Familial Gigantiform Cementoma Research Articles

Familial florid cemento-osseous dysplasia: an overview of a rare entity

Familial florid cemento-osseous dysplasia (FFCOD) is distinct from the sporadic variant and may often be confused with familial conditions presenting with lesions resembling cemento-ossifying fibromas. The current review aims to elucidate the FFCOD variant better and discuss distinguishing features with sporadic florid COD. A review of the literature on FFCOD cases using Google Scholar and PubMed was performed and summarised. A total of 11 articles with 36 patients were included in the current review. The clinical and radiologic presentations and the pertinent differences from the sporadic variant were discussed. The familial form shows advanced sclerosis and extensive distribution at a younger age, together with impacted teeth and bony expansion in the anterior mandible. Furthermore, distinguishing features from the most important differential diagnoses of other hereditary fibro-osseous conditions, including familial gigantiform cementoma (FGC), hyperparathyroidism jaw tumour syndrome (HP-JTS) and gnathodiaphyseal dysplasia (GDD) are discussed.

Familial Gigantiform Cementoma: Life-Saving Total Midface Resection and Reconstruction Using Virtual Surgical Planning and 3D Printed Patient-Specific Implant-A Clinical Study.

Familial gigantiform cementoma (FGC) is a rare benign fibrocemento-osseous lesion of the jaw characterized by well-circumscribed, extensive, mixed radiolucent-radiopaque masses in the mandible and the maxilla that can cause severe facial deformity. This condition is extremely rare with less than 40 cases reported in the literature. The purpose of the paper is to highlight the importance of virtual surgical planning and patient-specific implant in the treatment of a complex lesion and reconstruction of the facial skeleton. The clinical presentations, and diagnostic challenges encountered when managing the lesion have been discussed in this article with emphasis on the treatment plan. The sequence of treatment planned was resection of the lesion and immediate reconstruction with a patient-specific implant to improve the patient's quality of life. The management of FGC was a challenging one keeping in mind the rapid expansion of the lesion, widespread involvement of the jaws, and needs of the pediatric patient. Virtual surgical planning (VSP) along with 3D printed implant was instrumental in reconstructing the facial form of the child where the maxilla was completely resected and rehabilitation provided support to the vital structures of the face.

Clinical and pathological features and differential diagnosis of fibro-osseous tumours and dysplasias

Fibro-osseous lesions is a class of diseases with obvious similarities in clinical manifestations and pathological features, which has been attracting the attention of clinicians and pathologists. The latest WHO 2022 Classification (5th edition) included six of these diseases (cemento-osseous dysplasia, segmental odontomaxillary dysplasia, fibrous dysplasia, juvenile trabecular ossifying fibroma, psammomatoid ossifying fibroma and familial gigantiform cementoma) in the " fibro-osseous tumours and dysplasias ", and put forward new ideas on the diagnosis and treatment of these diseases. According to the latest WHO 2022 Classification (5th edition), the clinical and pathological features, diagnosis and differential diagnosis of these six diseases were described.

Ossifying fibroma a case report with review of literature

Ossifying fibroma also known as cemento ossifying fibroma is a slow-growing benign fibroosseous lesions of the oral and maxillofacial regions. They present clinically as asymptomatic swelling of the cortical layer of jaw bones, producing a marked extraoral facial asymmetry. Fibrous dysplasia a developmental anomaly, wherein bone gets replaced by fibrous tissue, whereas ossifying fibroma, cemento-osseous dysplasia or familial gigantiform cementoma are either reactive or dysplastic change, derived from periodontal ligament, extraligamentary bone or remnants of cementum may create confusion to a clinician. Because of the presence of both bone and cementum-like products in ossifying fibromas, these lesions are designated as ossifying fibroma, cemento-ossifying fibroma, and cementifying fibroma. Fortunately ossifying fibromas are treated with surgical curettage with acceptable results. This paper is a humble attempt to present a case of Ossifying fibroma of maxilla which is otherwise common in mandible, and its esthetic outcome after the surgical treatment through Weber Fergusson's approach and a review of literature.

Clinical and imaging characteristics of familial gigantiform cementoma. A review of the literature

Familial gigantiform cementoma (FGC) is a rare benign fibro-cementum lesion, which follows an autosomal dominant inheritance pattern and presents during childhood. It is limited to the bones of the face, with a predilection for the jaw, is fast growing and painless and expands considerably over time. It is considered among the seven disorders that affect the physiognomy of the craniofacial skeleton. Radiographically, FGC occurs in three stages of maturation similar to bone dysplasia, being radiolucent, mixed and radiopaque and is described as a mixed lobular well delimited mass, which can occur in both maxillae, causing expansion of the buccal and palatal / lingual bone cortices. displacement and retention of teeth. The aim of this study was to perform a review of the literature to identify the clinical, radiographic and histopathological characteristics of FGC in the jaws and describe the imaging tools that are useful for the diagnosis and follow-up of this lesion.

A family of familial gigantiform cementoma: clinical study.

Familial gigantiform cementoma is an autosomal dominant fibro-cement osseous lesion that causes massive expansion of facial skeleton. Patients with such massive lesions have a compromised quality of life. The main goal of treating such patients is to restore and preserve the jaw as far as possible which would enhance their quality of life. This study was conducted to identify the occurrence of gigantiform cementoma which had affected three generations of a family and also to focus on documentation of the clinical course and management. Patients (one family-mother, grandmother, aunt and grandson) who had visited the Department of Oral and Maxillofacial Surgery, Tamilnadu Government Dental College and Hospital, Chennai, over a period for their swelling in the maxillofacial region were clinically and radiographically evaluated and histopathologically diagnosed as familial gigantiform cementoma; later, they were surgically managed. It is very rare to document three generations of this disease which had shown varied clinical presentation (asymptomatic slow growth, arrested growth and one case of aggressive growth). Management of these cases varied from observation to aggressive resection. Gigantiform cementoma follows an autosomal dominant pattern of inheritance with variable phenotypic expression without gender predilection. These cases require regular observation and intervention if necessary.

SURGICAL TREATMENT OF OSSEOUS DYSPLASIA IN THE MANDIBLE

The osseous dysplasias (ODs) are nonneoplastic conditions characterized by replacement of bone with abnormal fibrous tissue accompanied by gradual deposition of mineralized masses in the jaws. The classification of ODs includes focal OD, periapical OD, florid OD, and familial gigantiform cementoma. An expansive growth is rare and occurs most frequently in the latter group. We will present a case of osseous dysplasia in the mandible. The first surgical procedure occurred when the patient was 13 years old. The patient was surgically treated because of the large bone volume developed in the mandible, making it asymmetric. The patient was followed up until the age of 20 and again presented bone growth in the body region and mandibular angle. There was a second surgical intervention to regularize the area and return mandibular symmetry. The patient has 3 years of follow-up of the second surgical intervention and no intercurrences of the lesion.

Osseous Dysplasia with Gross Jaw Expansion: A Review of 18 Lesions.

Fourteen cases with 18 grossly expansive lesions diagnosed over a period of 15years as either "familial gigantiform cementoma" or "osseous dysplasia with jaw expansion" in an African population sample were reviewed. Eight lesions occurred in the anterior mandible, the maxilla was affected by four, three patients presented with more than one lesion and the most common associated pathologies were tooth displacement, conventional non expanding florid osseous dysplasia and simple bone cyst. No history of similar lesions in relatives of the diseased were recorded. The radiolucent fibrous component contained globular bone deposits and cellular osteoid with trabecular differentiation which matured into radiodense mineralized masses. Resorption of the cellular bone created cavities which are proposed to represent the early stage of simple bone cyst formation. It is recommended that "expansive osseous dysplasia" replace the out-dated term "familial gigantiform cementoma". The differential diagnoses of expansive osseous dysplasias are discussed.

Familial gigantiform cementoma: distinctive clinical features of a large Chinese pedigree

Familial gigantiform cementoma is a rare benign fibrocemento-osseous lesion of the jaws that can cause severe facial deformity. It has an autosomal dominant mode of inheritance, but varies in its phenotype. It is more common in white, African, and East-Asian patients. Here we report what is to our knowledge the first distinctive Chinese family with familial gigantiform cementoma involving 4 generations and 13 patients, and which suggests that the tumour presents with 3 distinctive growth phrases.

Follow-up CT findings of recurrent familial gigantiform cementoma of a female child

Familial gigantiform cementoma (FGC) is a rare autosomal dominant, benign fibro-cemento-osseous lesion of the jaws that can cause severe facial deformity. True FGC with familial history is extremely rare and there has been no literature regarding the radiological follow-up of FGC. We report a case of recurrent FGC in an Asian female child who has been under our observation for 6 years since she was 15 months old. After repeated recurrences and subsequent surgeries, the growth of the tumor had seemed to plateau on recent follow-up CT images. The transition from an enhancing soft tissue lesion to a homogeneous bony lesion on CT may indicate decreased growth potential of FGC.

Florid osseous dysplasia

The concept of ‘fibro-osseous lesions’ of bone has evolved over the last several decades and now includes two major entities, viz., fibrous dysplasia and ossifying fibroma, as well as other less common entities such as periapical dysplasia, focal osseous dysplasia, florid osseous dysplasia and familial gigantiform cementoma. Florid osseous dysplasia is a central lesion of the bone and periodontium, which has caused considerable controversy because of confusion regarding terminology and criteria for diagnosis. This paper reports a rare case of florid osseous dysplasia affecting maxilla and mandible bilaterally in a 14-year-old Indian male patient.

A case report of Gardner syndrome with hereditary widespread osteomatous jaw lesions

Gardner syndrome (GS) is an abnormality of familial adenomatous polyposis accompanied by characteristic jaw lesions. Gardner syndrome intestinal polyps have a 100% risk of undergoing malignant transformation; consequently, early identification and surgical intervention of the disease are important to prolong the life of the patient. We present a case of GS in a 55-year-old woman. Familial adenomatous polyposis, osteomatous jaw and ocular lesions, several dental abnormalities, and an abdominal desmoid tumor are the characteristic features of this case. This case demonstrates the presence of unusual, widespread, hereditary, osteomatous jaw lesions that caused diagnostic confusion with familial gigantiform cementoma.

Familial gigantiform cementoma with brittle bone disease, pathologic fractures, and osteosarcoma: A possible explanation of an ancient mystery

We describe four individuals of an African-American family with a predominantly diaphyseal bone disease associated with familial gigantiform cementoma (FGC), a disorder typically seen in Caucasians. The mother and her children presented with deformities of the jaws, abnormalities of the long bones, and pre-pubertal pathologic fractures. The index patient carried the diagnosis of osteosarcoma (OS). In addition, we provide a possible explanation for the jaw abnormalities of King Tutankhamen's father in the 18th dynasty in Egypt around 1350 BC.

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.

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.