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BACKGROUND: Salivary gland tumors (SGTs) are rare and highly heterogeneous lesions, making diagnosis a challenging activity. In addition, the small number of studies and samples evaluated difficults the determination of prognosis and diagnosis. Despite the solid advances achieved by research, there is still an intense need to investigate biomarkers for diagnosis, prognosis and that explain the evolution and progression of SGTs. METHODS: We performed a comprehensive literature review of the molecular alterations focusing on the most frequent malignant SGTs: mucoepidermoid carcinoma and adenoid cystic carcinoma. RESULTS: Due to the importance of biomarkers in the tumorigenenic process, this review aimed to address the mechanisms involved and to describe molecular and biomarker pathways to better understand some aspects of the pathophysiology of salivary gland tumorigenesis. CONCLUSIONS: Molecular analysis is essential not only to improve the diagnosis and prognosis of the tumors but also to identify novel driver pathways in the precision medicine scenario.

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OBJECTIVE: Mucoepidermoid Carcinoma (MEC) is the most common malignant salivary gland neoplasm, representing 10 to 15% of all salivary neoplasms. A review of the literature was conducted in order to determine trends in presentation, diagnostic features, treatment, and outcomes. METHODS: A PubMed, Embase, and Scopus search was carried out. The search process was performed by 2 independent reviewing authors and inclusion criteria included systematic reviews, meta-analyses, case-controls studies, cohort studies, comparative studies, clinical trials, cross-sectional studies, descriptive studies, experimental studies, case reports, case series studies, and human studies evaluating MEC. RESULTS: Females were more affected (54.5%) and the average age was 48.8 years. The most common location of MEC was the parotid glands (56.8%) followed by hard palate (18%). The most frequent clinical presentation was mass (65.2%) followed by ulcer (29.4%) with pressure as their main symptom (64.4%). The most frequent histologic presentation was Low grade (46.7%) followed by Intermediate grade (27.3%) and the most used treatment was surgery (76.2%). The average follow up was 138.5months, and recurrence was reported at 8.5%. CONCLUSION: MEC showed a strong predilection for the parotid glands is frequently painful, most frequently presents as a mass and most commonly has a low-grade histologic presentation.

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BACKGROUND: Primary sinonasal mucoepidermoid carcinoma (SN-MEC) is a malignancy arising from seromucinous glands of the nasal cavity and paranasal sinuses. Given its rarity, few large-scale studies have been performed. In this study we describe the incidence and determinants of survival of patients with SN-MEC leveraging the National Cancer Database (NCDB). METHODS: This was a retrospective, population-based cohort study of patients diagnosed with SN-MEC between 2004 and 2012 within the NCDB. The main outcome measure was overall survival (OS). RESULTS: A total of 164 patients were identified. The cohort was composed of 47.6% males. Mean age at diagnosis was 59.7 years. The maxillary sinus was the most common primary site, accounting for 45.7% of cases. Eleven percent of patients presented with nodal disease, whereas 2.1% had distant metastases. Stage IV disease was seen in 30.4% of cases. A total of 79.8% of the patients underwent surgery, 61.0% received radiation therapy, and 15.1% had chemotherapy. OS at 1, 2, and 5 years was 83%, 77.0%, and 57%, respectively. On multivariate analysis, Medicaid insurance status (hazard ratio [HR], 7.29; 95% confidence interval [CI], 1.74-30.57), advanced tumor size (HR, 4.94; 95% CI, 1.19-20.5), and advanced nodal disease (N1: HR, 9.48; 95% CI, 1.66-54.23; N2B: HR, 19.3; 95% CI, 1.07-350.64) were associated with worse OS. CONCLUSION: Mucoepidermoid carcinoma is the most common salivary gland malignancy but a rare sinonasal malignancy, with 5-year survival for SN-MEC approximating 50%. A significant proportion of patients present with advanced disease. Both socioeconomic factors and tumor characteristics are associated with survival.

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Resumen El carcinoma mucoepidermoide bronquial es una neoplasia infrecuente, representando el 0,1 a 0,2% de los tumores malignos primarios del pulmón. En general tiene un buen pronóstico, sin embargo, existe un subtipo de alto grado de pronóstico más ominoso. En este artículo se presentan dos casos clínicos de carcinoma mucoepidermoide bronquial de bajo grado, enfocado en su diagnóstico y manejo quirúrgico.

ABSTRACT Bronchopulmonary mucoepidermoid carcinoma is an uncommon neoplasm, accounting for 0.1 to 0.2% of primary malignant tumors of the lung. In general it has a good prognosis, however there is a subtype of high grade of more ominous prognosis. In this paper we present two clinical cases of low grade pulmonary mucoepidermoid carcinoma, focused on their diagnosis and surgical management.

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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.

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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 arcinoma of the ronchi. We describe the clinical manifestations, the diagnostic methods and the treatment of this rare neoplasia in pediatrics.

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.

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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.

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Objetivos: Determinar la tasa de resección quirúrgica completa en tumores seleccionados del Espacio parafaríngeo tratados por vía transoral o transnasal o por ambas. Diseño: descriptivo, retrospectivo. Materiales y métodos: Se incluyeron en este estudio los pacientes que tuvieron neoplasias localizadas en el espacio parafaríngeo y que fueron tratados con cirugía por vía transoral o transnasal. El abordaje transoral consistió en realizar una incisión con cauterio en la zona de mayor protrusión del tumor (pilar amigdalino y paladar), disección de la mucosa y del tumor de los planos profundos, traccionándolo hacia la cavidad oral. Para disecar el límite superior (rinofaringe) y lateral se utilizaron endoscopios que fueron introducidos por la incisión y por la cavidad nasal. El abordaje transnasal consistió en realizar una incisión en la pared lateral de la rinofaringe y disecar el tumor del plano profundo traccionándolo hacia el cavum. Resultados: Fueron tratados 3 pacientes por vía transoral y uno por vía endonasal por padecer tumores del espacio parafaríngeo. Tres tumores se originaron en glándulas salivales menores localizadas una en el espacio. Preestiloideo (1/3), otra en el espacio masticador (1/3) y otra en el sector superior del espacio preestiloideo (1/3). La histología dio como resultado dos adenoma pleomorfo y un carcinoma mucoepidermoide de bajo grado (1/3). (Está bien así? Qué es 1/3?). Una neoplasia se originó en el lóbulo profundo de la parótida y se extendió al espacio preestiloideo, su histología fue carcinoma mucoepidermoide de grado moderado. Conclusiones: La tasa de resección completa en pacientes con tumores del espacio parafaríngeo seleccionados tratados por vía transoral y endonasal fue del 100%. (AU)

Objectives: To determine the rate of complete surgical resection in parapharyngeal space selected tumors treated with transoral and / or transnasal approach. Design: Descriptive, retrospective. Materials and methods: Patients who had tumors localized in parapharyngealspace and who were treated with transoral or transnasal surgery. were included in this study. The transoral approach consisted in performing an incision with cautery in the area of greates tumor protrusion (tonsillar pillar and palate), dissection of the mucosa and tumor of the deep planes, pulling it into the oral cavity. To dissect the upper limit (nasopharynx) and lateral we used endoscopes that were inserted by the incision and the nasal cavity. The transnasal approach consisted in making an incision in the side wall of the nasopharynx and dissect the tumor of the deep plane pulling it towards the cavum. Results: Three patients were treated with transoral and one byendonasalapproacheswho had parapharyngeal space tumors. Three tumors originated in minor salivary glands located in prestyloidspace (1/3), masticator space (1/3) and upper sector of pree-styloid space (1/3). Histology was in two pleomorphic adenoma, and another onelow degree mucoepidermoid carcinoma (1/3). A neoplasm was originated in the deep lobe of the parotid gland and was extended to the prestyloid space, was a moderate degree of mucoepidermoid carcinoma. Conclusions: Complete resection rate in patients with selected parapharyngeal space tumors, treated by transorally and endonasal approach was 100%. (AU)

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