Clinical and imagiological findings of central giant cell lesion and cherubism

Cone beam computed tomography (CBCT) is the best examination for bone lesions of the maxilla, allowing the dentist to evaluate precisely the behavior and components of the lesion and their relationship to the surrounding structures. Central giant cell lesion and cherubism are histologically very similar lesions. Therefore clinical and radiological examinations are fundamentally important for the diagnosis. The aim of this paper is to report two cases diagnosed as central giant cell lesions and cherubism using CBCT. This imaging modality was very important for the diagnosis of the lesions presented in the current study. It also allowed observing precisely the limits of the lesions, the components, the behavior and the exact relationship to adjacent structures.

A tomografia computadorizada de feixe cônico (TCFC) é o melhor exame para lesões ósseas da maxila, permitindo que o dentista possa avaliar com mais confiabilidade o comportamento, os componentes da lesão, e sua relação com estruturas adjacentes. A Lesão central de células gigantes e o querubismo são patologias muito semelhantes histologicamente, portanto, exames clínicos e radiológicos são de fundamental importância para o diagnóstico. O objetivo deste trabalho é relatar dois casos diagnosticados usando TCFC, um de lesões centrais de células gigantes e um de querubismo. Esta modalidade de imagem foi muito importante para o diagnóstico das patologias apresentadas neste estudo. Também permitiu observar com mais confiabilidade os limites das lesões, os componentes, o comportamento e a relação exata com as estruturas adjacentes.

Case report on cherubism: non-familial variant / Relato de caso de querubismo: uma variante não familiar

Introduction: Cherubism is a rare, non-neoplastic, self-limiting, fibro-osseous disease, characterized by painless expansion of the maxilla, mandible or both. It usually develops in the first and second year of life. The radiographic appearance presentation is ordinarily bilateral, multilocular appearance in the mandible. To the best of our knowledge, very few cases (less than ten) of non-familial cherubism have been reported in the English literature. Objective: To describe non-familial case of cherubism in a 10-year-old child. Materials and methods: The current case was clinically, radiographically and histopathologically analysed for confirmatory diagnosis. Results: H & E stained section showed vascular and cellular stroma containing numerous multinucleated giant cells. Conclusion: Correlating radiographically and histopathologically the case was finally diagnosed as non-familial variant of cherubism.

Introdução: Querubismo é uma doença rara, não neoplásica, autolimitada, fibro-óssea, caracterizada pela expansão indolor da maxila, mandíbula ou ambas. Ela geralmente se desenvolve no primeiro e no segundo ano de vida. A aparência radiográfica é normalmente bilateral, multilocular e localizada na mandíbula. Para melhor conhecimento, poucos casos (menos de dez) de querubismo não familiar foram relatados na literatura. Objetivo: Descrever um caso de querubismo não familiar em uma criança de 10 anos. Materiais e métodos: Para confirmação do diagnóstico, foram realizadas avaliações clínicas, radiográficas e histológicas deste caso. Resultados: Seção corada de H & E mostrou estroma vascular e celular contendo numerosas células gigantes multinucleadas. Conclusão: Na correlação radiográfica e histológica, ficou confirmado diagnóstico de variante não familiar de querubismo.

Cherubism - A case report with long term follow up.

Cherubism is a rare non-neoplastic disease of the bone characterized by bilateral painless enlargement of the jaws giving a cherubic appearance to the patient. It is an autosomal dominant disorder but may occur sporadically. In this paper, we have tried to explain about the clinical, radiological and histopathologic features of cherubism by presenting a case study. A case study of a 7 year old male patient, who first presented with mandibular bilateral swellings to our department in 1998, has been presented. In-depth clinical, radiological and histopathologic examination was done. An extensive long-term follow-up till 11 years was maintained. When presented for the first time, radiological investigation showed characteristic multilocular lytic lesions of the mandible bilaterally. Histopathological examination of the biopsy specimen showed proliferating fibrous connective tissue interspersed by multinucleated giant cells. It was diagnosed as a case nonfamilial cherubism. Follow-up after 5 years showed involvement of the maxilla as well, which was then corrected by surgical methods. Upon follow up 11 years after the first presentation, it was seen that the lesion was regressing by itself and there was improvement in facial contour. The natural course of Cherubism through its progression, stabilization and involution of the disease after puberty, has been highlighted in this case. More, in-depth studies to understand the nature and the pathogenesis of this condition better are required.

Querubismo: reporte de un caso / Cherubism: case study

El querubismo constituye una entidad patológica que se hereda con carácter autosómico dominante, con una expresividad variable, una penetración incompleta en algunas familias y que predomina en hombres en una proporción 2:1, aunque existen casos reportados de patologías espontáneas. Se caracteriza por ser una patología rara, benigna, autolimitada, que se presenta en edades tempranas y afecta los maxilares principalmente la mandíbula bilateralmente. El propósito de esta investigación es evaluar las características clínicas, radiográficas e histopatológicas del querubismo. Paciente niño de ocho años de edad, con expansión mandibular bilateral posterior, asintomática; y con apariencia de querubín. Las características de la patología estudiada se encuentran relacionadas con los resultados reporta dos por la literatura mundial.

Cherubism in siblings: A case report.

Cherubism is a benign disease of bones affecting the jaws and giving a characteristic cherubic appearance to the patient. On radiography, the lesions exhibit bilateral multilocular radiolucent areas. Histopathology shows numerous multinucleated giant cells in the background of proliferating fibrous connective tissue. Cherubism can be a solitary case. The present report describe cherubism in two siblings and briefly review the literature on this subject.

Non-familial cherubism--a case report.

A case of cherubism in a 9 year old boy with no familial history is presented. Clinical features, histologic appearance, radiographic findings, differential diagnosis and rationale for treatment is discussed.

Querubismo / Cherubism

El querubismo es una anomalía genética de carácter hereditario autosómico dominante que afecta el tejido óseo en forma uni o bimaxilar. Junto con la displasia fibrosa -de acuerdo con la clasificación de la IRCOT- se encuentra dentro de las lesiones óseas no neoplásicas. Las manifestaciones comienzan entre los 18 meses y los 4 años de edad. El tamaño de los maxilares aumenta hasta aproximadamente los 7 años. Durante los últimos años de la adolescencia, la enfermedad puede sufrir involución o ser activa hasta aproximadamente los 20 años, especialmente cuando se presenta en el maxilar inferior. Radiográficamente se observan zonas osteolíticas extensas. El cuadro histológico muestra un estroma fibroso, altamente vascular, con células gigantes multinucleadas y un pequeño manguito colágeno alrededor de los vasos sanguíneos. Según nuestra experiencia, el tratamiento a seguir será efectuar controles frecuentes clínico-radiográficos, reservando la cirugía para aquellos casos en los que los trastornos funcionales y estéticos deben ser resueltos antes de esperar la remisión espontánea.