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BMJ Case Reports May 2014Radicular cysts are inflammatory odontogenic cysts of tooth bearing areas of the jaws. Most of these lesions involve the apex of offending tooth and appear as...
Radicular cysts are inflammatory odontogenic cysts of tooth bearing areas of the jaws. Most of these lesions involve the apex of offending tooth and appear as well-defined radiolucencies. Owing to its clinical characteristics similar to other more commonly occurring lesions in the oral cavity, differential diagnosis should include dentigerous cyst, ameloblastoma, odontogenic keratocyst, periapical cementoma and Pindborg tumour. The present case report documents a massive radicular cyst crossing the midline of the palate. Based on clinical, radiographical and histopathological findings, the present case was diagnosed as an infected radicular cyst. The clinical characteristics of this cyst could be considered as an interesting and unusual due to its giant nature. The lesion was surgically enucleated along with the extraction of the associated tooth; preservation of all other teeth and vital structures, without any postoperative complications and satisfactory healing, was achieved.
Topics: Adult; Humans; Male; Maxillary Diseases; Radicular Cyst; Radiography
PubMed: 24792022
DOI: 10.1136/bcr-2014-203678 -
Journal of Maxillofacial and Oral... Mar 2022Familial gigantiform cementoma is an autosomal dominant fibro-cement osseous lesion that causes massive expansion of facial skeleton. Patients with such massive lesions...
BACKGROUND
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.
PURPOSE
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.
METHOD
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.
RESULT
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.
CONCLUSION
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.
PubMed: 35400930
DOI: 10.1007/s12663-021-01515-2 -
Journal of Oral and Maxillofacial... May 2011The 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...
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.
PubMed: 22529580
DOI: 10.4103/0973-029X.84497 -
The Journal of Clinical Pediatric... 2004Crouzon craniostenosis [MIM 123500], is identified on the basis of the additional phenotypical manifestations of acanthosis nigricans, vertebral changes and cementomas...
Crouzon craniostenosis [MIM 123500], is identified on the basis of the additional phenotypical manifestations of acanthosis nigricans, vertebral changes and cementomas of the jaws. Choanal atresia and hydrocephalus are other features. The molecular defect in CDSS is a point mutation in the FGFR3 gene on chromosome 4p, whereas, the mutation in the Crouzon syndrome is in the FGFR2 gene at 10q25.3-26. An affected girl aged 2 years presented at the UWC dental genetics unit with a prior diagnosis of Crouzon syndrome. Choanal atresia had necessitated a permanent tracheostomy, and hydrocephalus was managed by a shunt operation. Clinical examination revealed acanthosis nigricans in the axilliary regions, a diagnosis confirmed by a biopsy of the lesion. Eruption of the primary dentition was delayed with only six out of twenty teeth present. Radiographic examination conducted shortly after birth revealed the presence of several tooth buds in both the maxillae and the mandible. The delayed eruption of the teeth will be of significance in future orthodontic and maxillofacial measures for the improvement of the patient's facial Crouzonodermoskeletal syndrome (CDSS) was separated from the classical appearance. Molecular investigations in the girl and her parents are underway. If the specific mutation in FGFR3 is observed, a positive diagnosis of CDSS will be confirmed and the status of her parents and other family members will be determined. On this basis, appropriate genetic management can be offered to the kindred.
Topics: Acanthosis Nigricans; Anodontia; Choanal Atresia; Chromosomes, Human, Pair 4; Craniofacial Dysostosis; Facies; Female; Fingers; Humans; Hydrocephalus; Infant, Newborn; Point Mutation; Protein-Tyrosine Kinases; Receptor, Fibroblast Growth Factor, Type 3; Receptors, Fibroblast Growth Factor; Retrognathia; Spine; Syndrome
PubMed: 14969379
DOI: 10.17796/jcpd.28.2.72m01l5g50448548 -
BMC Musculoskeletal Disorders Mar 2017The cementoma is a common disease of the dental root apex, which generally occurs in the maxilla and the mandible, but the cementoma occurring in the long bone is rare....
BACKGROUND
The cementoma is a common disease of the dental root apex, which generally occurs in the maxilla and the mandible, but the cementoma occurring in the long bone is rare. Moreover, the incidence of cementoma in the calcaneus is extremely infrequent.
CASE PRESENTATION
The present study reports an unusual case of a 19-year-old girl, who complained of pain in the left heel. Subsequent radiographs and computed tomography (CT) were used in the diagnosis. The imaging features of the lesion included a radiopaque matrix and radiolucent tissue, particularly an arc-shaped fat band. An excisional biopsy was performed. Histopathological examination confirmed the diagnosis of cementoma in the calcaneus. After the operation, the patient was followed up without recurrence.
CONCLUSIONS
Imaging examination plays an important role in the differential diagnosis of cementoma of the calcaneus.
Topics: Bone Transplantation; Calcaneus; Cementoma; Female; Follow-Up Studies; Humans; Young Adult
PubMed: 28288612
DOI: 10.1186/s12891-017-1460-1 -
Head and Neck Pathology Jun 2009
Topics: Adolescent; Cementoma; Dental Cementum; Humans; Incidental Findings; Male
PubMed: 19644548
DOI: 10.1007/s12105-008-0099-5 -
Head and Neck Pathology Mar 2020Gnathic fibro-osseous lesions are a diverse group of disease processes which share overlapping microscopic features characterized by fibroblastic stroma with variable... (Review)
Review
Gnathic fibro-osseous lesions are a diverse group of disease processes which share overlapping microscopic features characterized by fibroblastic stroma with variable cellularity and a range of bone forming pathological processes leading to woven, sclerotic and cementum-like structures. Some of the lesions are unique to craniofacial location and a combination of clinical, radiological and pathological correlation is often necessary for diagnostic accuracy. Gnathic osteosarcomas are rare tumors with differences in age distribution and behavior as compared to osteosarcoma of long bones. This review will discuss the clinicopathological and radiological features of gnathic fibro-osseous lesions and osteosarcoma with updates on current genetics and molecular pathogenesis.
Topics: Cementoma; Fibroma, Ossifying; Fibrous Dysplasia of Bone; Head and Neck Neoplasms; Humans; Osteomyelitis; Osteosarcoma
PubMed: 31950477
DOI: 10.1007/s12105-019-01094-2 -
Head and Neck Pathology Jun 2023Cementoblastoma (CB) is unique among odontogenic tumors because its gross pathological anatomy is pathognomonic in most cases, i.e., a rounded calcified growth that is...
Cementoblastoma (CB) is unique among odontogenic tumors because its gross pathological anatomy is pathognomonic in most cases, i.e., a rounded calcified growth that is fused to the root of a tooth and completely encapsulated by fibrous tissue. The resulting radiographic appearance is a well-defined, globular mixed radiopaque/lucent or completely radiopaque mass obliterating some details of the root, with a thin radiolucent zone surrounding the central opacity. Although hundreds of publications have covered the clinicopathologic features of CB, almost nothing is known about its true history. Also it seems there is little understanding about how the term "CB" was originally introduced as a pathologic entity. This report covers some overlooked papers on CB dating back to the 19th century, including the first complete description in 1888 and the first radiographic presentation in 1906.
Topics: Humans; Cementoma; Odontogenic Tumors
PubMed: 36723849
DOI: 10.1007/s12105-022-01499-6 -
Revista Cientifica Odontologica... 2021Familial gigantiform cementoma (FGC) is a rare benign fibro-cementum lesion, which follows an autosomal dominant inheritance pattern and presents during childhood. It is... (Review)
Review
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.
PubMed: 38464860
DOI: 10.21142/2523-2754-0903-2021-073 -
Molecular Genetics & Genomic Medicine Jan 2024Familial gigantiform cementoma (FGC) is a rare tumor characterized by the early onset of multi-quadrant fibro-osseous lesions in the jaws, causing severe maxillofacial... (Review)
Review
BACKGROUND
Familial gigantiform cementoma (FGC) is a rare tumor characterized by the early onset of multi-quadrant fibro-osseous lesions in the jaws, causing severe maxillofacial deformities. Its clinicopathological features overlap with those of other benign fibro-osseous lesions. FGC eventually exhibits progressively rapid growth, but no suspected causative gene has been identified.
METHODS
In this study, three patients with FGC were recruited, and genomic DNA from the tumor tissue and peripheral blood was extracted for whole-exome sequencing.
RESULTS
Results showed that all three patients harbored the heterozygous mutation c.1067G > A (p.Cys356Tyr) in the ANO5 gene. Furthermore, autosomal dominant mutations in ANO5 at this locus have been identified in patients with gnathodiaphyseal dysplasia (GDD) and are considered a potential causative agent, suggesting a genetic association between FGC and GDD. In addition, multifocal fibrous bone lesions with similar clinical presentations were detected, including five cases of florid cemento-osseous dysplasia, five cases of polyostotic fibrous dysplasia, and eight cases of juvenile ossifying fibromas; however, none of them harbored mutations in the ANO5 gene.
CONCLUSION
Our findings indicate that FGC may be an atypical variant of GDD, providing evidence for the feasibility of ANO5 gene testing as an auxiliary diagnostic method for complex cases with multiple quadrants.
Topics: Humans; Cementoma; Mutation; Jaw Neoplasms; Anoctamins; Osteogenesis Imperfecta
PubMed: 37649308
DOI: 10.1002/mgg3.2277