Beneficios de la descompresión y enucleación en lesiones quísticas de gran tamaño en la mandíbula / Benefits of decompression and enuclation in large cystic lesions in the jaw / Benefícios da descompressão e enuclação nas grandes lesões císticas na mandíbula

Las lesiones quísticas ocurren en los maxilares, por la presencia de células remanentes del neuroectodermo embrionario. La descompresión es una técnica conservadora para disminuir la presión intraquística mediante drenaje constante, permitiendo el crecimiento de nuevo hueso centrípeto de las paredes óseas del quiste. Objetivo: determinar los beneficios de la descompresión y enucleación en lesiones quísticas mandibulares, tomando como base la metodología de un caso clínico. Descripción del caso: se diagnosticó una lesión quística mandibular en paciente masculino de 27 años, que acudió a consulta mostrando secreción purulenta en mucosa trígono retromolar de UD 37, inicialmente asintomática. Se utilizaron como materiales la tomográfica computarizada de haz cónico, artefacto de drenaje autocurado, hemiarcada izquierda elaborada con Metil Metacrilato y aparato a base de cilindro. Como resultados se reveló imagen hipodensa de bordes definidos localizada en el límite posterior de cuerpo mandibular, borde anterior y parte de la rama ascendente mandibular del lado izquierdo; extendida en sentido cefálico caudal desde la cresta alveolar y borde anterior de la rama hasta la cortical superior del conducto mandibular. Conclusión: Se confirmó diagnóstico de quiste periapical, quiste residual y ameloblastoma. Se realizó biopsia incisional de la lesión para estudio histopatológico y la descompresión con dispositivo personalizado a enucleación conminada con solución de Carnoy, resultando el tratamiento conservador efectivo complementado por la enucleación de una membrana quística más gruesa y menos friable.

Cystic lesions occur in the jaws due to the presence of remnant cells of the embryonic neuroectoderm. Decompression is a conservative technique to decrease intracystic pressure by constant drainage, allowing the growth of new centripetal bone from the bony walls of the cyst. Objective: to determine the benefits of decompression and enucleation in mandibular cystic lesions, based on the methodology of a clinical case. Case description: a cystic mandibular lesion was diagnosed in a 27 year old male patient, who came for consultation showing purulent secretion in the trigone retromolar mucosa of UD 37, initially asymptomatic. The materials used were cone beam computed tomography, self-curing drainage device, left hemiarch made with Methyl Methacrylate and cylinder based apparatus. The results revealed a hypodense image with defined borders located in the posterior limit of the mandibular body, anterior border and part of the ascending mandibular branch on the left side; extended in a caudal cephalic direction from the alveolar crest and anterior border of the branch to the superior cortical of the mandibular duct. Conclusion: Diagnosis of periapical cyst, residual cyst and ameloblastoma was confirmed. An incisional biopsy of the lesion was performed for histopathological study and decompression with a customized device to enucleation with Carnoy's solution, resulting in effective conservative treatment complemented by enucleation of a thicker and less friable cystic membrane.

As lesões císticas ocorrem nos maxilares, devido à presença de células remanescentes da neuroectoderme embrionária. A descompressão é uma técnica conservadora que visa reduzir a pressão intracística por meio de drenagem constante, permitindo o crescimento de novo osso centrípeto a partir das paredes ósseas do cisto. Objetivo: determinar os benefícios da descompressão e da enucleação em lesões císticas mandibulares, com base na metodologia de um caso clínico. Descrição do caso: foi diagnosticada uma lesão cística mandibular em um paciente do sexo masculino, 27 anos, que se apresentou para consulta apresentando secreção purulenta na mucosa do trígono retromolar do UD 37, inicialmente assintomática. Os materiais utilizados foram tomografia computadorizada de feixe cônico, dispositivo de drenagem autopolimerizável, hemiarco esquerdo confeccionado com metacrilato de metila e aparelho de base cilíndrica. Os resultados revelaram uma imagem hipodensa com limites definidos localizada no limite posterior do corpo mandibular, bordo anterior e parte do ramo mandibular ascendente do lado esquerdo; estendendo-se em direção cefálica caudal desde a crista alveolar e bordo anterior do ramo até ao córtex superior do ducto mandibular. Conclusão: Foi confirmado o diagnóstico de quisto periapical, quisto residual e ameloblastoma. Foi efectuada uma biopsia incisional da lesão para estudo histopatológico e descompressão com um dispositivo adaptado à enucleação cominutiva da solução de Carnoy, resultando num tratamento conservador eficaz complementado pela enucleação de uma membrana quística mais espessa e menos friável.

Epithelial-mesenchymal markers and their correlation with clinical aspects in odontogenic keratocysts / Marcadores epitélio-mesênquima e sua correlação com aspectos clínicos em lesões de ceratocisto odontogênico

ABSTRACT Objective: Odontogenic keratocysts have a high recurrence rate and aggressive clinical behavior. The event called epithelial-mesenchymal transition is a process in which the epithelial cell loses its epithelial characteristics and acquires properties typical of mesenchymal cells. Studies have already demonstrated that odontogenic keratocysts has expression of tumor markers, but the lack of clarification about its development mechanism and molecular composition makes the therapeutic options remain limited. The aim of this study is to evaluate the expression of epithelial-mesenchymal transition marker proteins in these lesions, correlating the expression of these proteins with clinical aspects of each case. Methods: Patients with odontogenic keratocysts diagnoses, treated by the Department of Oral and Maxillofacial Surgery of the Erasto Gaertner Hospital, Curitiba, Brazil in the period between 2016 and 2019 were evaluated by immunohistochemical analysis, to assess the expression of epithelial-mesenchymal transition markers (Vimentin, beta-catenin and E-cadherin) by qualitative analysis. Results: Eighteen patients were included, with a mean age of 43 years, and most of them were male. The mandible was more affected than the maxilla. No association between the clinical characteristics of the cysts and the immunohistochemical profile for epithelial-mesenchymal transition proteins was observed. Conclusion: The positivity of E-cadherin and negativity of vimentin demonstrates that its function is preserved. Loss of function of E-cadherin is associated with worse prognosis. The identification of the epithelial-mesenchymal transition process as a prognostic marker for odontogenic cysts and tumors could be an important tool for defining treatment.

RESUMO Objetivo: O ceratocisto odontogênico têm uma alta taxa de recorrência e comportamento clínico agressivo. O evento chamado transição epitelial-mesênquima (TEM) é um processo no qual a célula epitelial perde suas características epiteliais e adquire propriedades típicas das células mesenquimais. Estudos já demonstraram que o ceratocisto odontogênico tem expressão de marcadores tumorais, mas a falta de esclarecimento sobre seu mecanismo de desenvolvimento e composição molecular faz com que as opções terapêuticas permaneçam limitadas. O objetivo deste estudo é avaliar a expressão das proteínas marcadoras de transição epitelial-mesênquima nestas lesões, correlacionando a expressão destas proteínas com os aspectos clínicos de cada caso. Métodos: Os pacientes com diagnóstico de ceratocisto odontogênico, tratados pelo Serviço de Cirurgia Bucomaxilofacial do Hospital Erasto Gaertner, Curitiba, Brasil, no período entre 2016 e 2019, foram avaliados por análise imunohistoquímica, para avaliar a expressão dos marcadores transição epitelial-mesênquima (Vimentina, beta-catenina e E-cadherina). Resultados: Foram incluídos 18 pacientes, com idade média de 43 anos, e a maioria deles eram do sexo masculino. A mandíbula foi mais afetada do que a maxila. Não foi observada associação entre as características clínicas dos cistos e o perfil imuno-histoquímico das proteínas transição epitelial-mesênquima. Conclusão: A positividade da E-caderina e a negatividade da vimentina demonstram que a sua função está preservada. A perda da função da E-caderina está associada a um pior prognóstico. Identificar o processo da transição epitelial-mesênquima como um marcador de prognóstico para cistos e tumores odontogênicos pode ser uma ferramenta importante para definir o tratamento dessas lesões.

Cyst or Tumor? A systematic review and meta-analysis on the expression of p53 marker in Odontogenic Keratocysts.

The odontogenic keratocyst (OKC) is a potentially aggressive odontogenic lesion and there is an ongoing debate regarding its biological behavior and classification. The present systematic review aims to assess the expression of the p53 protein in the odontogenic keratocyst in comparison to the dentigerous cyst and ameloblastoma. We searched MEDLINE, Web of Science and Scopus for immunohistochemical studies reporting OKC's, dentigerous cysts and solid/multicystic ameloblastomas. The Risk Difference between the lesions expressing the p53 was the effect measure and a P value < 0.05 was considered to provide evidence to the effect estimates. Results: The first hit retrieved 126 records. After duplicates removal, there were 84 articles, of which eighteen were assessed for eligibility. Thirteen articles were included in the meta-analysis, showing that OKC's have an estimated difference of 23% (P < 0.003) in the probability to express the p53 over dentigerous cysts, and an estimated difference of 4% (P = 0.28) in the probability to express the p53 over ameloblastomas. OKCs seem to behave more similarly to a tumor rather than an odontogenic cyst regarding its p53 expression and the classification of this lesion into Keratocystic Odontogenic Tumor should be carefully revaluated.

Expresión Inmunohistoquímica: Citoqueratinas CK19, CK14, β-Catenina y Ki67 en el Ameloblastoma y Queratoquiste Odontogénico / Immunohistochemical expression: Cytokeratins CK19, CK14, β-Catenin and Ki67 in Ameloblastoma and Odontogenic Keratocyst / Expressão imunohistoquímica: Citoqueratinas CK19, CK14, β-Catenina e Ki67 no ameloblastoma e queratocisto odontogênico

Resumen Introducción y objetivo: Los tumores y quistes odontogénicos son lesiones que se presentan en los maxilares al derivarse del tejido odontogénico embrionario. El ameloblastoma es un tumor benigno de origen epitelial, intraóseo y extraóseo caracterizado por su expansión e invasión local. Por otro lado, el queratoquiste odontogénico es una lesión quística, intraósea, con un comportamiento agresivo localmente destructivo y altamente recurrente. Se estudio la expresión de las proteínas CK19, CK14, β-Catenina, Ki-67 en las biopsias procesadas de amaloblastomas y queratoquistes odontogénicos durante el 2015-2018 del Servicio de Patología Oral y Maxilofacial de la facultad de Odontología de la Universidad Nacional de Colombia. Materiales y métodos: Estudio de serie de casos donde se tomaron bloques de parafina con diagnóstico histopatológico ya confirmado ameloblastoma (9 bloques): CK19 / 14, Ki67, β-Catenina y queratoquiste odontogénico (16 bloques): CK19 / 14, Ki67. Resultados: El promedio de Ki67 en el ameloblastoma y el queratoquiste odontogénico fue del 32% y 22%, respectivamente. Para el ameloblastoma y el queratoquiste odontogénico la CK19 / 14 fueron positivo para todos los casos. Finalmente, la β-Catenina marcó intensamente positiva en todos los casos de ameloblastoma. Conclusiones: Estas lesiones pueden diagnosticarse usando hematoxilina eosina, apoyándose en marcadores inmunohistoquímicos para corroborar el diagnóstico o cuando desee determinar metástasis en lesiones malignas. La CK14 / 19 son marcadores odontogénicos que determinan el origen de la lesión, la β-Catenina determina el comportamiento agresivo de la patología y el Ki67 determina el comportamiento, pronóstico y el tratamiento de la patología presente.

Abstract Introduction and objective: Odontogenic tumors and cysts are lesions that occur in the jaws when derived from embryonic odontogenic tissue. Ameloblastoma is a benign tumor of epithelial origin, intraosseous, characterized by its expansion and local invasion. On the other hand, odontogenic keratocyst is a cystic, intraosseous and extraosseous lesion, with aggressive local destructive behavior and highly recurrent. to find the expression of the CK19, CK14, β-Catenin, Ki-67 proteins in the processed biopsies of odontogenic amaloblastomas and keratocysts during the 2015-2018 Department of Oral and Maxillofacial Pathology of the Faculty of Dentistry of the National University of Colombia. Materials and methods: Case series study where paraffin blocks were taken with histopathological diagnosis already confirmed ameloblastoma (9 blocks): CK19 / 14, Ki67, β-Catenin and odontogenic keratocyst (16 blocks): CK19 / 14, Ki67. Results: The average Ki67 in ameloblastoma and odontogenic keratocyst was 32% and 22%, respectively. For the ameloblastoma and the odontogenic keratocyst, CK19 / 14 was positive for all cases. Finally, β-Catenin marked intensely positive in all cases of ameloblastoma. Conclusions: These lesions can be diagnosed using only hematoxylin eosin, relying on immunohistochemical markers to corroborate the diagnosis or when you want to determine metastases in malignant lesions. The CK14 / 19 are odontogenic markers that determine the origin of the lesion, β-Catenin determines the aggressive behavior of the pathology and the Ki67 determines the behavior, prognosis and treatment of the present pathology.

Resumo Introdução e objetivo: Tumores e cistos odontogênicos são lesões que ocorrem nas mandíbulas quando derivadas de tecido odontogênico embrionário. O ameloblastoma é um tumor benigno de origem epitelial, intraóssea, caracterizado por sua expansão e invasão local. Por outro lado, o ceratocisto odontogênico é uma lesão cística intraóssea, com comportamento destrutivo local agressivo e altamente recorrente. Objetivo: encontrar a expressão das proteínas CK19, CK14, β-Catenin, Ki-67 nas biópsias processadas de amaloblastomas odontogênicos e queratocistos durante o Departamento de Patologia Oral e Maxilofacial 2015-2018 da Faculdade de Odontologia da Universidade Nacional de Colombia. Materiais e métodos: Estudo de séries de casos em que foram realizados bloqueios de parafina com diagnóstico histopatológico de ameloblastoma já confirmado (9 blocos): CK19 / 14, Ki67, β-catenina e queratocisto odontogênico (16 blocos): CK19 / 14, Ki67. Resultados: O Ki67 médio no ameloblastoma e no ceratocisto odontogênico foi de 32% e 22%, respectivamente. Para o ameloblastoma e o ceratocisto odontogênico, a CK19 / 14 foi positiva para todos os casos. Finalmente, a β-catenina marcou intensamente positiva em todos os casos de ameloblastoma. Conclusões: Essas lesões podem ser diagnosticadas usando apenas hematoxilina eosina, utilizando marcadores imunohistoquímicos para corroborar o diagnóstico ou quando você deseja determinar metástases em lesões malignas. Os CK14 / 19 são marcadores odontogênicos que determinam a origem da lesão, a β-catenina determina o comportamento agressivo da patologia e o Ki67 determina o comportamento, o prognóstico e o tratamento da patologia atual.

A systematic review on the expression of bcl-2 in the nonsyndromic odontogenic keratocyst: should it be considered a cyst or a tumor?

The purpose of this study is to find out if the biological behavior and the capacity of the odontogenic keratocyst (OKC) in maintaining pathologic cells alive are more similar to the dentigerous cyst or to the ameloblastoma by assessing bcl-2. We searched MEDLINE, Web of Science, and Scopus for immunohistochemical studies reporting OKCs, dentigerous cysts, and ameloblastomas. Risk difference between the lesions expressing bcl-2 was the effect measure and a P value < 0.05 was considered to provide evidence to the effect estimates. OKCs have an estimated difference of 91% in the probability to express the bcl-2 over dentigerous cysts, but there is no difference in the expression of bcl-2 between OKCs and ameloblastomas. The present study demonstrated a great risk difference in the expression of bcl-2 between OKCs and dentigerous cysts and no risk difference between OKCs and ameloblastomas. OKC's growth may indirectly be attributed to the anti-apoptotic effect of bcl-2 in the cystic epithelium and not only to the increase of its intraluminal pressure. Therefore, the classification of this lesion into keratocystic odontogenic tumor should be carefully reconsidered.

Síndrome de Gorlin-Goltz. Diagnóstico molecular, nuevos tratamientos / Gorlin-Goltz syndrome. Molecular diagnosis, new treatments

Introducción: El síndrome de Gorlin-Goltz o síndrome de carcinoma de nevo basocelular es un desorden hereditario autosómico dominante que predispone principalmente a la proliferación de múltiples carcinomas basocelulares, queratoquistes odontogénicos y defectos del desarrollo, causados por la mutación del gen Patched localizado en el cromosoma 9. Presentación del caso: Se reporta un paciente con características de este síndrome, en la clínica de COMF de la UNAM. El diagnóstico fue basado en los estudios clínicos, imagenológicos y moleculares. Conclusiones: El conocimiento de esta enfermedad puede orientarnos a la sospecha diagnóstica de lesión quística o premaligna en forma oportuna, lo que permite prevenir complicaciones y brindar un tratamiento integral para así mejorar la calidad de vida de este tipo de pacientes (AU)

Introduction: Gorlin-Goltz syndrome or cell-based nevus carcinoma syndrome is an autosomal dominant inherited disorder that predisposes mainly to the proliferation of multiple basal cell carcinomas, maxillary keratocysts and developmental defects, caused by the mutation of the Patched gene located on chromosome 9. Case presentation: A patient with specific characteristics compatible with this syndrome was reported in the COMF Department of the UNAM. The diagnosis was based on clinical studies, radiology and genetic studies. Conclusions: Knowledge of this problem can guide us to the diagnostic suspicion in a timely manner, thus preventing complications, and to provide an improved integral treatment of the quality of life of this type of patients (AU)

A Brazilian multicentre study of 2,497 isolated cases of odontogenic keratocysts.

We present the frequency of cases of isolated odontogenic keratocysts submitted to microscopic examination at 10 Brazilian referral centres in Oral and Maxillofacial Pathology. In a retrospective (1953-2017) analysis, data on clinicoradiographic features and treatment of these lesions were collected and analysed descriptively. Among the 258,867 cases retrieved, 2,497 (0.96%) were isolated odontogenic keratocysts. In summary, an overview of individuals affected with isolated odontogenic keratocysts is reported herein. This lesion showed predilection for the posterior mandible of young adult men.

Queratoquiste Odontogénico en un Paciente con Displasia Cleidocraneal: reporte de un Caso / Odontogenic Keratocyst in a Patient with Cleidocranial Dysplasia: case Report

RESUMEN: La displasia cleidocraneal (DCC), es un trastorno autosómico dominante poco común, que involucra principalmente a los huesos que se osifican por vía membranosa; afectando el cierre de fontanelas craneales y el desarrollo de las clavículas, además de anomalías dentales y vertebrales. El objetivo de este manuscrito fue reportar el caso de una paciente con DCC que presentó un queratoquiste odontogénico (QQO) intrasinusal. Presentamos el caso de una paciente de 81 años, diagnosticada en su niñez con DDC, que consultó por un desajuste protésico y molestias en relación a la zona del seno maxilar derecho. Clínicamente se observó desajuste de la prótesis y aumento de volumen de márgenes poco definidos en la zona maxilar derecha, color rosa coral; que se extendía por todo el margen hemimaxilar derecho hasta el fondo de vestíbulo; doloroso a la palpación, con un mes de evolución. Se solicitó CBCT, con el que se pudo verificar la presencia de un desarrollo tumoral de contenido similar a dentículos, ubicado en la totalidad del seno maxilar derecho; extendiéndose hasta el piso de la cavidad nasal y orbitaria. Se estableció la hipótesis diagnóstica de "odontoma compuesto". Se le intervino quirúrgicamente, bajo anestesia general, realizándose una excisión de la lesión; la que era de márgenes definidos, con cambios de coloración en tonos oscuros, con la inclusión de tres piezas dentarias; de aspecto maligno. Se logró enucleación completa, dejando remanente óseo limpio. La pieza fue enviada a estudio histopatológico. En informe histopatológico, describió la presencia de una lesion quistica con pared compatible con queratoquiste.

ABSTRACT: Cleidocranial dysplasia (CCD) is an uncommon autosomal dominant disorder that mainly involves bones that ossify via the membrane, affecting the closure of cranial fontanels and the development of the clavicles, as well as presenting dental and vertebral anomalies. The aim of this manuscritpt was to report a case of a patient with CCD who presented an intrasinusal odontogenic keratocyst.We present an 81-year-old female patient, diagnosed with this syndrome in childhood, who comes to our service for a prosthetic misalignment and discomfort of the right maxillary sinus area. Clinically, there was a mismatch of the prosthesis and an increase in the volume of undefined margins under it, coral pink color, which extended all over the right hemimaxillary margin to the bottom of the vestibule, painful on palpation, with a one month evolution. A CBCT was requested, which revealed the presence of a tumor development with content similar to denticles, located in the entire right maxillary sinus, extending to the floor of the nasal and orbital cavity. The diagnostic hypothesis of "compound odontoma" was established. The patient was operated on in the central ward, under general anesthesia performing the excisional biopsy of the lesion, which showed changes in coloration in dark tones, with defined edges, with the inclusion of three teeth showing malignancy aspects. Complete enucleation was achieved, leaving tumor-free clean bone remnant. In a histopathological report, the presence of a keratocyst wall was described, which is not very compatible given the appearance of the lesion, the presence of the dental pieces included in it, and the behavior of the lesion.

Myofibroblasts and mast cells: influences on biological behavior of odontogenic lesions.

Odontogenic lesions differ in their rate of recurrence and aggressiveness. This study aimed to evaluate the presence of myofibroblasts and mast cells in odontogenic lesions. Sample consisted of 20 cases each of dentigerous cysts, odontogenic keratocysts, and solid ameloblastomas. Histologic sections were submitted to immunohistochemistry using anti-α-smooth muscle actin and anti-tryptase antibodies. Myofibroblasts and mast cells were counted at ×400 magnification in 5 and 10 fields, respectively. Myofibroblasts were more frequent in ameloblastomas (24.41), followed by odontogenic keratocysts (16.21) and dentigerous cysts (11.85; P=.002). Granulated and degranulated mast cells were more frequent in dentigerous cysts (7.88 and 8.96, respectively), followed by odontogenic keratocysts (6.53 and 7.08) and ameloblastomas (5.21 and 1.88). The difference was only significant for degranulated mast cells (P<.05). Analysis of the correlation between myofibroblasts and mast cells (granulated and degranulated) revealed a moderate positive correlation only in ameloblastomas (R=0.621, P=.003). Probably, myofibroblasts are related to the biological behavior of the odontogenic lesions studied, particularly their aggressiveness. On the other hand, mast cells seem to be associated with inflammatory processes, which are more frequent in cystic lesions than in benign neoplasms. In addition, mast cells may induce the differentiation of fibroblasts into myofibroblasts, thus increasing the number of the latter.

Absence of BRAFV600E mutation in odontogenic keratocysts.

BACKGROUND: Mutations in the patched 1 (PTCH1) gene are the main genetic alteration reported in sporadic and nevoid basal cell carcinoma-associated odontogenic keratocyst (OKC). Oncogenic mutations, including BRAFV600E, previously considered exclusive of malignant neoplasms have been reported in odontogenic tumors. Recently, a high frequency of BRAFV600E mutation has been reported in OKC. Because of the considerable recurrence rate of OKC, the identification of druggable genetic mutations can be relevant in the management of extensive lesions. METHODS: A set of 28 OKCs was included in this work. Initially, 10 sporadic and eight OKC samples from four NBCCS patients (a pair of lesions from each syndromic patient) were submitted to targeted next-generation sequencing (NGS) of 2800 different mutations in 50 oncogenes and tumor suppressor genes, including BRAF. Ten extra sporadic OKC samples were included to assess BRAFV600E mutation using TaqMan allele-specific qPCR. RESULTS: The following missense mutations occurred in one case each: ATM p.Ser333Phe, SMO p.Gly416Glu, PIK3CA p.Ser326Phe, FBXW7 p.Ser438Phe, JAK2 p.Ser605Phe, PTEN p.Arg173His, ATM p.Cys353Arg, PTEN p.Ser294Arg, MET p.His1112Tyr. None of the 18 samples showed the BRAFV600E (or any other V600) mutation in the NGS. BRAFV600E mutation was detected by qPCR in one of the 10 OKC. Collectively, our results show BRAFV600E mutation in 1 of 28 OKC cases. CONCLUSION: On the basis of our results, OKCs do not present recurrent hotspot mutations in these 50 genes commonly mutated in cancer. In addition, BRAFV600E does not play a central role in OKC pathogenesis.

Use of refrigerant spray of a propane/butane/isobutane gas mixture in the management of keratocystic odontogenic tumors: a preliminary study.

PURPOSE: Keratocystic odontogenic tumor (KCOT) is an aggressive benign tumor and the management by complete enucleation followed by cryotherapy maintains the inorganic bone matrix, resulting in better repair and reduces the rates of recurrence. A refrigerant spray with a propane/butane/isobutane gas mixture has been pointed to as an alternative to liquid nitrogen, because the device is easy to handle and contain within the cavity, providing better control and lower risk of injury to the adjacent soft tissue. Thus, the aim of this study was to evaluate the outcome of enucleation followed by cryosurgery using a refrigerant spray of this gas mixture in ten patients diagnosed with KCOT. METHOD: The biggest lesions received a prior treatment consisting of marsupialization to decrease the tumor size. During the surgeries, the lesions were removed by enucleation and the surgical site was sprayed with the gas mixture. RESULTS: Wound dehiscence was observed in all cases, which healed by the second intention. The mean follow-up period was 64.3 months (range 24-120 months). Eight of the ten patients showed no evidence of clinical or radiographic recurrence. Pathologic fractures and infections were not observed. CONCLUSIONS: The results obtained suggest that enucleation followed by cryosurgery is an effective therapy for managing KCOT.

Expresión inmunohistoquímica de ki-67 y p53 en tumor odontogénico queratoquístico en comparación con ameloblastoma multiquístico / Immunohistochemical expression of Ki-67 and p53 in odontogenic keratocysts in comparison with ameloblastomas

Introducción: Ha sido descrita la inmunodetección de p53 y Ki-67 en el tumor odontogénico queratoquístico (TOQ) y en ameloblastomas mutiquísticos (AM). Sin embargo, hay escasa y contradictoria evidencia respecto de la comparación de estos dos marcadores entre estas neoplasias. Su estudio podría contribuir a comprender las diferencias que presentan en su comportamiento clínico y ser un complemento discriminatorio al momento de definir tratamiento, pronóstico y recidiva. Objetivo: Comparar el recuento de inmunomarcación de p53 y Ki-67 en células epiteliales de TOQ y AM, presentes en el registro de biopsias del Instituto de Referencia de Patología Oral (IREPO), de la Facultad de Odontología de la Universidad de Chile entre los años 2000-2011. Metodología: Estudio observacional de corte transversal. Se estudiaron mediante inmunohistoquímica 8 casos de TOQ y 6 casos de AM con diagnóstico histopatológico según la clasificación de la OMS del 2005. Las muestras se encontraban en bloques de parafina, fijadas en formalina. Resultados: Los datos mostraron una distribución normal en cuanto al número de células positivas para ambos inmunomarcadores. No hubo diferencias estadísticamente significativas en la inmunomarcación de Ki-67 y p53 para TOQ y AM. Sin embargo, en ambas neoplasias, la inmunomarcación de p53 fue mayor respecto a Ki-67, con una diferencia estadísticamente significativa tanto en TOQ (p=0.0134) como en AM (p=0.0079). Conclusión: Los resultados sugieren que la inhibición de apoptosis predominó en ambos tumores por sobre la multiplicación celular. Estas diferencias podrían relacionarse con su potencial de crecimiento.

Introduction: p53 and Ki-67 immunodetection have been described in the keratocystic odontogenic tumor (TOQ) and multicystic ameloblastomas (AM). However, there is limited and contradictory evidence regarding the comparison of these two markers between these neoplasias. Their study could help to understand the differences that occur in their clinical behavior and be a complement when defining discriminatory treatment, prognosis and recurrence. Objective: To compare the immunomarking count of p53 and Ki-67 in epithelial cells in AM and TOQ present in the biopsies registered at the Oral Pathology Reference Institute (IREPO), Faculty of Dentistry, University of Chile from 2000 to 2011. Methods: Cross-sectional observational study. 8 cases of TOQ and 6 cases of AM with histopathological diagnosis according to the WHO classification of 2005 were studied using immunohistochemistry. The samples were formalin-fixed and paraffin-embedded. Results: The data showed a normal distribution in the number of positive cells for both immunomarkers. There were no statistically significant differences in the Immunohistochemical expression of Ki-67 and p53 of TOQ and AM. However in both tumors, the immunohistochemical expression of p53 was higher compared to Ki-67, with a statistically significant difference in TOQ (p=0.0134) and AM (p=0.0079). Conclusion: The results suggest that inhibition of apoptosis in both tumors predominated over cell multiplication. These differences may be related to their growth potential.

Non-syndromic multiple odontogenic keratocysts: report of case / Keratocistos múltiplos não-sindrômicos: relato de caso

Odontogenic keratocysts (OKCs) are epithelial developmental cysts which were first described byPhillipsen in 1956. Lesions are frequently multiple and a component of Nevoid Basal Cell CarcinomaSyndrome (NBCCS) (Gorlin Goltz syndrome/Bifid rib syndrome). We hereby report a case of multipleOKCs in a non – syndromic patient and highlight the general practitioner the importance of diagnosingthe disease and enforcing a strict long-term follow-up whenever such a case is identified.

Os keratocistos odontogênicos são cistos de desenvolvimento que foram descritos primeiramentepor Phillipsen, em 1956. As lesões são frequentemente múltiplas, sendo componentes da síndromedo carcinoma nevoide de células basais (síndrome de Gorlin, síndrome das costelas bífidas).Descreve-se um caso de keratocistos múltiplos em paciente não-sindrômico, enfatizando-se aimportância do clínico geral no diagnóstico da doença e reforçando a necessidade de umapreservação a longo prazo tão logo a doença seja diagnosticada.

Sindrome de goliin: a proposito de n caso / Gorlin’s Syndrome: A case

El llamado Síndrome de Gorlin es un raroy poco frecuente desorden clínico-patológico hereditario de carácter autosómico dominante bien reconocido y expresión variable. El Síndrome se caracteriza por múltiples nódulos cutáneos en piel no expuesta al sol y también en la piel de cara que tienden a volverse malignos con la edad (nevos de células basales), queratoquistes odontogénicos múltiples, hipertelorismo, amplia raíz nasal entre otras. 1, 2 Nos trazamos como objetivo, actualizar el tema, así como presentar un caso diagnosticado como síndrome de Gorlin, en el departamento de Cirugía Máxilofacial del Hospital General Calixto García.

The so called Gorlin’s Syndrome is a rare infrequent clinical pathological disorder with a well known and multi expression hereditary autosomic dominant characteristic pattern. The syndrome is characterized by multiple nodules in non exposed skin and facial skin which tend to become malignant with age (basal cell moles), multiple odontogenic keratocysts, and hypertelorism, a wide nose base among others. Our main purpose is to update the subject as well as to present the case diagnosed as a Gorlin’s Syndrome in the plastic surgery department of CalixtoGarciaHospital.