, yet its application should be restricted by the criteria defined herein. In this article, we report 3 cases of gigantiform cementoma. Clinical, radiographic, and microscopic features of these lesions are presented, along with criteria to differentiate gigantiform cementoma from other fibro-osseous diseases of the jaws. A possible pathogenetic mechanism and treatment recommendations are discussed.
Adolescent↗
The cementomas--a clinicopathological re-appraisal.
The aim of this study was to assess whether sub-classification of cemental tumors was warranted and to define the clinicopathological features of the definitive entities. Our sample consisted of 127 cases which were divided into the following categories; gigantiform cementoma (84 per cent), cementifying fibroma (12 per cent), benign cementoblastoma (4 per cent). Gigantiform cementoma lesions were either single, multiple or florid and ranged in size from 1 to 10 cm. Most occurred in patients in their 6th and 7th decades who were black (78 per cent) and female (96 per cent). All lesions showed typical solid sheets of acellular cementum and some (22 per cent) were characterised by peripheral proliferative areas which were often indistinguishable from lesions of cementifying fibroma and periapical cemental dysplasia. Infection and sequestration was very common (54 per cent). No cases of periapical cemental dysplasia were found and we suggest that this lesion is a variant of gigantiform cementoma. We believe cementifying fibroma to be part of the histomorphological spectrum of cemento-ossifying fibroma. Cemento-osseous dysplasia is a more accurate and appropriate term than gigantiform cementoma and we recommend the following classification for cemental 'tumors': cemento-ossifying fibroma; cementoblastoma; cemento-osseous dysplasia, single, multiple and florid sub-types.
Adolescent↗
Cementoma-like tumours of bone.
The authors summarize the morphological findings on cementoma-like fibromas of long bones registered at the Reference Centre for Bone Tumours in Brno or referred to it. One of the cases registered is described in detail from the submicroscopic point of view. Their experience has shown that cementoma-like fibromas of long bones are genuine bone tumours sui generis, differing to some extent from cementomas of the jaws. The cementicles in such tumours are identical to acellular dental cement and structurally quite different from osteoid.
Adult↗
Cementoma--presentation predicates approach.
Cementomas are benign jaw tumors that originate from periodontal ligament elements. Four classifications of cementomas are described: periapical fibrous dysplasia, benign cementoblastoma, cementifying fibroma, and florid osseous dysplasia. Usual treatment regimens vary from observation to limited surgical resection based upon tumor type and clinical characteristics. We present an unusual case report of an extensive cementifying fibroma that required mandibular and inferior alveolar nerve resection followed by osseous and neural reconstruction.
Adult↗
[Cementoma of the skull base].
Cementomas result from the odontotic epithelial-mesenchymal complex and are located mainly in the area of large and small molar teeth of the mandible. There was an extremely rare cement location in the analyzed clinical cases. In the first case, cementoma of the frontal sinus spread into the ethmoidal sinus and eye socket in a female patient aged 38 years. The tumor is manifested by right frontalorbital pain and exophthalmos. There were no problems in removing the tumor and repairing the defect of the skull base. The second case was an extensive tumor in a 11-year-old child, which involves the maxillary, ethmoidal, and sphenoidal sinuses, by damaging the bones of the skull base. Tumor removal gave rise to an extensive defect of the skull base which the dura mater protruded through. In this case the important stage was closure of the defect of the dura mater and that of the skull base with the dura mater protruding through. This defect could be successfully closed with a pedicle musculoperiosteal flap of the musculus temporalis by fixation with sutures and fibrinthrombin glue.
Adult↗
A case of gigantiform cementoma associated with multiple unerupted teeth.
Both the occurrence of gigantiform cementoma in the jaws or multiple unerupted teeth are very rare conditions. The purpose of this paper is to report on a case of a combination of these two conditions and to review relevant literature. A 47-year-old dwarfish male was referred to our Oral and maxillofacial Surgical Department with the chief complaint of pain and swelling in the right lateral nasal region with the discharge of pus from the right upper premolar area. Clinical and roentgenographic examinations revealed that there were 17 unerupted teeth and some radiopaque and radiolucent shadows on both jaws. This patient was treated with saucerization and extraction of unerupted teeth on the right upper and lower jaws for controlling infected lesions. A surgical specimen was sent for histopathological examination and the report indicated gigantiform cementoma.
Cementoma↗
Cementum-like bone production in solitary bone cysts. (so-called "cementoma" of long bones). Report of three cases. Electron microscopic observations supporting a synovial origin to the simple bone cyst.
Three cases of simple bone cysts (S.B.C) in association with cementum-like bone production are reported. Analysis of our cases and the 2 previously reported in the literature as so-called cementomas has led us to conclude that the "cementoma" of long bone is not a distinct entity, but merely, a form of S.B.C. associated with a peculiar, poorly cellular form of bone which mimics tooth cementum by light microscopy only. Electron microscopic studies of this substance demonstrate collagen fibers and numerous matrix vesicles which form the initial sites of calcification. Matrix vesicles are a product of osteoblastic activity and are never found in the cementum of the tooth or oral cementum producing tumors. We also report the first ultramiscroscopic observations on the wall of the simple bone cyst and its lining. Two cell types constitute the lining, having features identical to those described for type A and type B synovial cells. Because of this new observation, we believe, the most reasonable explanation for the simple bone cyst is that it represents a congenital "rest" of synovial tissue displaced into the thin, cortical metaphyseal region of bones at the synovial-capsularbone reflection. Its benign nature and slow growth would explain its discovery in early childhood and the marked preponderance of its proximal humerofemoral location dependent upon the fact that these 2 bones have the largest area of capsular to metaphyseal bone reflection.
Adolescent↗
Gigantiform cementoma of the jaws.
Fibro-osseous cemental lesions of the jaw remain a controversial area of bone/cementum pathology. This article describes the clinicopathological features of 28 lesions classified as gigantiform cementoma. Black women are affected more frequently than other race and sex groups, with a peak frequency of occurrence in the fourth and fifth decades. The posterior mandible is preferentially affected and unifocal lesions (67.9%) occur more frequently than multifocal lesions (32.1%). Dull pain and a centrifugal size increase are the most prevalent presenting symptoms. Of 28 cases with parental and sibling clinical information, there were no cases which exhibited an autosomal dominant genetic association. The pathogenesis of this lesion in relation to other cemental lesions of the jaws, as well as the clinical management of cemental jaw lesions, is discussed.
Adult↗
Tumour-like lesions in the femur with cementum-like material. Does a "cementoma" of long bone exist?
Five hundred and seventy nine juvenile bone cysts were analysed to evaluate the radiological and histological structures. Most of the juvenile bone cysts showed a typical appearance radiographically; histologically they had a cyst wall consisting of fibrous tissue without an epithelial lining. In many of these lesions newly formed bone trabeculae, granulation tissue, calcium deposits, and in some cases focal deposits of a homogeneous material resembling cementum of tooth were observed. In 28 lesions a well circumscribed, round focus was observed within the proximal femur or femoral neck radiologically, showing dense calcification with a small central radiolucency. In some cases, the intraosseous focus was almost solid without a cystic appearance. Histologically the unusual bone lesions consisted almost exclusively of calcified cementum devoid of cells. Lesions of this type were found exclusively in the proximal femur of patients between 47 and 56 years of age. Because of the radiological and histological appearance we would like to call this solid lesion "cementoma of long bones". Operative therapy is not necessary.
Adolescent↗
Gigantiform cementoma: review of the literature and a case report.
Gigantiform cementoma is a very rare lesion in the jaw. A 64-year-old caucasian female who presented with this condition is reported and the relevant literature reviewed. The difficulty of making a definitive diagnosis on histological grounds is discussed. An attempt has been made to analyse the lesion biochemically in order to confirm its cemental origin.
Cementoma↗
Sclerotic cemental masses of the jaws (so-called chronic sclerosing osteomyelitis, sclerosing osteitis, multiple enostosis, and gigantiform cementoma.
A series of thirty-eight cases of densely sclerotic lesions of the jaws was studied. The lesions were all at least 1.5 cm, in diameter. Pain, drainage, or localized expansion of the jaw was present in two thirds of the patients. Thirty-four of the thirty-eight patients were women, and twenty-five were negros. In fourteen instances, a radiographic survey of the entire jaws was available. Thirteen of these fourteen patients had multiple lesions which were usually symmetrically distributed throughout the jaws. In twenty-four patients radiographs of the lesional area only were available for review. In thirty-four of the thirty-eight cases (89 per cent), the sclerotic masses were interpreted as cementum. Although this condition has previously been described as chronic sclerosing osteomyelitis, sclerosing osteitis, multiple enostosis, or gigantiform cementoma, it appears more appropriate to consider these lesions as part of the spectrum of the benign fibro-osseous lesions of periodontal ligament origin. Four of the sclerotic lesions, which were radiographically indistinguishable from the cemental lesions, consisted only of bone. Two of these appeared to be examples of true chronic sclerosing osteomyelitis, and two may be appropriately designated as enostosis or osteomas.
Adult↗
Gigantiform cementoma affecting a Caucasian family.
A Caucasian family is presented in which two out of three children showed lesions resembling 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.
Adolescent↗
Gigantiform cementoma occurring in two populations, London and Hong Kong.
The gigantiform cementoma presents as multiple opacities in the tooth-bearing areas of the jaws. Sixteen cases are presented, six of British Negresses and 10 of Hong Kong Chinese females. The differential diagnosis and management are briefly reviewed.
Asian People↗
Familial gigantiform cementoma.
Familial gigantiform cementoma is a rare autosomal dominant tumor that is benign but can result in disfigurement of the facial skeleton. Two families with a total of five patients presented for treatment. Because of a lack of opportunity to obtain treatment early, three of the patients presented in adult life with massive tumors requiring extensive resection and complex reconstruction in multiple stages. The two female patients had chronic anemia caused by multifocal polypoid adenomas of the uterus and required hysterectomy before treatment. The last three patients had elevated alkaline phosphatase levels before tumor resection, and these levels decreased after surgery. With extensive resection of the tumors and reconstruction of both the soft tissues and facial skeleton, good functional and aesthetic results can be obtained. There has been no tumor recurrence with 3 years of follow-up.
Adult↗
Gigantiform cementoma showing apparent activity on a bone scan.
A bone scan in a Negroid female suspected of myeloma showed no uptake other than in the jaws. A panoramic radiograph revealed multiple mixed-density lesions, in particular in the mandible, suggestive of gigantiform cementoma. The significance of this association is discussed.
Cementoma↗