Pindborg tumor associated with a supernumerary tooth: a case report

The calcifying epithelial odontogenic tumor is a rare benign neoplasm that accounts for approximately 1% of all odontogenic tumors. Most of the cases occur in the posterior mandible, and a few involve the maxilla. Despite their relatively indolent biological behavior, tumors in the maxilla tend to grow fast. We report the case of a 33-year-old female patient exhibiting swelling in the right maxilla. An isodense area associated with an impacted supernumerary tooth was found on imaging examination. The histopathologic diagnosis was a calcifying epithelial odontogenic tumor. The treatment of choice was surgical removal of the lesion and associated dental elements. The patient has been followed up for 11 months and shows no signs of recurrence. Besides describing this case, we reviewed the literature on the association of calcifying epithelial odontogenic tumors with supernumerary teeth and found two case reports addressing this subject.

Calcifying odontogenic cyst associated with the impacted third molar: a case report.

Calcifying odontogenic cyst (COC) is a benign, locally aggressive, slow-growing lesion. Its occurrence constitutes about 2% of all odontogenic cysts. The most frequent sign is painless, slow growing swelling. Radiographically it appears as a well-defined unilocular radiolucency. The microscopical features of lesion showed well-delineated cystic proliferation of odontogenic epithelium with ghost cells and fibrous connective tissue wall. In the present study, 42 years old man who has COC associated with the impacted third molar treated with enucleation is reported after 6 months follow-up.

Calcifying Odontogenic Cyst Associated With Dentigerous Cyst in a 15-Year-Old Girl.

Calcifying odontogenic cyst (COC) is a rare odontogenic cyst with ameloblastic epithelial lining containing clusters of ghost cells. COCs have been described in association with several odontogenic tumors, more commonly odontomas and rarely with dentigerous cyst (DC). In this article, we describe a case of COC associated with DC in a 15-year-old girl, who presented with a swelling on the right middle third of the face, producing facial asymmetry. Panoramic radiography showed a well-circumscribed, corticated, and unilocular radiolucency at the level of the right maxillary sinus, involving 2 unerupted premolars. The lesion was enucleated and histologically revealed a COC associated with DC, which presented mucous metaplasia. Immunohistochemical reactions were performed to better illustrate this rare synchronous occurrence of COC and DC, showing positivity for CK5, CK14, CK19, and p63 in both lesions. CK18 was negative in COC, and Bcl-2 was negative in DC. Periodic acid Schiff highlighted the mucous cells in the DC lining.

Ortho Keratinized Odontogenic Cyst with Dentinoid Formation.

Orthokeratinized odontogenic keratocyst (OOC) was first identified as orthokeratinized variant of odontogenic keratocyst in 1981 by Wright, due to its different histology and relatively low recurrence rate. Parakeratinized odontogenic cyst is now considered as keratocystic odontogenic tumor (KCOT) owing to its neoplastic nature. Although rare, calcification in the form of dystrophic calcification, dentinoid and cartilage has been reported in KCOT, but calcification in OOC is extremely rare and no case in English literature has been reported so far. Here, we report the first case of OOC with calcification located in the mandible of a 40-year female.

Orthokeratinized Odontogenic Cyst with an Associated Keratocystic Odontogenic Tumor Component and Ghost Cell Keratinization and Calcifications in a Patient with Gardner Syndrome.

Gardner syndrome (GS) is caused by mutations in the APC and besides adenomatous colorectal polyps includes such manifestations as osteomas, epidermoid cysts (ECs) and occasionally multiple pilomatricomas. More than 50 % of ECs in patients with GS exhibit pilomatricoma-like ghost cell keratinization. The latter may be explained by the fact that the development of both GS and pilomatricoma is driven by activation of the Wnt/ß-catenin signaling pathway. A 62-year-old, Caucasian male with history of GS presented with a unilocular, mixed radiopaque/radiolucent mandibular lesion causing divergence and external root resorption of involved teeth. Histopathologically, the lesion was composed of two cystic components, an orthokeratinized odontogenic cyst (OOC) and a smaller one with characteristics of keratocystic odontogenic tumor (KCOT) featuring, focally, ghost cells and an epithelial morule-like structure. Dystrophic calcifications essentially similar to those seen in pilomatricomas were observed in the fibrous connective tissue wall. The KCOT and OOC epithelia revealed strong and diffuse cytokeratin (AE1/AE3) and ß-catenin immunoreactivity. CD10 positive immunostaining was seen in the keratin and superficial spinous cell layers in both OOC and KCOT. The intraepithelial and mural ghost cells showed a cytokeratin (+), ß-catenin and CD10 (-) immunophenotype. The diagnosis of OOC with ghost cell calcifications in association with KCOT was rendered. The patient was lost to follow-up. Although a coincidental co-existence cannot be excluded, ghost cell calcifications mimicking pilomatricoma-like changes in an unusual odontogenic cyst combining OOC and KCOT features as seen in this patient with GS may be explained by the common molecular mechanisms underlying the pathogenesis of cutaneous pilomatricomas and GS.

Mixed periapical lesion: differential diagnosis of a case.

A radicular cyst associated with carious teeth is a very common odontogenic lesion in the oral cavity, but calcifications in residual radicular cysts are quite rare. We report one such case where a routine pre-implant radiographic assessment revealed a mixed periapical radiopaque radiolucent lesion in the right maxillary central incisor region. Histological and radiographic studies show that there is a slow increase in the mineralized deposits within the cyst lumen with time. This becomes prominent histochemically in cysts more than 8 years old and radiographically 6 years later, as seen in our case. In this paper we would like to highlight the importance of a residual radicular cyst with calcifications in the differential diagnosis of a mixed periapical radiopaque radiolucent lesion.

Genetic screening for nevoid basal cell carcinoma syndrome in patients with a solitary keratocystic odontogenic tumour is not useful.

AIM: The aim of this study was to investigate the need for routine genetic counselling for identification of features of nevoid basal cell carcinoma syndrome (NBCCS) in patients presenting with a solitairy keratocystic odontogenic tumour (KCOT) of the jaws. METHODS: Sixty-nine patients treated for a solitary KCOT have been followed for the possible development of second KCOTs or other signs indicative of NBCCS. In addition, 11 randomly selected patients of this group were referred for genetic counselling, including identification of germ-line mutations in the Patched gene (PTCH gene). RESULTS: In none of the 69 patients clinical and radiographic manifestations of second KCOTs and/or other features associated with NBCCS were found during a follow-up period of 49.8 months. In the 11 patients referred for genetic counselling, there were no features indicative of the presence of NBCCS. No mutations in the PTCH gene could be identified. CONCLUSION: This study does not support the need for routine genetic counselling in patients presenting with a solitairy keratocystic odontogenic tumour of the jaws.

Tumor odontogênico epitelial calcificante: relato de um caso clínico / Calcifyng epithelial odontogenic tumor: Case report

O tumor odontogênico epitelial calcificante (TOEC) ou tumor de Pindborg é uma neoplasia odontogênica rara de natureza benigna e comportamento biológico localmente agressivo. Apresenta imagem radiográfica de padrão misto devido à presença de calcificações concêntricas observadas microscopicamente. É caracterizado ainda pela presença de células epiteliais poliédricas, pontes intercelulares e depósitos eosinofílicos identificados como amilóide. O objetivo deste trabalho é apresentar um caso clínico de TOEC em paciente do sexo feminino, 51 anos, que procurou o Serviço de Estomatologia da Faculdade de Odontologia da Universidade Federal da Bahia com limitação de abertura bucal e discreto aumento de volume na região posterior da mandíbula no lado esquerdo. Exames imaginológicos demonstraram presença de lesão radiolúcida extensa com áreas de calcificações dispersas, associadas ao terceiro molar não irrompido. A biopsia incisional da lesão revelou o diagnóstico de TOEC e devido à extensão da lesão optou-se pela hemimandibulectomia. O exame da peça cirúrgica confirmou o diagnóstico de TOEC. Após a reconstrução cirúrgica da mandíbula, a paciente encontra-se sob acompanhamento clínico-radiográfico. Com base nos dados revistos da literatura, foram discutidas as características clínicas, imaginológicas e histopatológicas da lesão

Tumor odontogénico epitelial calcificante (tumor de Pindborg): a propósito de un caso / Calciying epithelial odontogenic tumor (Pindborg tumor): a case report

El tumor odontogénico epitelial calcificante es una neoplasia odontogénica benigna muy infrecuente que fue descrita por primera vez por Pindborg en 1955. La literatura registra sólo unos 160 casos, lo que representa menos de 1 por ciento de todas las lesiones odontogénicas. Presentamos el caso de una mujer de 57 años de edad que consultó por crecimiento progresivo e indoloro del maxilar inferior, de varios meses de evolución. Se realizó una mandibulectomía parcial con resección completa de la lesión, emitiéndose el diagnóstico histológico de tumor odontogénico epitelial calcificante. Se comentan las características histológicas, inmunohistoquímicas y ultraestructurales del tumor, así como los principales aspectos de su histogénesis, diagnóstico diferencial, pronóstico y tratamiento (AU)

Cisto odontogênico calcificante: revisäo de literatura e relato de um caso / Odonttogenic cyst: literature review and case related

É apresentado um caso de cisto odontogênico calcificante associado a odontoma, em uma paciente de 25 anos de idade, no qual se optou pela enucleaçäo da lesäo sob anestesia geral. Com base na literatura revisada, discutem-se a etiologia, os apectos clínicos, radiográficos, histológicos e seus grupos básicos com suas subdivisöes. O tratamento desta condiçäo é, na maioria dos casos, conservador, sendo muito pequeno o índice de recidiva

Calcifying odontogenic cyst associated with odontoma: a possible separate entity (odontocalcifying odontogenic cyst).

The calcifying odontogenic cyst (COC) has been reported to be associated with odontoma in about 24% of cases. Separation of the cases of COC associated with odontoma (COCaO) may lead to a better understanding of the pathogenesis of this lesion. A screen of the literature revealed 52 cases of COCaO. The male to female ratio was 1:1.9, with a mean age of 16 years. The most common location was the maxilla (61.5%). The radiographic appearance of most cases (80.5%) was of a well-defined, mixed radiolucent-radiopaque lesion. Histologically, the lesions usually consisted of a single large cyst (which is similar to simple COC) with tooth-like structures that appear to be an integral part of the lesion, giving the impression of a single lesion. In addition to the unique histologic features, differences in gender and in distribution were found between the cases of COCaO and those of simple COC. COCaO may be regarded as a separate entity and classified as a benign, mixed odontogenic tumor. The term odontocalcifying odontogenic cyst is suggested.

Cisto odontogênico calcificante: relato de um caso e revisäo da literatura / Calcifying odontogenic cyst: case related and literature review

O cisto odontogênico calcificante compreende uma lesäo rara dos maxilares que apresenta como característica principal estar localizado centralmente no interior do osso. Ocorre com mais freqüência na segunda década de vida e é praticamente igual na maxila e mandíbula. Alguns foram encontrados (aproximadamente 1/4) em tecidos moles relacionados com os ossos já citados. No presente trabalho, é relatado um caso localizado na maxila, acompanhado de revisäo de uma regiäo da literatura pertinente à essa patologia