Cementoblastoma: relato de caso / Cementoblastoma: case report / Cementoblastoma: reporte de caso

O cementoblastoma é uma neoplasia odontogênica benigna, rara, responsável por 1% dos tumores odontogênicos. A localização mais comum é a região do primeiro molar inferior. Ela afeta principalmente a segunda e terceira décadas de vida, com crescimento lento, pode causar abaulamento cortical. Radiograficamente apresenta-se como uma lesão radiopaca, circunscrita por uma fina área radiolúcida, envolvendo a raiz dentária. Histologicamente, pode-se observar uma massa mineralizada, com aspecto cementóide, aderida às raízes do dente. Casos que apresentam expansão da cortical óssea aos exames clínico e imaginológico requerem tratamento cirúrgico por meio da curetagem da lesão e remoção do elemento dentário associado. Paciente do sexo feminino, leucoderma, 30 anos, procurou atendimento sem queixa de dor, apresentando abaulamento do osso cortical na região inferior esquerda do primeiro molar. Exames de imagem mostraram lesão radiopaca associada à raiz do elemento dentário 36, circunscrita com halo radiolúcido. A tomografia computadorizada mostra uma massa radiopaca na região apical do elemento. Biópsia excisional e exodontia do dente 46 foram realizadas. Material foi enviado para análise histopatológica e o diagnóstico foi cementoblastoma. A paciente está em proservação(AU)

Cementoblastoma is a benign, rare odontogenic neoplasm responsible for 1% of odontogenic tumors. The most common location is the lower first molar region. It mainly affects the second and third decades of life, with slow growth, can cause cortical bulging. Radiographically it presents as a radiopaque lesion, circumscribed by a thin radiolucent area, surrounding the dental root. Histologically, one can observe a mineralized mass, with cementoid aspect, adhered to the roots of the tooth. Cases that present expansion of the cortical bone to the clinical and imaging exams require surgical treatment by means of curettage of the lesion and removal of the associated dental element. A 30-year-old female patient, leucoderma, sought care without complaint of pain, showing bulging of the cortical bone in the lower left region of the first molar. Imaging studies showed radiopaque lesion associated with the root of the dental element 36, circumscribed with radiolucent halo. Computed tomography shows a radiopaque mass in the apical region of the element. Excisional biopsy and tooth extraction 46 were performed. Material was sent for histopathological analysis and the diagnosis was cementoblastoma. The patient is in proservation(AU)

El cementoblastoma es una neoplasia odontogénica benigna, rara, responsable del 1% de los tumores odontogénicos. La ubicación más común es la región del primer molar inferior. Se afecta principalmente a la segunda y tercera décadas de vida, con un crecimiento lento, puede causar abombamiento cortical. Radiograficamente se presenta como una lesión radiopaca, circunscrita por una fina área radiolúcida, envolviendo la raíz dental. Histológicamente, se puede observar una masa mineralizada, con aspecto celoide, adherida a las raíces del diente. Los casos que presentan expansión de la cortical ósea a los exámenes clínicos e imaginológico requieren tratamiento quirúrgico por medio del curetaje de la lesión y remoción del elemento dental asociado. El paciente de sexo femenino, leucoderma, 30 años, buscó atención sin queja de dolor, presentando abombamiento del hueso cortical en la región inferior izquierda del primer molar. Los exámenes de imagen mostraron lesión radiopaca asociada a la raíz del elemento dental 36, circunscrita con halo radiolúcido. La tomografía computarizada muestra una masa radiopaca en la región apical del elemento. Se realizó la biopsia excisional y la exodoncia del diente 46. El material fue enviado para análisis histopatológicos y el diagnóstico fue cementoblastoma. El paciente está en reposo(AU)

Dental Abnormalities Caused by Novel Compound Heterozygous CTSK Mutations.

Cathepsin K (CTSK) is an important protease responsible for degrading type I collagen, osteopontin, and other bone matrix proteins. The mutations in the CTSK gene can cause pycnodysostosis (OMIM 265800), a rare autosomal recessive bone dysplasia. Patients with pycnodysostosis have been reported to present specific dental abnormalities; however, whether these dental abnormalities are related to dysfunctional CTSK has never been reported. Here we investigated the histologic changes of cementum and alveolar bone in a pycnodysostosis patient, caused by novel compound heterozygous mutations in the CTSK gene (c.87 G>A p.W29X and c.848 A>G p.Y283C). The most impressive manifestations in tooth were extensive periradicular high-density clumps with unclear periodontal space by orthopantomography examination and micro-computed tomography scanning analysis. Hematoxylin/eosin and toluidine blue staining and atomic force microscopy analysis showed that the cementum became significantly thickened, softened, and full of cementocytes. The disorganized bone structure was the main character of alveolar bone. The p.W29X mutation may represent the loss-of-function allele with an earlier termination codon in the precursor CTSK polypeptide. Residue Y283 is highly conserved among papain-like cysteine proteases. Three-dimensional structure modeling analysis found that the loss of the hydroxybenzene residue in the Y283C mutation would interrupt the hydrogen network and possibly affect the self-cleavage of the CTSK enzyme. Furthermore, p.Y283C mutation did not affect the mRNA and protein levels of overexpressed CTSK in COS-7 system but did reduce CTSK enzyme activity. In conclusion, the histologic and ultrastructural changes of cementum and alveolar bone might be affected by CTSK mutation via reduction of its enzyme activity (clinical trial registration: ChiCTR-TNC-10000876).

Concrescence: assessment of case by periapical radiography, cone beam computed tomography and micro-computed tomography.

The aim of this article was to describe imaging aspects of concrescence analyzed by three imaging modalities. A second molar joined together with a third molar was imaged using digital periapical radiography, cone beam computed tomography (CBCT) and micro-computed tomography (Micro-CT). On periapical radiograph, the mesial root of the third molar is superimposed on the distal root of the second molar. On CBCT images, a large cementum union between bulbous roots was detected, confirming the diagnosis of concrescence. On micro-CT images, the cementum union appeared limited to the apical third of the roots. In conclusion, both computed tomography modalities allowed for the diagnosis of concrescence. However, only micro-CT provided the real extension of the cementum union.

Root malformation associated with a cervical mineralized diaphragm--a distinct form of tooth abnormality?

OBJECTIVE: Deviations in length and shape of tooth roots result from hard tissue resorption or occur as a developmental disorder. The purpose of this report is to present a type of root malformation which seems to have gone unreported so far. STUDY DESIGN: Two patients showing severely dysplastic roots of all permanent first molars were evaluated using radiography, histology as well as scanning and transmission electron microscopy. RESULTS: Medical histories of the patients revealed significant, but diverse events in the first year after birth. Radiographically the pulp cavity floors of the affected molars in large part were occupied by ectopic mineralized plates. Microscopically these plates consisted of hard tissue, densely calcified globules, and a network of canals which contained large blood vessels and were lined by cementum and periodontal ligament. CONCLUSIONS: We propose that the ectopic mineralized plate was derived from the dental follicle, had developed during crown formation around the vascular plexus at the base of the dental papilla, and represented a mechanical obstacle interfering with normal root development.

Dental abnormalities in a mouse model for craniometaphyseal dysplasia.

Mice carrying a knock-in mutation (Phe377del) in the Ank gene replicate many skeletal characteristics of human craniometaphyseal dysplasia, including hyperostotic mandibles. Ank (KI/KI) mice have normal morphology of erupted molars and incisors but excessive cementum deposition with increased numbers of Ibsp- and Dmp1-positive cells on root surfaces. The cervical loops of adult Ank (KI/KI) lower incisors are at the level of the third molars, while they are close to the mandibular foramen in Ank (+/+) mice. Furthermore, Ank (KI/KI) incisors show decreased eruption rates, decreased proliferation of odontoblast precursors, and increased cell apoptosis in the stellate reticulum. However, their capability for continuous elongation is not compromised. Quantification of TRAP-positive cells in the apical ends of Ank (KI/KI) incisors revealed decreased osteoclast numbers and osteoclast surfaces. Bisphosphonate injections in Ank (+/+) mice replicate the Ank (KI/KI) incisor phenotype. These results and a comparison with the dental phenotype of Ank loss-of-function mouse models suggest that increased cementum thickness may be caused by decreased extracellular PPi levels and that the incisor phenotype is likely due to hyperostosis of mandibles, which distinguishes Ank (KI/KI) mice from the other Ank mouse models.

Hypoplastic root cementum and premature loss of primary teeth in Coffin-Lowry syndrome: a case report.

BACKGROUND: Coffin-Lowry syndrome (CLS) is a rare genetic disorder. The syndrome presents with psychomotor retardation, short stature, skeletal deformations, digit abnormalities, and distinctive facial features. Oral and dental findings in CLS are common and they include thick prominent lips, high palate, midline lingual furrow, hypodontia, microdontia, delayed eruption, and early tooth loss. Only one earlier case suggesting hypoplastic root cementum as cause for primary loss of teeth in CLS has been published. CASE REPORT: This case describes a 3-year-old boy with premature loss of primary incisors without preceding root resorption. In addition to the dental findings, the boy had several general signs and symptoms and the dental findings together with the other characteristics led to the clinical diagnosis of CLS, which later was genetically verified. Histological analysis of an extracted primary incisor showed hypoplastic root cementum. CONCLUSION: Hypoplastic root cementum may explain early tooth loss in CLS. As early loss of primary teeth is rare, especially when there is no previous root resorption, the individual is likely to seek dental care. Thus, the dentist may play an important role in assisting in the diagnosing of CLS.

Genetic etiology and dental pulp cell deficiency of hypophosphatasia.

Hypophosphatasia is caused by mutations of the tissue-non-specific alkaline phosphatase (TNSALP) gene with deficiency of dentin structure. The aim of this study was to examine whether TNSALP mutation in dental pulp cells contributes to dentin dysplasia in hypophosphatasia. Mutation analysis showed that compound heterozygous mutations of TNSALP were identified in three hypophosphatasia patients, including 3 novel mutation sites. Exfoliated teeth from the patients showed abnormal dentin mineralization and loss of cementum, as assessed by ground sections and scanning electron microscope analysis. Dental pulp cells isolated from one of the patients showed a significantly reduced TNSALP activity and mineralization capacity when compared with those in dental pulp cells from the unaffected individuals. Our results suggested that dentin dysplasia in hypophosphatasia may be associated with the decreased mineralization ability of dental pulp cells.

Clinical, pathological and genetic evaluations of Chinese patients with autosomal-dominant hypophosphatasia.

OBJECTIVES: Hypophosphatasia (HPP) is an inherited disorder characterised by defective bone and tooth mineralisation and deficient serum and bone alkaline phosphatase activity, and it results from mutations in alkaline phosphatase (ALPL) encoding tissue-nonspecific alkaline phosphatase (TNAP). The objective of the present work was to explore the correlations between genotype and phenotype in a Chinese family affected by autosomal-dominant HPP. DESIGN: We examined all individuals of a HPP family by clinical and radiographic examinations as well as laboratory assays. Furthermore, a prematurely exfoliated tooth was observed histopathologically. Based on the clinical and pathological manifestations, the causative gene ALPL was selected for further analysis and screened for mutations. RESULTS: The proband presented the characteristic clinical features of childhood HPP such as rachitic skeletal changes, early loss of primary teeth, and short root anomalies of the permanent teeth. Histopathological evaluation of a tooth revealed a "shell" structure, severe mineralisation defects of dentin, and an absence of cementum. The patient's mother and grandfather were clinically diagnosed with adult HPP. The family showed autosomal-dominant moderate hypophosphatasia. DNA sequencing and analysis revealed a novel missense mutation (c.251A>T) in exon4 of ALPL. This mutation (p.E84V) is located in the secondary structure of TNAP's homodimer interface, and it was predicted to have a dominant negative effect. CONCLUSION: Our findings suggest the missense transversion (c.251A>T, p.E84V) should be responsible for the HPP phenotype in this Chinese family.

Cleidocranial dysplasia: clinico-radiological illustration of a rare case.

Cleidocranial dysplasia is an autosomal dominant condition caused by mutation of RUNX2, characterized by generalized dysplasia of the bones and teeth. Affected individuals have short stature, atypical facial features, and skeletal anomalies affecting mainly the skull and clavicle. The dental manifestations are mainly delayed exfoliation of the primary teeth and delayed eruption of the permanent teeth, with multiple impacted supernumeraries, and absence of cellular cementum. The frequency of this disorder is 1 per million individuals. Here we report a rare case of CCD in a 9-year-old male patient having most of the characteristic features of this syndrome. Interestingly, disorganized dentinal tubules were found in the roots of an extracted deciduous first molar, which seems to be a unique feature not reported previously.

Familial hypophosphatemic rickets.

Rickets is the failure of mineralization of osteoid and newly formed bones in a child skeleton. It is commonly associated with vitamin D deficiency; however, it can be because of a decrease in the serum phosphate levels leading to inadequate mineralization of cartilage and bone, consequent skeletal deformities, and growth retardation. The hypophosphatemic conditions that interfere in bone mineralization comprise many hereditary or acquired diseases. One of the hereditary types of hypophosphatemic rickets is the familial hypophosphatemic rickets. This rare variety was diagnosed in a 9-year-old patient who had come with a chief complaint of a missing tooth. In the present case, radiographic aspects of oral and systemic manifestations of familial hypophosphatemic rickets are highlighted.

Aberrant cementum phenotype associated with the hypophosphatemic hyp mouse.

BACKGROUND: Cementogenesis is sensitive to altered local phosphate levels; thus, we hypothesized a cementum phenotype, likely of decreased formation, would be present in the teeth of X-linked hypophosphatemic (Hyp) mice. Mutations in the phosphate-regulating gene with homologies to endopeptidases on the X chromosome (Phex) cause X-linked hypophosphatemia, characterized by rickets, osteomalacia, and hypomineralized dentin formation, a phenotype recapitulated in the Hyp mouse homolog. Here, we report a developmental study of tooth root formation in Hyp mouse molars, focusing on dentin and cementum. METHODS: Light and transmission electron microscopy were used to study molar tissues from wild-type (WT) and Hyp mice. Demineralized and hematoxylin and eosin-stained tissues at developmental stages 23 to 96 days postcoital (dpc) were examined by light microscopy. Immunohistochemistry methods were used to detect bone sialoprotein (BSP) distribution in Hyp and WT mouse molar tissues, and transmission electron microscopy was used to study similar molar tissues in the non-demineralized state. RESULTS: Dentin in Hyp mice exhibited mineralization defects by 33 dpc, as expected, but this defect was partially corrected by 96 dpc. In support of our hypothesis, a cementum phenotype was detected using a combination of immunohistochemistry and transmission electron microscopy, which included thinner BSP-positive staining within the cementum, discontinuous mineralization, and a globular appearance compared to WT controls. CONCLUSION: Mutations in the phosphate-regulating Phex gene of the Hyp mouse resulted in defective cementum development.

Crouzon's syndrome: tooth morphological and microanalytical evaluation.

AIM: To assess the morphological and microanalytical structure of teeth in Crouzon's Syndrome. METHODS: A 21 year old patient with this condition was evaluated to demonstrate tooth morphology in this syndrome. Assessment of 4 teeth from this patient consisted of morphological analysis in SEM as well as microanalysis of elements. Sections were created in order to assess any defects of the enamel, dentine, root, and any demineralisation. RESULTS: Micro-analysis using x-ray techniques revealed lower levels of calcium and magnesium from this patient with Crouzon's as compared with samples from a normal healthy patient of comparable age. Furthermore, in the subject's teeth notable amounts of lead were found. CONCLUSION: Histological images revealed morphological abnormalities especially in dentine. Furthermore, in the dentine levels of calcium and magnesium were lowered according to the microanalytical tests.

Tratamiento de conducto en pieza dentaria con hipercementosis / Endodontic treatment in root with hipercementosis

Se presenta el caso de una paciente de 40 años que manifiesta a los cambios térmicos, dolor espontáneo y nocturno. Al examinar la pieza 1.2 se observa lesión cariosa externa. A la evaluación radiográfica se observa una imagen radiolúcida compatible con caries que compromete la cámara pulpar, y una imagen radiopaca de aproximadamente 4mm. de diámetro compatible con hipercementosis a nivel del tercio apical. Se realiza el tratamiento endodóntico de la pieza dentaria. Finalmente desaparecen los síntomas y el resultado es satisfactorio.

Were present the case of a 40-year-old, who expreses sensitivity to termal changes, spontaneous pain, nocturnal pain. In reviewing the piece 1.2 carious lesión is observed extensive. The evaluation notes radiographic shows a radiolucent image that fits with caries, and compromisos the pulpar chamber; there are also was a radiopaque image with 4.0 mm. diameter that fits with hipercementosis apical third leve lis observed partial obliteration duct. We proceed to make endodontic treatment and finally the symptoms gave up having a good end.

Root surface characteristics of children teeth with periodontal diseases.

The present study examined the root surfaces of teeth from children with or without periodontal diseases and with or without systemic diseases. Light microscopy revealed that when compared to control teeth: teeth with chronic periodontitis had similar radicular histology; teeth from children with leukocyte adhesion deficiency, Down syndrome and aggressive periodontitis had narrower cementum areas; teeth from children with hypophosphatasia showed cementum aplasia. Cementum anomalies may facilitate the establishment and progress of periodontitis in children.

Concrescence: report of rare complication.

Concrescence is an uncommon developmental anomaly that may influence exodontia as well as periodontal, endodontic, orthodontic and even prosthodontic diagnosis and treatment planning. Unexpected complications arising from this condition may lead to legal complications. To minimize risk and adverse outcome of treatment, consideration should be given to recognizing this condition and, possibly, modifying treatment planning. This article presents an unsuspected case of concrescence with a review of the literature.

An overview of the dens invaginatus with case examples.

Dens invaginatus is an uncommon dental anomaly in which there is a deepening of the cingulum with infolding of the enamel and dentine. The severity varies, from mild extension into the pulp cavity, to extreme forms in which the invagination extends through the root, resulting in a second opening at or near the apex, and complex morphology. The following cases report on the different management approaches to dens invaginatus and illustrate the difficulties encountered in managing such cases.

Florid cemento-osseous dysplasia: a case report

No disponible

Cemento-osseous dysplasias are a group of disorders known to originate from periodontal ligament tissues and involve, essentially, the same pathological process. They are usually classified, depending on their extent and radiographic appearances, into three main groups: periapical (surrounds the periapical region of teeth and are bilateral), florid (sclerotic symmetrical masses) and focal (single lesion) cemental dysplasias. Florid cemento-osseous dysplasia clearly appears to be a form of bone and cemental dysplasia that is limited to jaws. Patients do not have laboratory or radiologic evidence of bone disease in other parts of the skeleton. For the asymptomatic patient, the best management consists of regular recall examinations with prophylaxis and reinforcement of good home hygiene care to control periodontal disease and prevent tooth lose. Management of the symptomatic patient is more difficult. At this stage, there is an inflammatory component to the disease and the process is basically a chronic osteomyelitis involving dysplastic bone and cementum. Antibiotics may be indicated but may not be effective. A case of florid cemento-osseous dysplasia occurring in a 47-year-old Caucasian female is reported which was rare in regard to race and sex