Importancia del diagnóstico precoz en el carcinoma mucoepidermoide. Relato de caso clínico / Importance of early diagnosis in mucoepidermoid carcinoma. Case report

El carcinoma mucoepidermoide es el tumor maligno más frecuente de las glándulas salivales menores localizándose por lo general en el paladar. El objetivo del presente reporte de caso es evidenciar en la literatura científica la importancia del diagnóstico precoz de carcinomas orales, así como, el rol fundamental que cumple el odontólogo general para identificar, guiar y derivar al paciente a un especialista. Se presenta el caso clínico de un paciente de sexo masculino que acudió a la Facultad de Odontología de la Universidad Nacional de Asunción, quien fue derivado por su odontóloga tras percibir un pequeño pero perceptible cambio en la coloración normal de la mucosa y molestias ocasionales en la zona del paladar duro. Al examen intraoral se observó en el paladar duro, a la altura de los premolares en la hemiarcada izquierda, una lesión nodular ovoidea, color violáceo, de 1cm de diámetro, depresible a la palpación. Se realizó una biopsia incisional para su estudio histopatológico, reportando un carcinoma mucoepidermoide. El diagnóstico precoz de este tipo de patologías es un desafío para el odontólogo general, quien debe orientar al paciente, ante cualquier cambio de la estructura normal de la cavidad bucal, para que acuda a un especialista.

Mucoepidermoid carcinoma is the most frequent malignant tumor of the minor salivary glands, usually located in the palate. The objective of this case report is to demonstrate in the Paraguayan scientific literature the importance of early diagnosis of oral carcinomas as well as the fundamental role of the general dentist in identifying, guiding and referring the patient to the corresponding specialist. The clinical case presented is about a male patient who attended the Faculty of Dentistry of the National University of Asuncion. His dentist referred him after noticing a small but perceptible change in the normal coloration of the mucosa and occasional discomfort in the area of the hard palate. In the intraoral examination, an ovoid nodular lesion, purplish in color, 1cm in diameter, depressible on palpation, observed on the hard palate at the level of the premolars in the left hemiarch. An incisional biopsy performed for histopathological study, reporting a mucoepidermoid carcinoma. Regarding the early diagnosis of this type of pathology, the challenge for the general dentist will continues to be his continuous training in order to be able to successfully guide the patient in seeking care from the right specialist in case of any change in the oral cavity.

Methylation analysis of Rb, P16, MGMT, and hMLH-1in mucoepidermoid carcinomas. A pilot study

Mucoepidermoid carcinoma (MC) is the most common malignant epithelial neoplasm in the salivary glands. This neoplasm has varying proportions of mucous, epidermoid, intermediate, columnar, and clear cells. MCs have been associated with CRTC1-MAML2 genes; however, their pathogenesis is uncertain. Recently, epigenetic changes have been considered a possible aetiologic factor. To identify the methylation state of RB, P16, MGMT, and hMLH genes in the three severity grades of MC were used five MCs and one healthy minor salivary gland as a control group (CG) obtained from the Pathology and Oral Medicine Laboratory and analyzed using MS-PCR to compare the presence or absence of methylation in promotor regions. The Kruskal- Wallis test was performed, with p≤0.05 considered significant. CG was employed as the normalizer of methylation levels. All assays were performed in triplicate. The mean age of our population was 52.6±18.6 years old; the total population was female and included 2 low grade, 2 intermediate grade, and 1 high grade levels of severity. When comparing the methylation status of the three histopathological grades of MC against the control, statistically significant differences were observed in Rb-M, MGMT-M, and hMLH-1-NM for high-grade severity, with p values of 0.03, 0.05, and 0.04, respectively. Methylation is a possible mechanism for pathogenesis processing of high-grade MC. However, a larger sample population is necessary to validate this finding.

El carcinoma mucoepidermoide (CM) es la neoplasia epitelial maligna más frecuente de glándulas salivales. Esta neoplasia tiene proporciones variables de células mucosas, epidermoides, intermedias, cilíndricas y claras. Los CM se han asociado con los genes CRTC1-MAML2; sin embargo, su patogenia es incierta. Recientemente, los cambios epigenéticos se han considerado un posible factor etiológico. Para identificar el estado de metilación de los genes RB, P16, MGMT y hMLH en los tres grados de severidad de CM se utilizaron cinco CM y una glándula salival menor sana como grupo control (GC) obtenidos del Laboratorio de Patología y Medicina Oral y analizados mediante MS-PCR para comparar la presencia o ausencia de metilación en regiones promotoras. Se realizó la prueba de Kruskal-Wallis, considerándose significativa una p≤0,05. Se empleó GC como normalizador de los niveles de metilación. Todos los ensayos se realizaron por triplicado. La edad media de nuestra población fue de 52,6 ± 18,6 años; la población total era femenina e incluía 2 niveles de severidad de grado bajo, 2 de grado intermedio y 1 de alto grado. Al comparar el estado de metilación de los tres grados histopatológicos de CM contra el GC, se observaron diferencias estadísticamente significativas en Rb-M, MGMT-M y hMLH-1-NM para severidad de alto grado, con valores de p de 0.03, 0.05, y 0,04, respectivamente. La metilación es un posible mecanismo para el procesamiento de patogénesis de CM de alto grado. Sin embargo, se necesita una población de muestra más grande para validar este hallazgo.

Compromiso tumoral de vía aérea en pediatría / Pediatric airway compromise by tumors

Los tumores traqueo bronquiales son extremadamente infrecuentes en la edad pediátrica e incluyen lesiones benignas y malignas. Por la baja frecuencia en niños y sintomatología respiratoria inespecífica, la sospecha diagnostica es habitualmente tardía. El tratamiento de elección en la mayoría de ellos es la resección quirúrgica abierta, sin embargo, la remoción endoscopia podría estar indicada es casos muy seleccionados con histología benigna y de localización accesible.

Tracheobronchial tumors are extremely rare in children and include benign and malignant lesions. Due to the low frequency in children and nonspecific respiratory symptoms, diagnostic suspicion is usually late. The treatment of choice in most of them is open surgical resection, however, endoscopy removal could be indicated in highly selected cases with benign histology and accessible location.

Desafío diagnóstico y terapéutico de carcinoma mucoepidermoide palatino: reporte de un caso

Resumen Las neoplasias en glándulas salivales son infrecuentes, representando menos del 3% de los tumores de cabeza y cuello. El carcinoma mucoepidermoide es el tumor maligno más común en glándulas salivales, siendo su principal ubicación la parótida. Clínicamente se asemeja a otras lesiones de mucosa oral, por lo cual, es importante realizar un correcto diagnóstico diferencial. Su comportamiento biológico se relaciona con el grado histológico tumoral, factor relevante en el pronóstico y tratamiento de esta neoplasia. Presentamos el caso de un paciente hombre de 75 años afectado con un tumor en paladar con diagnóstico de carcinoma mucoepidermoide de bajo grado. Como tratamiento se realizó una maxilectomía parcial y una placa obturadora en base a una prótesis removible y posterior reconstrucción con un colgajo libre microvascularizado. Actualmente el paciente se encuentra en controles periódicos, libre de enfermedad. Los tumores de glándulas salivales son un desafío diagnóstico, requieren de exámenes imagenológicos y del estudio histopatológico. Cuando existen dudas en el diagnóstico, se debe considerar repetir la toma de la muestra o la obtención de biopsias de más de una zona representativa que permita el diagnóstico de la lesión.

Abstract Salivary gland neoplasms are infrequent lesions representing less than 3% of head and neck tumors. Mucoepidermoid carcinoma is the most common malignant tumor in salivary glands, being the parotid the most usual location. Clinically, it resembles other oral mucosa lesions, therefore, it is important to make a correct differential diagnosis. Its biological behavior is related to the tumor histological grade, a relevant factor in the prognosis and treatment of this neoplasm. We reported a case of a 75-year-old-man, with a tumor in the palate, diagnosed as low-grade mucoepidermoid carcinoma. A partial maxillectomy and an obturator plate were performed based on a removable prosthesis and subsequent reconstruction with a microvascularized free flap. The patient is currently undergoing regular checkups, maintaining disease free. Salivary gland tumors are a diagnostic challenge, requiring imaging tests and histopathological study. In case of doubts with the diagnosis, it should be considered to biopsy more than area or to repeat the biopsy in order to obtain a representative sample that allows the diagnosis of the lesion.

Carcinoma mucoepidermoide intraóseo en mandíbula: Reporte de caso y revisión de la literatura / Intraosseous mucoepidermoid carcinoma in mandible: Case report and literature review Intrao

Resumen Introdución: El carcinoma mucoepidermoide intraóseo es una neoplasia maligna muy rara de las glándulas salivales, posee la capacidad de desarrollarse en cualquier parte de la boca, siendo la zona de molares- premolares y ángulo mandibular los sitios más frecuentes de su hallazgo. Objetivo: Reportar el caso de un carcinoma mucoepidermoide intraóseo de bajo grado en mandíbula, de una paciente adulta mayor que fue diagnosticada en un Servicio de Cirugía Oral y Maxilofacial después de ser referida por un odontólogo particular general. Métodos: Se le realizo un examen clínico e indico la toma de radiografía panorámica, tomografía y biopsia incisional. Resultados: La biopsia dio como resultado carcinoma mucoepidermoide intraóseo y posteriormente fue transferida al Servicio de Oncología. Conclusión: El carcinoma mucoepidermoide intraóseo es una patología infrecuente, de pronóstico favorable cuando es detectado a tiempo y posee características similares a otras patologías no tan agresivas.

Abstract Introduction: Intraosseous mucoepidermoid carcinoma is a very rare malignant neoplasm of the salivary glands, it has the ability to develop in any part of the mouth, being the molar-premolar area and mandibular angle the most frequent sites of its finding. Objective: To report the case of a low grade intraosseous mucoepidermoid carcinoma in the jaw, in an older adult patient who was diagnosed in an Oral and Maxillofacial Surgery Service after being referred by a general private dentist. Methods: A clinical examination was carried out and indicated the taking of panoramic radiography, tomography and incisional biopsy. Results: The biopsy resulted in intraosseous mucoepidermoid carcinoma and was later transferred to the Oncology Service. Conclusion: Intraosseous mucoepidermoid carcinoma is an infrequent pathology, with a favorable prognosis when it is detected early and has similar characteristics to other not so aggressive pathologies.

Caracterización de pacientes diagnosticados de carcinoma epidermoide de canal anal. Cienfuegos, 2017-2019 / Characterization of patients diagnosed with squamous cell carcinoma of the anal canal. Cienfuegos, 2017-2019

RESUMEN Fundamento: el carcinoma epidermoide de canal anal constituye un problema sanitario de gran magnitud, debido a su elevada morbimortalidad. El riesgo de padecer esta enfermedad difiere entre los individuos según sus modos y estilos de vida. Objetivo: caracterizar a los pacientes diagnosticados con carcinoma epidermoide de canal anal en la provincia de Cienfuegos en el período del primero de enero de 2017 al treinta y uno de diciembre de 2019. Métodos: estudio descriptivo y prospectivo sobre pacientes diagnosticados con carcinoma epidermoide de canal anal. Los datos se obtuvieron de las historias clínicas y las actas del comité de fallecidos. Se analizaron las variables: edad, sexo, color de la piel, enfermedades asociadas, etapas clínicas, forma de presentación, clasificación de la cirugía, tipo de tratamiento, técnica quirúrgica empleada, tratamiento oncológico, entre otras. Los datos obtenidos fueron procesados mediante el programa estadístico SPSS versión 21, los resultados se presentaron en tablas mediante números absolutos y porcentaje. Resultados: predominaron pacientes en edades entre 50 y 70 años, femeninas; la rectorragia y el dolor anal fueron los síntomas fundamentales, acompañados o no de cambios en el hábito intestinal, pujo y tenesmo; el virus del papiloma humano fue el antecedente patológico personal más observado; la colostomía fue la técnica quirúrgica más empleada. Conclusiones: el carcinoma epidermoide de canal anal constituye un problema de salud, asociado a vida sexual desprotegida y enfermedades de transmisión sexual. El diagnóstico temprano permite un mejor estudio y estadiaje y por consiguiente el uso de técnicas quirúrgicas más conservadoras.

ABSTRACT Background: squamous cell carcinoma of the anal canal constitutes a health problem of great magnitude, due to its high morbidity and mortality. The risk of suffering from this disease differs between individuals according to their modes and lifestyles. Objective: to characterize the patients diagnosed with squamous cell carcinoma of the anal canal in the Cienfuegos province from January the 1st, 2017 to December 31st, 2019. Methods: descriptive and prospective study on patients diagnosed with squamous cell carcinoma of the anal canal. The data were obtained from the medical records and the minutes of the deceased committee. The variables were analyzed: age, sex, skin color, associated diseases, clinical stages, form of presentation, classification of surgery, type of treatment, surgical technique used, cancer treatment, among others. The data obtained were processed using the SPSS version 21 statistical program; the results were showed in tables using absolute numbers and percentage. Results: female patients between 50 and 70 years old predominated; rectal bleeding and anal pain were the fundamental symptoms, accompanied or not by changes in bowel habit, straining and tenesmus; human papillomavirus was the most observed personal pathological history; the colostomy was the most widely used surgical technique. Conclusions: squamous cell carcinoma of the anal canal constitutes a health problem, associated with unprotected sexual life and sexually transmitted diseases; early diagnosis allows a better study and staging and therefore the use of more conservative surgical techniques.

Avaliação da expressão gênica associada à via de sinalização TGFß em tumores de glândulas salivares / Expression evaluation of genes associated with the TGFß signaling pathway in salivary gland tumors

As glândulas salivares são estruturas formadas por um sistema de ductos e ácinos responsáveis por secretar saliva. Apesar de raros, os tumores de glândulas salivares compreendem um grupo heterogêneo de lesões, apresentando diferentes características histológicas, sendo de difícil classificação e comportamento clínico diverso. A identificação de novos marcadores moleculares tem sido alvo de pesquisas para melhor compreensão e classificação dessas neoplasias, visto que a avaliação da expressão gênica e suas vias envolvidas permite identificar genes associados à regulação que modula o desenvolvimento neoplásico. Assim, novos achados podem direcionar a aplicação de novas técnicas para o diagnóstico, prognóstico e tratamento terapêutico. Contudo, pouco se sabe sobre a via de sinalização TGFß em neoplasias mais comuns em glândulas salivares, como: Adenoma Pleomórfico (AP), Carcinoma Mucoepidermoide (CME) e Carcinoma Adenoide Cístico (CAC). Diante disso, torna-se necessário ampliar a pesquisa de genes associados para a determinação de um painel de marcadores e, deste modo, fornecer informações que possam contribuir com o diagnóstico dessas neoplasias. O objetivo desse trabalho foi avaliar a expressão gênica relacionada à via de sinalização TGFß por meio da técnica de RT-PCR em tempo real (qPCR) destacando os marcadores TGFß1, ITGB6, SMAD2, SMAD4, FBN1, LTBP1 e c-MYC. Para tanto, foram selecionadas 13 amostras de AP, 17 de CME e 13 de CAC, além de 10 amostras de glândulas salivares não neoplásicas provenientes de cirurgias realizadas no A.C.Camargo Cancer Center no período do ano 2000 a 2015 e fornecidas pelo Biobanco de Tumores. Os resultados indicam que em pacientes com AP há aumento da expressão dos genes TGFß1, LTPB1, c-MYC e FBN1, enquanto a expressão de SMAD2 diminui quando comparados às amostras não neoplásicas. Em pacientes com CME, foi observada expressão aumentada dos genes TGFß1, ITGB6, FBN1 e c-MYC enquanto a expressão dos genes SMAD2 e SMAD4 diminui ao serem comparados às amostras não neoplásicas. Nos pacientes com CAC, foi observada expressão aumentada em quase todos os genes avaliados. Na análise de clusterização hierárquica não foi possível classificar nas diferentes neoplasias de glândula salivar. Para a validação dos resultados de expressão gênica foi realizada uma meta-análise utilizando dados da literatura, sendo possível observar concordância nos valores de expressão dos genes ITGB6, LTBP1 e TGFß1 em amostras de CME e dos genes FBN1, ITGB6, LTBP1, c-MYC, SMAD2 e SMAD4 nas amostras de CAC. Comparando-se a expressão dos genes entre os três tipos de neoplasias estudados, foi observado aumento de expressão dos genes c-MYC, SMAD2 e SMAD4 nos casos de CAC e aumento da expressão do gene ITGB6 nos casos de CME. A análise de sobrevida demonstrou que, em pacientes com Carcinoma Mucoepidermoide foi observado que a ausência de linfonodo comprometido e ausência de recidiva estão associadas a melhor probabilidade de sobrevida global em 5 anos. Nossos resultados sugerem que a expressão diminuída dos genes SMAD2 e SMAD4 parece não interferir na regulação transcricional de c-MYC, especialmente no AP e CME. Considerando os genes ITGB6, TGFß1, LTBP1, FBN1 e c-MYC a expressão aumentada parece ser relevante para a regulação da via de sinalização no processo de tumorigênese. Sendo assim, este estudo contribui para um melhor entendimento da via de sinalização TGFß em neoplasias de glândulas salivares, além de fornecer informações para o desenvolvimento de potenciais marcadores biológicos para essas neoplasias.

Salivary glands are structures formed by a system of ducts and acini responsible for secreting saliva. Although rare, salivary gland tumors comprise a heterogeneous group of lesions, presenting different histological features, difficult classification, and diverse clinical behavior. Identification of new molecular markers has been the subject of researchers for better comprehension and classification of these tumors, since gene expression evaluation and their signaling pathways allow the identification of genes associated with regulation that modulated tumor development. Therefore, new findings can direct the application of new technologies for diagnosis, prognosis, and therapeutic treatment. However, little is known about the TGFß signaling pathway in the most common salivary gland tumors, such as: Pleomorphic adenoma (PA), mucoepidermoid carcinoma (MEC), and adenoid cystic carcinoma (ACC). In addition, it is necessary to expand research of genes and associated genes for determining a panel of markers and, thus, provide information that could be contribute with the diagnostic of these neoplasms. The aim of this study is to evaluate the expression of genes associated with the TGFß signaling pathway by real-time RT-PCR (qPCR) highlighting the markers TGFß1, ITGB6, SMAD2, SMAD4, FBN1, LTBP1, and c-MYC. For this purpose, 13 PA samples, 17 MEC samples, 13 ACC samples, and histologically normal salivary glands samples were selected from surgeries performed at A.C.Camargo Cancer Center between 2000 and 2015. These samples were provided by Tumor Biobank. The results indicate that PA patients presented an increased TGFß1, LTPB1, c-MYC, and FBN1 gene expression whereas SMAD2 expression was decreased when compared to the normal samples. In MEC patients, increased expression of TGFß1, ITGB6, FBN1, and c-MYC genes was observed whereas SMAD2 and SMAD4 genes presented decreased expression. In ACC patients, increased expression in almost all genes was observed. In hierarchical clustering analysis it was not possible to classify the different salivary gland tumors. For the validation of the gene expression results it was carried out a meta-analysis using the literature date, being possible to observe an agreement in the expression values of the genes ITGB6, LTBP1 and TGFß1 in MEC samples and FBN1, ITGB6, LTBP1, c-MYC, SMAD2 and SMAD4 in ACC samples. Comparing gene expression among the three tumor types studied it was observed higher expression of c-MYC, SMAD2 and SMAD4 genes in ACC cases and higher expression of ITGB6 in MEC cases. Survival analysis demonstrated that, in MEC patients it was observed that absence of affected lymph nodes and absence of recurrence are associated with better overall survival in 5 years. Our results suggest that the decreased expression of SMAD2 and SMAD4 genes seems not to interfere with the transcriptional regulation of c-MYC, especially in PA and MEC. Considering ITGB6, TGFß1, LTBP1, FBN1 and c-MYC increased gene expression appears to be relevant for the regulation of the signaling pathway in tumorigenic process. Thus, this study contributes to a better understanding of TGFß signaling pathway in salivary gland tumors, apart from supplying information in development of potential biomarkers for these tumors.

Reconstrucción de cavidad oral con colgajo de músculo temporal: caso clínico / Reconstruction of oral cavity with temporal muscle flap: a clinical case

Objetivo: Describir la técnica de reconstrucción oral con colgajo de músculo temporal por resección de lesión oncológica. Caso clínico: Un paciente de sexo masculino, de 70 años de edad, fue derivado al Hospital Sirio Libanés desde la Facultad de Odontología de la Universidad de Buenos Aires con diagnóstico de carcinoma mucoepidermoide de siete meses de evolución, ubicado en el reborde alveolar del maxilar superior izquierdo, a nivel de las piezas dentarias 25 a 28. Se realizó la resección del tumor bajo anestesia general y la consiguiente reconstrucción del lecho mediante la técnica de reconstrucción oral con colgajo de músculo temporal. Conclusión: El colgajo temporomiofascial resultó ser versátil para la reconstrucción maxilofacial en lo que respecta a la proximidad, el tamaño, la fiabilidad de la vascularización y la facilidad de la técnica (AU)

Aim: To describe the temporalis myofacial flap technique for reconstruction in the maxillofacial region following oral cancer resection. Clinical case: A 70-year-old male patient was referred to the Sirio Libanés Hospital from the Dental School, University of Buenos Aires with a diagnosis of mucoepidermoid carcinoma with 7 months of evolution located in the alveolar ridge of the left upper jaw, at the level of teeth 25 to 28. The tumor was resected under general anesthesia and a rotational flap of the temporalis muscle was used for the reconstruction of the area. Conclusion: The temporalis flap was a versatile option for the reconstruction of maxillofacial defects due to its proximity to the oral cavity, the reliable vascularity and minor donor site morbidity (AU)

Mucoepidermoid carcinoma of the retromolar region: case report / Carcinoma mucoepidermoide em região retromolar: relato de caso

ABSTRACT The present study aimed to report a case of mucoepidermoid carcinoma (MEC), focusing on its clinical-pathological characteristics. At intraoral clinical examination, a nodular lesion was observed in the right pterygomandibular raphe region, with three years of evolution. An incisional biopsy was performed, and the diagnostic hypotheses of salivary gland injury and sialadenitis were considered. Histopathologically, a malignant neoplastic process characterized by the proliferation of epidermoid, intermediate and mucosal cells was observed. The histopathological diagnosis of MEC was emitted. The present case praises the importance of early diagnosis and correct management of this disease, providing a better prognosis for these patients.

RESUMEN Reportamos un caso de carcinoma mucoepidermoide (CME) con enfoque en sus rasgos clinicopatológicos. En la exploración clínica intraoral, se observó una lesión de aspecto nodular en región del rafe pterigomandibular derecho, con tiempo de evolución de tres años. Se realizó una biopsia por incisión, considerándose las hipótesis diagnósticas de lesión de glándula salival y sialadenitis. Histopatológicamente, se observó un proceso neoplásico maligno caracterizado por la proliferación de células epidermoides, intermedias y mucosas. El diagnóstico histopatológico fue de CME. El presente caso destaca la importancia del diagnóstico temprano y del manejo correcto de esa enfermedad, ofreciendo un mejor pronóstico para los pacientes portadores de CME.

RESUMO Relatamos um caso de carcinoma mucoepidermoide (CME) com enfoque em suas características clinicopatológicas. No exame clínico intraoral, observou-se lesão de aspecto nodular em região de rafe pterigomandibular direita, com tempo de evolução de três anos. Biópsia incisional foi realizada, e as hipóteses diagnósticas de lesão de glândula salivar e sialadenite foram consideradas. Histopatologicamente, observou-se um processo neoplásico maligno caracterizado pela proliferação de células epidermoides, intermediárias e mucosas. O diagnóstico histopatológico de CME foi emitido. O presente caso enaltece a importância do diagnóstico precoce e do correto manejo dessa patologia, proporcionado um melhor prognóstico para os pacientes portadores de CME.

Intraosseous maxillary mucoepidermoid carcinoma: a rare case report / Carcinoma mucoepidermoide intraósseo maxilar: um raro relato de caso

ABSTRACT An unusual case of intraosseous mucoepidermoid carcinoma is reported in a 22-year-old female, located in the posterior maxilla region. The article summarizes the main characteristics of the disease, including clinical-pathologic characteristics, treatment and prognosis.

RESUMEN Reportamos un caso raro de carcinoma epidermoide intraóseo ubicado en región posterior de la maxila en una paciente de 22 años de edad. El artículo resume las principales características del proceso neoplásico, incluyendo las características clínico-patológicas, el tratamiento y el pronóstico.

RESUMO Relatamos um raro caso de carcinoma mucoepidermoide intraósseo localizado em região posterior da maxila em uma paciente de 22 anos de idade. O artigo resume as principais características do processo neoplásico, incluindo as características clinicopatológicas, o tratamento e o prognóstico.

Overexpression of CDC7 in malignant salivary gland tumors correlates with tumor differentiation / Superexpressão de CDC7 em tumores malignos de glândulas salivares correlaciona-se com a diferenciação dos tumores

Abstract Introduction: Cell division cycle-7 protein is a serine/threonine kinase that has a basic role in cell cycle regulation and is a potential prognostic or therapeutic target in some human cancers. Objectives: This study investigated the expression of cell division cycle-7 protein in benign and malignant salivary gland tumors and also its correlation with clinicopathologic factors. Methods: Immunohistochemical expression of cell division cycle-7 was evaluated in 46 cases, including 15 adenoid cystic carcinoma, 12 mucoepidermoid carcinoma, 14 pleomorphic adenoma, and 5 normal salivary glands. Cell division cycle-7 expression rate and intensity were compared statistically. Results: The protein was expressed in almost all tumors. The intensity and mean of cell division cycle-7 expression were higher in malignant tumors in comparison with pleomorphic adenomas (p = 0.000). The protein expression was correlated with tumor grades (p = 0.000). Conclusions: The present study demonstrated cell division cycle-7 overexpression in malignant salivary gland tumors in comparison with pleomorphic adenomas, and also a correlation with tumor differentiation. Therefore, this protein might be a potential prognostic and therapeutic target for salivary gland tumors.

Resumo Introdução: A cell division cycle-7 é uma serina/treonina quinase que tem um papel básico na regulação do ciclo celular e é um potencial marcador prognóstico ou terapêutico em alguns tipos de câncer humano. Objetivos: Este estudo investigou a expressão de cell division cycle-7 em tumores de glândulas salivares benignos e malignos e também sua correlação com fatores clínico-patológicos. Método: A expressão imuno-histoquímica de cell division cycle-7 foi avaliada em 46 casos, incluindo 15 carcinomas adenoide císticos, 12 carcinomas mucoepidermoides, 14 adenomas pleomórficos e 5 glândulas salivares normais. A taxa de expressão e a intensidade da proteína cell division cycle-7 foram comparadas estatisticamente. Resultados: A proteína foi expressa em quase todos os tumores. A intensidade e a média da expressão de cell division cycle-7 foram maiores em tumores malignos em comparação com adenoma pleomórfico (p = 0,000). A expressão da proteína foi correlacionada com os graus do tumor (p = 0,000). Conclusões: O presente estudo demonstrou a superexpressão de cell division cycle-7 em tumores malignos de glândulas salivares quando comparada com o adenoma pleomórfico, além de uma correlação com a diferenciação de tumores. Portanto, essa proteína pode ser um potencial marcador prognóstico e terapêutico para tumores de glândulas salivares.

Overexpression of CDC7 in malignant salivary gland tumors correlates with tumor differentiation.

INTRODUCTION: Cell division cycle-7 protein is a serine/threonine kinase that has a basic role in cell cycle regulation and is a potential prognostic or therapeutic target in some human cancers. OBJECTIVES: This study investigated the expression of cell division cycle-7 protein in benign and malignant salivary gland tumors and also its correlation with clinicopathologic factors. METHODS: Immunohistochemical expression of cell division cycle-7 was evaluated in 46 cases, including 15 adenoid cystic carcinoma, 12 mucoepidermoid carcinoma, 14 pleomorphic adenoma, and 5 normal salivary glands. Cell division cycle-7 expression rate and intensity were compared statistically. RESULTS: The protein was expressed in almost all tumors. The intensity and mean of cell division cycle-7 expression were higher in malignant tumors in comparison with pleomorphic adenomas (p=0.000). The protein expression was correlated with tumor grades (p=0.000). CONCLUSIONS: The present study demonstrated cell division cycle-7 overexpression in malignant salivary gland tumors in comparison with pleomorphic adenomas, and also a correlation with tumor differentiation. Therefore, this protein might be a potential prognostic and therapeutic target for salivary gland tumors.

Carcinoma mucoepidermoide intraóseo de mandíbula: presentación de un caso clínico y revisión de la literatura / Intraosseous mucoepidermoid carcinoma of mandible: report of a clinical case and review of the literature

El carcinoma mucoepidermoide intraóseo (CMEI) es una neoplasia maligna de los maxilares muy poco frecuente. Es idéntica en casi todos sus aspectos al carcinoma mucoepidermoide de glándulas salivales pero, a diferencia de éste, asienta completamente en tejido óseo. Presentamos un caso clínico de CMEI en una paciente de 66 años de edad. Se describen las características clínicas, radiográficas e histopatológicas, tratamiento y evolución. Además se llevó a cabo una revisión de la literatura en PubMed acerca de CMEI de mandíbula publicada en los últimos diez años (AU)

Intraosseous mucoepidermoid carcinoma (IMCC) is an infrequent malignant tumor of the jaws that is identical in most respects to the mucoepidermoid carcinoma of salivary glands but arises entirely within the bone. We report a case of IMEC in a 66-year old woman. We describe the clinical, radiographic and histopathologic aspects as well as the treatment and follow-up. Additionally, we performed a literature review on PubMed regarding IMEC of the mandible published in last ten years (AU)

Carcinomatosis peritoneal secundaria a carcinoma mucoepidermoide de glándula salival / Peritoneal carcinomatosis secondary to mucoepidermoid carcinoma of the salivary gland

Resumen: Introducción: Los tumores de glándulas salivales son neoplasias poco frecuentes y representan menos del 5% de todos los tumores de cabeza y cuello. El carcinoma mucoepidermoide representa un 10-15% de todas las neoplasias de las glándulas salivales y aproximadamente un 30% de los tumores malignos salivales. El comportamiento biológico y las manifestaciones clínicas de este tipo de tumores son variables y se correlacionan con el estadio y grado histológico, siendo la presencia de metástasis a distancia un hallazgo inhabitual (en especial, en tumores de grado bajo o intermedio). Caso clínico: Paciente de 65 anos de edad con antecedentes de tabaquismo, a quien se diag nostica carcinoma mucoepidermoide de grado intermedio de glándula submandibular izquierda tratado con cirugía más radioterapia adyuvante, y que presenta progresión metastásica hepática y carcinomatosis peritoneal a los 26 meses de seguimiento.

Abstract: Introduction: Tumors of salivary glands are uncommon and comprise of about 5% of all head and neck tumors. Although constituting less than 15% of all salivary gland tumors, mucoepidermoid carcinoma account for approximately 30% of all malignant salivary gland neoplasms. Commonly these tumours are metastatic to local lymph nodes and distant metastases are rare (especially, in low and intermediate grade tumors). Case report: We report a case of 65 years old man who developed peritoneal carcinomatosis secondary to metastatic dissemination of mucoepidermoid carcinoma of the major salivary glands, which is an uncommon occurrence with intermediate grade tumors.

Carcinoma mucoepidermoide bronquial en la infancia: Caso clínico / Bronchial mucoepidermoid carcinoma in childhood: Case report

Los tumores bronquiales son raros en los niños. La mayoría son malignos; el más común es el tumor carcinoide. Con menor frecuencia, se asienta en el árbol traqueobronquial el carcinoma mucoepidermoide. El carcinoma mucoepidermoide representa del 0,1% al 0,2% de los tumores malignos broncopulmonares. Se manifiesta con síntomas de obstrucción de la vía aérea y/o neumonías recurrentes. El diagnóstico precoz y el tratamiento adecuado son de gran importancia en el pronóstico. La broncoscopía con biopsia de la masa endobronquial es el método de elección para confirmar el diagnóstico. El tratamiento consiste en la resección quirúrgica de la lesión. Se presenta a una niña de 11 años con disnea de esfuerzo y tos debidas a un carcinoma mucoepidermoide bronquial de bajo grado de malignidad. Se describen las manifestaciones clínicas, los métodos diagnósticos y el tratamiento de esta rara neoplasia en pediatría.

Bronchial tumors are rare in children. Most of them are malignant, being the carcinoid tumor the most common. Less frequently the mucoepidermoid carcinoma is found in the tracheobronchial tree. Mucoepidermoid carcinoma accounts for 0.1 to 0.2% of all malignant bronchopulmonary tumors. It manifests with symptoms of airway obstruction and/or recurrent pneumonias. Early diagnosis and appropriate treatment are of great importance in the prognosis. Bronchoscopy with biopsy of the endobronchial mass is the method of choice to confirm the diagnosis. Treatment consists of surgical resection of the lesion. We present an 11-year-old girl with dyspnea on exertion and cough due to a low malignant mucoepidermoid carcinoma of the bronchi. We describe the clinical manifestations, the diagnostic methods and the treatment of this rare neoplasia in pediatrics.

Carcinoma Mucoepidermóide do Pulmão ­ estado da arte a propósito de um caso clínico / Mucoepidermoid carcinoma of the lung ­ state of art with regard to a case report

O Carcinoma Mucoepidermóide do Pulmão (CMP) é um tumor com baixo potencial de malignidade; acredita-se que é indolente, mas pouco se sabe sobre as suas características dada a sua baixa incidência (0,2% de todos os tumores pulmonares). Os autores apresentam um caso clínico de um Carcinoma Mucoepidermóide do Pulmão de alto grau, a que se associa, habitualmente, metastização à distância, recorrência do tumor e mau prognóstico. Destaca-se a exuberante metastização (pleural, pericárdica, ganglionar e cerebral), que contrasta com o curso da doença, relativamente indolente, volvidos 12 meses do diagnóstico. Dado não ter indicação cirúrgica, a doente foi tratada com radioterapia e quimioterapia, apesar de ainda não estar definido um tratamento standard para este tipo histológico. A raridade do diagnóstico e os escassos estudos da literatura condicionam a abordagem terapêutica sistémica destes doentes, constituindo um desafio para a comunidade médica. O possível papel da terapêutica dirigida, como os inibidores da tirosina cinase do receptor do fator de crescimento epidérmico (EGFR) ou a terapêutica contra o oncogene de fusão CRTC1-MAML2, tem sido investigado, em doentes com Carcinoma Mucoepidermóide do Pulmão de alto grau.

Mucoepidermoid Carcinoma of the Lung (MECL) is a tumor of low malignant potential; we believe it is indolent, but little is known about its clinical features because of the low incidence rate (incidence of 0.2% of all lung cancers). We present a clinical case of a high degree Mucoepidermoid carcinoma of the lung, which frequently have distant metastasis, tumor recurrence and a bad prognosis. It is highlighted in this case the exuberant metastases (pleural, pericardial, cerebral, lymph nodes), contrasting with the relatively indolent course of the disease since the diagnosis (10 months ago). Our patient was treated with radiotherapy and chemotherapy, although effective treatment measures for high-grade tumors have not been established. The rarity of the diagnosis and the rare studies in the literature affect the systemic approach to these patients, becoming a challenge for the scientific community. The role of targeted therapy directed against the epidermal growth factor receptor (EGFR) and the novel fusion oncogene CRTC1-MAML2, is being investigated in high-grade tumors.

Carcinoma mucoepidermoide intraóseo en mandíbula: Reporte de caso y revisión de la literatura

Introdución: El carcinoma mucoepidermoide intraóseo es una neoplasia maligna muy rara de las glándulas salivales, posee la capacidad de desarrollarse en cualquier parte de la boca, siendo la zona de molares- premolares y ángulo mandibular los sitios más frecuentes de su hallazgo. Objetivo: Reportar el caso de un carcinoma mucoepidermoide intraóseo de bajo grado en mandíbula, de una paciente adulta mayor que fue diagnosticada en un Servicio de Cirugía Oral y Maxilofacial después de ser referida por un odontólogo particular general. Métodos: Se le realizo un examen clínico e indico la toma de radiografía panorámica, tomografía y biopsia incisional. Resultados: La biopsia dio como resultado carcinoma mucoepidermoide intraóseo y posteriormente fue transferida al Servicio de Oncología. Conclusión: El carcinoma mucoepidermoide intraóseo es una patología infrecuente, de pronóstico favorable cuando es detectado a tiempo y posee características similares a otras patologías no tan agresivas.

Introduction: Intraosseous mucoepidermoid carcinoma is a very rare malignant neoplasm of the salivary glands, it has the ability to develop in any part of the mouth, being the molar-premolar area and mandibular angle the most frequent sites of its finding. Objective: To report the case of a low grade intraosseous mucoepidermoid carcinoma in the jaw, in an older adult patient who was diagnosed in an Oral and Maxillofacial Surgery Service after being referred by a general private dentist. Methods: A clinical examination was carried out and indicated the taking of panoramic radiography, tomography and incisional biopsy. Results: The biopsy resulted in intraosseous mucoepidermoid carcinoma and was later transferred to the Oncology Service. Conclusion: Intraosseous mucoepidermoid carcinoma is an infrequent pathology, with a favorable prognosis when it is detected early and has similar characteristics to other not so aggressive pathologies.

Apoptose nas glândulas salivares humanas: avaliação de marcadores no processo de tumorigênese da glândula utilizando como modelos adenoma pleomórfico e o carcinoma mucoepidermóide / Apoptosis in human salivary glands: evaluation of markers during the tumorigenesis process using pleomorphic adenoma and mucoepidermoid carcinoma as models

Os tumores de glândulas salivares compreendem um grupo heterogêneo de lesões, apresentando diferentes características histológicas e comportamento clínico diverso. Esses tumores são classificados em benignos e malignos e correspondem a cerca de 3% dos tumores de cabeça e pescoço. Apesar dos avanços alcançados pelas pesquisas, biomarcadores que auxiliem no diagnóstico das neoplasias das glândulas salivares e que expliquem a sua evolução e progressão ainda são motivos de intensa investigação. O mecanismo de apoptose desempenha um papel importante na formação do lúmen durante a organogênese de estruturas glandulares, incluindo o desenvolvimento das glândulas salivares. Neste estudo, foi investigada a expressão das proteínas: PAR-4, PHLDA1, FAS, FAS-L, SPARC, MUC1 e Ki-67, que estão associadas aos processos de apoptose e proliferação celular, em 40 casos de carcinoma mucoepidermoide e em 49 casos de adenoma pleomórfico utilizando a técnica de imunoistoquímica. A proteína PAR-4 foi expressa em 98,0% dos casos de adenoma pleomórfico e em 100,0% dos casos de carcinoma mucoepidermóide, apresentando positividade nuclear e/ou citoplasmática. A proteína MUC-1 foi expressa em 96,0% dos casos de adenoma pleomórfico e em 94,9% dos casos de carcinoma mucoepidermóide, apresentando um padrão citoplasmático de expressão. A proteína Ki-67 foi expressa em média em 5,7 (variando de 0 a 54,3 células) células por 10 campos de grande aumento e em média em 29,8 (variando de 0 a 146,9 células) células por 10 campos de grande aumento nos casos de carcinoma mucoepidermóide. A proteína SPARC foi expressa em 79,5% dos casos de adenoma pleomórfico e em 45,5% dos casos de carcinoma mucoepidermóide. Nos casos de carcinoma mucoepidermóide a positividade foi observada nas células de suporte do tumor. Nos casos de adenoma pleomórfico a positividade foi observada nas células mioepiteliais. A proteína PHLDA1 foi expressa em 100,0% dos casos de adenoma pleomórfico e em 80,6% dos casos de carcinoma mucoepidermóide. A proteína FAS foi expressa de maneira focal em 88,6% dos casos de adenoma pleomórfico e em 66,7% dos casos de carcinoma mucoepidermóide. A proteína FAS-L foi expressa em 10,4% dos casos de adenoma pleomórfico e em 12,8% dos casos de carcinoma mucoepidermóide. Comparando-se a expressão das proteínas nos diferentes tumores, foi observada uma maior expressão das proteínas SPARC, FAS e PHLDA1 nos casos de adenoma pleomórfico e maior expressão da proteína Ki-67 nos casos de carcinoma mucoepidermóide. A expressão da proteína SPARC está associada a tumores de baixo grau histológico e expressão da proteína Ki-67 está associada com a presença de metástase em linfonodo nas amostras de carcinoma mucoepidermóide. A expressão da proteína FAS e menor expressão da proteína Ki-67 estão associadas a melhores taxas de sobrevida global e câncer específica. Dessa forma, a expressão das proteínas SPARC, FAS e Ki-67 parece apresentar um papel prognóstico nos os tumores de glândula salivar.

Salivary gland tumors comprise a heterogeneous group of lesions, with different histological characteristics and different clinical behavior. These tumors are classified as benign and malignant and correspond to 3% of the head and neck tumors. Despite the advances in the research field, biomarkers that aid in the diagnosis of salivary gland tumors and that explain their evolution and progression are still matter of intensive research. Apoptosis plays an important role in organogenesis of glandular structures, including the development of salivary glands. In this study, the expression of PAR-4, PHLDA1, FAS, FAS-L, SPARC, MUC1 and Ki-67 proteins was investigated in 40 cases of mucoepidermoid carcinoma and 49 cases of pleomorphic adenoma by immunohistochemistry. PAR-4 protein was expressed in 98.0% of pleomorphic adenoma cases and 100.0% of mucoepidermoid carcinoma cases, presenting nuclear and/or cytoplasmic positivity. MUC1 protein was expressed in 96.0% of pleomorphic adenoma cases and 94.9% of mucoepidermoid carcinoma cases, presenting a cytoplasmic pattern. Ki-67 protein was expressed in 5.7 (range 0 to 54.3 cells) cells per 10 high power fields in pleomorphic adenoma cases and in 29.8 (range 0 to 146.9 cells) cells per 10 high power fields in mucoepidermoid carcinoma cases. SPARC protein was expressed in 79.5% of pleomorphic adenoma cases and in 45.5% of mucoepidermoid carcinoma cases. In pleomorphic adenoma cases the positivity was observed in myoepithelial cells whereas in mucoepidermoid carcinoma the positivity was observed in the surrounding stroma. PHLDA1 protein was expressed in 100.0% of pleomorphic adenoma cases and 80.6% of mucoepidermoid carcinoma cases. FAS protein was focally expressed in 88.6% of pleomorphic adenoma cases and in 66.7% of mucoepidermoid carcinoma cases. FAS-L protein was expressed 10.4% of pleomorphic adenoma cases and 12.8% of mucoepidermoid carcinoma cases. Comparing protein expression in both tumors it was observed an increased expression of FAS, SPARC and PHLDA1 proteins in pleomorphic adenoma cases and increased expression of Ki-67 protein in mucoepidermoid carcinoma cases. The expression of SPARC protein was associated with low grade tumors and expression of Ki-67 protein was associated with lymph node metastasis in mucoepidermoid carcinoma samples. The expression of FAS protein and decreased expression of Ki-67 protein were associated with better overall cancer-specific survival rates. Therefore, the expression of SPARC, FAS and Ki-67 proteins seems to play a prognostic role for salivary gland tumors.

Mucoepidermoid carcinoma of the salivary glands: a retrospective study of 51 cases and review of the literature / Carcinoma mucoepidermoide de glándulas salivales. estudio retrospectivo de 51 casos y revisión de la literatura

The aim of this study is to present the casuistic of mucoepidermoid carcinoma of salivary glands in patients diagnosed at “Dr. Eduardo Cáceres Graziani” National Institute for Neoplastic Diseases, Lima, Perú. From January 2002 to December 2012, 51 cases were diagnosed as mucoepidermoid carcinoma. The number of female patients was higher, with 28 cases (54.9%), and regarding age distribution, 33.3% of the patients were under 30 years old. Pain was one of the main symptoms, and 74.5% of the mucoepidermoid carcinomas were located in the parotid gland. It is concluded that epidemiology regarding age and gender of the 51 cases analyzed was in the same range as other studies, and that most cases were located in major salivary glands, in agreement with reports on other populations. Other characte ristics showed a homogeneous distribution.

El propósito de este estudio es presentar la casuística del carcinoma mucoepidermoide de glándulas salivales de pacientes diagnosticados en el Instituto Nacional de Enfermedades Neoplásicas “Dr. Eduardo Cáceres Graziani” Lima, Perú, desde el 2002 hasta el 2012. Realizamos un estudio retrospectivo en el cual fueron incluidos sujetos con diagnóstico primario de carcinoma mucoepidermoide en glándulas salivales. Entre enero de 2002 y diciembre de 2012, se registraron 51 casos. El número de pacientes de sexo femenino fue mayor, con 28 casos (54,9%) y con respecto a la distribución por edades, el 33,3% de los pacientes eran menores de 30 años de edad. El dolor fue uno de los síntomas principales. El 74,5% de los carcinomas mucoepidermoides se localizaron en la glándula parótida. De los hallazgos obtenidos se concluye principalmente que en lo que respecta a la distribución epidemiológica de edad y género de los 51 casos analizados estas variaron en el mismo rango de otros estudios. También se distingue que el mayor número de casos estuvieron localizados en glándulas salivales mayores, dato en concordancia con otras poblaciones reporta das. Las demás características presentaron una distribución homogénea.

Carcinoma mucoepidermoide de tiroides, reporte de un caso / Thyroid Mucoepidermoid Carcinoma, A Case Report

El carcinoma mucoepidermoide es un tumor que se presenta muy raramente en la glándula tiroides. Sólo existen 47 casos reportados en la literatura hasta la actualidad, con diferente evolución clínica y regímenes de tratamiento. Presentamos un paciente con metástasis a distancia a un año del diagnóstico, donde reportamos la utilización del PET-CT por primera vez en la evaluación de la respuesta al tratamiento quimioterápico, demostrando ser una herramienta útil en el seguimiento y evaluación de la respuesta al mismo y la remisión completa lograda con el esquema de quimioterapia utilizado.

Mucoepidermoid carcinoma is a tumor that occurs very rarely in the thyroid gland. There are only 47 cases reported in the literature until now, with different clinical course and treatment regimens. Here we present a patient with distant metastasis one year after the diagnosis, where we report the use of PET-CT for the first time in the evaluation of response to chemotherapy, proving to be a useful tool in monitoring and evaluating the response to it and achieved complete remission with the regimen used.