Analysis of the efficacy of endoscopic transnasal surgery for sinonasal and skull base adenoid cystic carcinoma / 中华耳鼻咽喉头颈外科杂志

Objective: To evaluate the efficacy of endoscopic transnasal surgery for sinonasal and skull base adenoid cystic carcinoma (ACC), and to analyze the prognostic factors. Methods: Data of 82 patients (43 females and 39 males, at a median age of 49 years old) with sinonasal and skull base ACC who were admitted to XuanWu Hospital, Capital Medical University between June 2007 and June 2021 were analyzed retrospectively. The patients were staged according to American Joint Committee on Cancer (AJCC) 8th edition. The disease overall survival(OS) and disease-free survival(DFS) rates were calculated by Kaplan-Meier analysis. Cox regression model was used for multivariate prognostic analysis. Results: There were 4 patients with stage Ⅱ, 14 patients with stage Ⅲ, and 64 patients with stage Ⅳ. The treatment strategies included purely endoscopic surgery (n=42), endoscopic surgery plus radiotherapy (n=32) and endoscopic surgery plus radiochemotherapy (n=8). Followed up for 8 to 177 months, the 5-year OS and DFS rates was 63.0% and 51.6%, respectively. The 10-year OS and DFS rates was 51.2% and 31.8%, respectively. The multivariate Cox regression analysis showed that late T stage and internal carotid artery (ICA) involvement were the independent prognostic factors for survival in sinonasal and skull base ACC (all P<0.05). The OS of patients who received surgery or surgery plus radiotherapy was significantly higher than that of patients who received surgery plus radiochemotherapy (all P<0.05). Conclusions: Endoscopic transonasal surgery or combing with radiotherapy is an effective procedure for the treatment of sinonasal and skull base ACC. Late T stage and ICA involvement indicate poor prognosis.

The outcome of transnasal endoscopic total maxillectomy in the treatment of sinonasal adenoid cystic carcinoma / 临床耳鼻咽喉头颈外科杂志

Objective:To assess the prognosis of sinonasal adenoid cystic carcinoma with hard palatine invasion treated by transnasal endoscopic total maxillectomy. Methods:Clinical data of twenty-six patients with sinonasal adenoid cystic carcinoma invading hard palatine treated by transnasal endoscopic total maxillectomy between May 2014 and December 2020 was analyzed retrospectively. Survival rate, local recurrence and distant metastasis were analyzed using Kaplan-Meier method. Cox regression was used to investigate the prognosis factors. Masticatory function after maxillectomy has also been assessed using the questionnaire of patients' satisfaction about masticatory function. Results:Margins in 8 patients（30%） were positive. The median time of follow-up was 38 months（6 to 85 months）. Twenty-five patients recurred. Four patients died of distant metastasis. The 5-year overall survival rate and relapse-free survival rate was 79.5% and 89.1%, respectively. Independent predictors of outcome on multivariate analysis were positive margin（P=0.018）, recurrence（P=0.006） and distant metastasis（P=0.04）. Conclusion:Transnasal endoscopic total maxillectomy could be performed for the treatment of the sinonasal adenoid cystic carcinoma with hard palatine invasion. Positive margin, local recurrence and distant metastasis were important predictors for patients' prognosis.

Carcinoma adenoide quístico / Adenoid cystic carcinoma

Introducción: El carcinoma adenoide quístico es una neoplasia maligna poco común y a menudo agresiva, que puede surgir en una variedad de órganos portadores de glándulas. Objetivo: Caracterizar la presentación de una neoplasia maligna agresiva infrecuente, el carcinoma adenoide quístico. Caso clínico: Paciente de 56 años que acudió a cuerpo de guardia con lesión en cuero cabelludo correspondiente con cilindroma maligno. Se estudia posteriormente y se constataron además lesiones a nivel de mamas, colon y recidiva en el cuero cabelludo, que dieron como resultado carcinoma adenoide quístico. Conclusiones: Es infrecuente; realizar su diagnóstico conlleva un alto índice de sospecha y su diagnóstico es histopatológico. Su pronóstico depende de la localización primaria y tiende a ser muy agresivo cuando se localiza fuera de la mama(AU)

Introduction: Adenoid cystic carcinoma is an uncommon and often aggressive malignant neoplasm that can arise in a variety of gland-bearing organs. Objective: To characterize the presentation of an uncommon aggressive malignancy, adenoid cystic carcinoma. Clinical case: 56-year-old patient who presented to the on-call department with a lesion on the scalp corresponding to malignant cylindroma. She was subsequently studied and lesions were also found in the breast, colon and recurrence in the scalp, which resulted in adenoid cystic carcinoma. Conclusions: It is infrequent; making its diagnosis involves a high index of suspicion and its diagnosis is histopathological. Its prognosis depends on the primary location and it tends to be very aggressive when located outside the breast(AU)

Sonda de Foley como auxiliar na reconstrução de terço médio de face / Foley´s cateter as a coadjuvant on middle face reconstruction / Sonda de Foley como um auxilio para reconstrucciones de terzo medio de la cara

Objetivos: O carcinoma adenoide cístico é uma lesão rara e agressiva. O presente trabalho visa relatar o caso de uma ressecção de carcinoma adenoide cístico e manutenção do arcabouço ósseo realizado por meio de sonda de Foley insuflada com soro fisiológico. Relato do caso: Um paciente submetido a ressecação de um carcinoma adenoide cístico em maxilla com destruição de soalho de órbita foi relatado. O caso foi realizado no Hospital Josina Machel em Luanda, Angola. A região apresenta uma considerável escassez de materiais de fixação e outros mais, o que impossibilita reconstruções maxilofaciais com a excelência necessária. A realização de enxertos microvascularizados ou implantes customizados torna-se inviável devido aos custos e tecnologia dispendida para tal. Conclusão: A sonda de Foley mostra-se como uma alternativa viável nos casos de reconstrução de terço médio de face com envolvimento de seio maxilar e soalho de órbita em locais de mais difícil acesso... (AU)

Objectives: Adenoid cystic carcinoma is a rare and aggressive lesion. The present work aims to report the case of a resection of adenoid cystic carcinoma and maintenance of the bone framework performed using a Foley catheter insufflated with saline solution. Case report: A patient who underwent resection of an adenoid cystic carcinoma in the maxilla with destruction of the orbital floor was reported. The case was carried out at Hospital Josina Machel in Luanda, Angola. The region has a considerable shortage of fixation materials and others, which makes maxillofacial reconstructions with the necessary excellence impossible. The realization of microvascularized grafts or customized implants becomes unfeasible due to the costs and technology used for this purpose. Conclusion: The Foley catheter is a viable alternative in cases of reconstruction of the middle third of the face with involvement of the maxillary sinus and orbital floor in areas that are more difficult to access... (AU)

Objetivos: El carcinoma adenoide quístico es una lesión rara y agresiva. El presente trabajo tiene como objetivo reportar el caso de una resección de carcinoma quístico adenoide y mantenimiento de la estructura ósea realizada mediante sonda de Foley insuflada con suero fisiológico. Caso clínico: Se reporta un paciente que fue sometido a resección de un carcinoma adenoide quístico en el maxilar con destrucción del piso orbitario. El caso se llevó a cabo en el Hospital Josina Machel de Luanda, Angola. La región tiene una escasez considerable de materiales de fijación y otros, lo que imposibilita las reconstrucciones maxilofaciales con la excelencia necesaria. La realización de injertos microvascularizados o implantes personalizados se vuelve inviable por los costes y la tecnología utilizada para tal fin. Conclusión: La sonda de Foley es una alternativa viable en los casos de reconstrucción del tercio medio de la cara con afectación del seno maxilar y suelo orbitario en zonas de más difícil acceso... (AU)

Avaliação da expressão da proteína EZH2 em carcinoma adenoide cístico e adenoma pleomórfico / EZH2 immunoexpression in adenoid cystic carcinoma and pleomorphic adenoma

As neoplasias de glândulas salivares apresentam comportamentos diferenciados, que não seguem os padrões clássicos das neoplasias benignas e malignas. A raridade de algumas destas lesões dificulta ainda mais o entendimento dos mecanismos envolvidos na etiopatogenia. Marcadores moleculares como a proteína EZH2 têm sido utilizados na investigação de alterações epigenéticas em diferentes neoplasias, auxiliando na definição do diagnóstico e prognóstico das lesões. O objetivo do presente trabalho é avaliar a expressão da proteína EZH2 e descrever as características clínicas e microscópicas de amostras de carcinoma adenoide cístico (CAC) e adenoma pleomórfico (AP) com ênfase na importância da definição da malignidade da lesão. A análise dos cortes microscópicos corados em Hematoxilina e Eosina dos casos de Adenoma pleomórfico mostraram células epiteliais e mioepiteliais glandulares dispostas em lençóis e estruturas ductiformes em meio a estroma variável. Os casos de Carcinoma adenoide cístico mostraram três padrões distintos de crescimento incluindo formações tubulares, cribriformes e sólidas. Todos os casos de AP e CAC foram positivos para reação imuno-histoquímica para EZH2. As amostras de CAC apresentaram expressão de EZH2 significativamente maior comparado ao AP. As covariáveis metástase em linfonodos, recorrência, padrão histológico, presença de áreas sólidas e invasão perineural foram descritas em relação à marcação de EZH2 em amostras de CAC. Dessa forma, os resultados do estudo melhoram o entendimento das características clínicas e histológicas do CAC, assim como sobre o comportamento das lesões. Além disso, a análise mostra que o EZH2 é um potencial marcador de malignidade e ressalta a importância da validação de marcadores moleculares de alterações epigenéticas.

Salivary gland neoplasms present different behaviors, which do not follow the classic patterns of benign and malignant neoplasms. The rarity of some of these lesions makes it even more difficult to understand the mechanisms involved in the etiopathogenesis. Molecular markers such as the EZH2 protein have been used to investigate epigenetic changes in different neoplasms, helping to define the diagnosis and prognosis of the lesions. The aim of the study was to evaluate the expression of the EZH2 protein and to describe the clinical and microscopic characteristics of adenoid cystic carcinoma (ACC) and pleomorphic adenoma (PA) which emphasizes the importance of defining the malignancy of the neoplasm. Histopathological analysis of PA cases showed myoepithelial and glandular epithelial cells arranged as duct-like structures and sheets intermingled in the variable stroma and ACC cases showed the three growth patterns, tubular, cribriform and solid forms. All ACC and PA cases were positive for EZH2, with diffuse nuclear staining in neoplastic cells. The ACC samples showed significantly higher EZH2 expression compared to the PA. The covariables nodal metastasis, recurrence, growth pattern, presence of solid areas and perineural invasion have been described in relation to EZH2 staining in ACC samples. The results of the study improve the understanding of the clinical and histological characteristics of ACC, as well as on the behavior of lesions. In addition, the analysis showed that EZH2 is a potential marker of malignancy and highlights the importance of validating molecular markers of epigenetic alterations.

Le carcinome adénoïde kystique des glandes salivaires accessoires: Particularités cliniques et modalités thérapeutiques / Adenoid cystic carcinoma of accessory salivary glands: clinical features and therapeutic modalities

Etudier les particularités cliniques des carcinomes adénoïdes kystiques des glandes salivaires accessoires et discuter les modalités thérapeutiques de ce type tumoral. Patients et méthodes: Il s'agit d'une étude rétrospective descriptive ayant colligé 11 patients présentant un carcinome adénoïde kystique (CAK) des glandes salivaires accessoires (GSA) pris en charge dans notre département sur une période de 20 ans. Résultats: L'âge moyen de nos patients était de 51 ans avec un sex ratio à 0,83. Le délai moyen de consultation était de 20 mois. Le site tumoral était: la cavité buccale dans 3 cas, le massif facial et le cavum dans 6 cas, la trachée et le larynx dans 2 cas. Les motifs de consultation étaient variables selon le site tumoral. L'extension péri neurale était objectivée dans 3 cas. Le traitement était chirurgical suivi de radiothérapie chez 5 patients. Une radiothérapie exclusive a été indiquée chez 3 patients et une radio chimiothérapie concomitante dans 3 cas. Un recul moyen de 42 mois a pu être obtenu pour 10 patients. Une patiente a été perdue de vue. L'évolution était favorable avec absence de récidive locale chez 8 patients. Conclusion: Le traitement de référence reste une chirurgie d'exérèse large, suivie de radiothérapie adjuvante. L'imagerie est indispensable pour le bilan d'extension locorégional et à distance qui conditionne l'attitude thérapeutique.

Le carcinome adénoïde kystique des glandes salivaires accessoires: Particularités cliniques et modalités thérapeutiques / Adenoid cystic carcinoma of accessory salivary glands: clinical features and therapeutic modalities

Objectif: Etudier les particularités cliniques des carcinomes adénoïdes kystiques des glandes salivaires accessoires et discuter les modalités thérapeutiques de ce type tumoral. Patients et méthodes: Il s'agit d'une étude rétrospective descriptive ayant colligé 11 patients présentant un carcinome adénoïde kystique (CAK) des glandes salivaires accessoires (GSA) pris en charge dans notre département sur une période de 20 ans. Résultats: L'âge moyen de nos patients était de 51 ans avec un sex ratio à 0,83. Le délai moyen de consultation était de 20 mois. Le site tumoral était: la cavité buccale dans 3 cas, le massif facial et le cavum dans 6 cas, la trachée et le larynx dans 2 cas. Les motifs de consultation étaient variables selon le site tumoral. L'extension péri neurale était objectivée dans 3 cas. Le traitement était chirurgical suivi de radiothérapie chez 5patients. Une radiothérapie exclusive a été indiquée chez 3 patients et une radio chimiothérapie concomitante dans 3 cas. Un recul moyen de 42 mois a pu être obtenu pour 10 patients. Une patiente a été perdue de vue. L'évolution était favorable avec absence de récidive locale chez 8 patients. Conclusion: Le traitement de référence reste une chirurgie d'exérèse large, suivie de radiothérapie adjuvante. L'imagerie est indispensable pour le bilan d'extension locorégional et à distance qui conditionne l'attitude thérapeutique

Treatment and prognosis analysis of perineural invasion on sinonasal adenoid cystic carcinoma / 中华肿瘤杂志

Objective: To analyze the efficacy of sinonasal adenoid cystic carcinoma (ACC) with perineural invasion (PNI), and explore the prognostic value of PNI on sinonasal adenoid cystic carcinoma. Methods: The clinical data of 105 patients with sinonasal ACC admitted to Cancer Hospital, Chinese Academy of Medical Sciences from January 2000 to December 2016 were retrospectively reviewed. All patients were restaged according to American Joint Committee on Cancer 8th edition. Follow-up visits were conducted to obtain information of treatment failure and survival outcome. The Log rank test was used for univariate analysis of prognostic factors, and Cox regression model was used for multivariate prognostic analysis. Results: The maxillary sinus (n=59) was the most common primary site, followed by the nasal cavity (n=38). There were 93 patients with stage Ⅲ-Ⅳ. The treatment modalities included surgery alone (n=14), radiotherapy alone (n=13), preoperative radiotherapy plus surgery (n=10), and surgery plus postoperative radiotherapy (n=68). The median follow-up time was 91.8 months, the 5-year local control (LC), distant metastasis-free survival (DMFS), progression-free survival (PFS), and overall survival (OS) rates were 72.6%, 73.0%, 52.9% and 78.0%, respectively. There were 33 patients (31.4%) with PNI-positive. The 5-year DMFS, PFS, and OS rates of PNI-positive group were 53.7%, 29.4% and 56.5%, respectively, which were significantly inferior to those of PNI-negative group (80.8%, 63.0% and 86.8%, respectively, P<0.05), while there was no significant difference in the 5-year LC rate between both groups (64.5% vs 76.5%, P=0.273). The multivariate Cox regression analysis showed PNI was one of the poor prognostic factors of DMFS (HR=3.514, 95%CI: 1.557-7.932), PFS (HR=2.562, 95%CI: 1.349-4.866) and OS (HR=2.605, 95%CI: 1.169-5.806). Among patients with PNI-positive, the 5-year LC, PFS and OS rates of patients received surgery combined with radiotherapy were 84.9%, 41.3% and 72.7%, respectively, which were significantly higher than 23.3%, 10.0% and 26.7% of patients receiving surgery or radiotherapy alone (P<0.05). Conclusion: The presence of PNI increases the risk of distant metastasis in patients with sinonasal ACC. Compared with patients with PNI-negative, the prognosis of patients with PNI-positive is relatively poor, and surgery combined with radiotherapy for PNI-positive sinonasal ACC results in good clinical outcomes.

Clinicopathological study in 28 cases of oral basaloid squamous cell carcinomas / 北京大学学报(医学版)

OBJECTIVE@#To investigate the clinicopathologic features and prognostic factors in oral basaloid squamous cell carcinoma.@*METHODS@#Retrospective analysis of oral basaloid squamous cell carcinomas patients who underwent tumor resection during the period from January 2002 to December 2020 in the authors' hospital, especially the clinicopathologic characteristics of 28 cases with confirmed diagnosis and follow-up data. Immunohistochemistry was performed to define the helpful markers for differentiation diagnosis. The factors influencing the prognosis were evaluated based on Kaplan-Meier method.@*RESULTS@#The tongue and mouth floor (11 cases, 39.3%) were the most frequently involved sites, followed by gingiva (6 cases, 21.4%), buccal (5 cases, 17.9%), palate (4 cases, 14.3%), and oropharynx (2 cases, 7.1%). The majority of basaloid squamous cell carcinomas were in advanced stage, with 12 cases in stage Ⅱ and 16 cases in stages Ⅲ-Ⅳ. Twelve of 28 patients were identified to have cervical lymph node metastasis, which was confirmed by histopathological examination. The incidence rate of lymph node metastasis was 42.9% (12/28). Nine tumors recurred, with one metastasized to the lung. At the meantime, the 28 conventional squamous cell carcinomas were matched with the same stage, among which 13 cases were identified with cervical lymph node metastasis. The incidence rate of lymph node metastasis was 46.4% (13/28). Five cases recurred, with two cases that metastasized to the lung and one to the brain. The 5-year overall survival rates of the basaloid squamous cell carcinoma and conventional squamous cell carcinoma patients were 54.6% and 53.8%, respectively. Histopathologically, basaloid cells consisted of tumor islands without evident keratinization but frequently with comedo-like necrosis within the tumor islands. CK5/6 and P63 exhibited strongly positive in all the 28 cases, whereas neuroendocrine markers, CgA and Syn, were negative. Eight cases positively expressed P16; one case showed focal SOX10 positive but CK7 negative.@*CONCLUSION@#The majority of oral basaloid squamous cell carcinomas present in advanced stage with a high tendency to lymph node metastasis, but the overall survival rates are not significantly different from conventional squamous cell carcinomas matched with the same stage. The human papilloma virus (HPV), as HPV-positivity rate is high, correlates to good prognosis. In addition, CK7 & SOX10 immunohistochemistry could contribute to differential diagnosis for basaloid squamous cell carcinoma with solid adenoid cystic carcinoma.

High-Grade Transformation in Adenoid Cystic Carcinoma of the Bartholin Gland: Case Report / Transformação de alto grau em carcinoma adenoide cístico de glândula de Bartholin: relato de caso

Abstract Introduction In the present study, we report a case of primary adenoid cystic carcinoma (ACC) of the Bartholin gland with high-grade transformation (HGT). Adenoid cystic carcinoma of the Bartholin gland is a rare tumor and HGT has only been reported in head and neck tumors. Case Report A 77-year-old woman with a non-ulcerated vulvar lesion on the topography of the right Bartholin gland. The patient was submitted to tumor resection followed by V-Y island flap and adjuvant radiotherapy. The histopathological examination revealed primary ACC of the Bartholin gland, with areas of HGT and extensive perineural invasion. The immunohistochemical study with p53 showed a diffuse and strong positive reaction in areas with HGT. After 24 months of follow-up, the patient presented distant metastases and died, despite having undergone to chemotherapy. Conclusion As far as we know, this case is the first description in the literature of HGT in ACC of the Bartholin gland, and HGT appears to be associated with tumor aggressiveness.

Resumo Introdução Este estudo relata o caso de um carcinoma adenoide cístico (CAC) de glândula de Bartholin com transformação de alto grau. O CAC de glândula de Bartholin é um tumor raro, e sua transformação de alto grau é relatada somente em tumores de cabeça e pescoço. Relato de caso Paciente de 77 anos de idade, do sexo feminino, com lesão vulvar não ulcerada na topografia da glândula de Bartholin direita. A paciente foi submetida a ressecção do tumor e realização de retalho em V-Y, seguidas de radioterapia adjuvante. O exame histopatológico revelou CAC primário de glândula de Bartholin, com áreas de transformação de alto grau e invasão perineural. O estudo imunohistoquímico com p53 mostrou reação positiva difusa e intensa em áreas com transformação de alto grau. Após 24 meses de seguimento, a paciente apresentou metástases à distância e faleceu, apesar de ter sido submetida a quimioterapia. Conclusão Pelo que sabemos, este caso é a primeira descrição na literatura de transformação de alto grau em CAC de glândula de Bartholin, e a transformação de alto grau parece estar associada à agressividade do tumor.

Tumor de glándula lacrimal: a propósito de un caso / Lacrimal gland tumor: a case proposal

INTRODUCCIÓN: El carcinoma adenoide quístico (CAQ) es conocido por su lento crecimiento, su tendencia a la recurrencia local y al desarrollo de metástasis a distancia incluso décadas después del diagnóstico inicial, y a pesar de terapias agresivas. OBJETIVO: Presentar el caso de una paciente con CAQ de la glándula lagrimal, tumor muy infrecuente que representa casi el 10% de las lesiones orbitarias ocupantes de espacio. De éstas el 20-30% son de origen epitelial, de las cuales el 55% son lesiones benignas y el 45% malignas. El CAQ es el más común de los tumores epiteliales malignos de la glándula lagrimal (65%). CASO CLÍNICO: Presentamos el caso clínico de una paciente con CAQ de glándula lacrimal diagnosticado de forma incidental tras la realización de TAC desde la consulta de psiquiatría. RESULTADOS Y CONCLUSIONES: El síntoma de presentación más frecuente suele ser la proptosis, acompañada o no de dolor, aunque también puede presentarse como ptosis mecánica, reducción de visión y diplopía. La cirugía radical no ha demostrado mejorar la supervivencia o reducir la recurrencia local en comparación con un abordaje quirúrgico, más conservador en pacientes con tumores de menor tamaño y sin afectación ósea. Las mayores tasas de supervivencia se han conseguido, en pacientes tratados con quimioterapia, con quimioterapia citorreductiva intra-arterial seguida de exenteración orbital y radioterapia.

INTRODUCTION:The adenoid cystic carcinoma (ACC) is known for its slow growth, a tendency to local recurrence and the development of distant metastases even decades after the initial diagnosis, and despite aggressive therapies. AIM:We present the case of a patient with a lacrimal gland tumor. They are very rare tumors that represent almost 10% of space-occupying orbital lesions, of which 20-30% are of epithelial origin, of these, 55% are benign and 45% are malignant. The ACC is the most common malignant epithelial tumor (65%). CASE REPORT :We present the clinical case of a patient with ACC of the lacrimal gland incidentally diagnosed after performing a CT scan from the psychiatric office. RESULTS AND CONCLUSIONS:The most frequent presentation symptom is usually proptosis, accompanied or not accompanied by pain, although it can also occur as mechanical ptosis, vision reduction, and diplopia. Radical surgery has not been shown to improve survival or reduce local recurrence compared to a more conservative surgical approach in patients with smaller tumors and without bone involvement. The highest survival rates have been achieved, in patients treated with chemotherapy, with intra-arterial cytoreductors (IACC) followed by orbital exenteration and radiotherapy.

Expressão estromal e epitelial de syndecan-1 em tumores de glândulas salivares benignos e malignos: qual delas reflete melhor o comportamento? / Stromal and epithelial syndecan-1 expression in benign and malignant salivary gland tumors: which is more reflective of behavior?

Resumo Introdução: Tumores de glândulas salivares são um grupo diversificado de lesões, com várias origens e comportamentos extremamente diferentes, resultam em distintos desfechos para os pacientes. Portanto, a necessidade de descobrir novos marcadores com a capacidade de predizer o comportamento de neoplasias de glândulas salivares benignas e malignas é crucial. O syndecan-1 é uma proteína da superfície celular com papéis significativos em vários aspectos da função tumoral. Sua expressão nas neoplasias das glândulas salivares, especialmente seu componente estromal, ainda não foi investigada. Objetivos: Avaliar a imunopositividade do syndecan-1 nos componentes epiteliais e estromais das neoplasias de glândulas salivares e compará-la entre os subtipos benigno e maligno, além de avaliar sua correlação com os parâmetros clínico-patológicos. Método: Foram corados 133 tumores de glândulas salivares imuno-histoquimicamente com syndecan-1 e a intensidade e porcentagem dessa proteína foram determinadas, comparadas entre as lesões e correlacionadas com fatores clínico-patológicos. Resultados: A análise estatística das lesões com tamanho amostral suficiente mostrou diferenças significantes em porcentagem e intensidade entre os componentes epiteliais e estromais de todos os tumores (p < 0,05). As comparações pareadas demonstraram uma porcentagem de coloração significantemente maior das células epiteliais (p = 0,02) no tumor de Warthin em comparação com o adenoma pleomórfico e o carcinoma adenoide cístico. Da mesma forma, foram observadas intensidades de coloração e/ou percentagens significantemente maiores no carcinoma mucoepidermoide e no carcinoma adenoide cístico em comparação ao adenoma pleomórfico e ao tumor de Warthin (p < 0,05). Dos fatores clinico-patológicos, houve apenas uma correlação negativa significante entre o percentual estromal de carcinoma mucoepidermoide e a idade; e uma diferença significante entre a intensidade estromal + porcentagem de carcinoma adenoide cístico e sexo (p < 0,05). Conclusões: De acordo com nossos achados, o syndecan-1 estromal se correlaciona com o comportamento maligno de tumores de glândulas salivares, demonstra uma expressão mais alta, indica um papel para o syndecan-1 na invasão e metástase tumoral.

Radioterapia definitiva en el carcinoma adenoide quístico de la tráquea. A propósito de un caso / Definitive radiotherapy in adenoid cystic carcinoma of the trachea. A case report

Los carcinomas adenoides quísticos son tumores extremadamente raros, para los cuales la cirugía es el pilar terapéutico; sin embargo, el abordaje quirúrgico puede estar contraindicado en función del tamaño tumoral, el compromiso de estructuras adyacentes o enfermedades concurrentes. En estos casos, la radioterapia externa definitiva puede ofrecer un adecuado control tumoral y alivio de síntomas. El presente reporte de caso describe un paciente con un tumor irregular que rodea la circunferencia de la tráquea, el cual genera disminución de la luz traqueal, en quién se decidió realizar una reducción de volumen de la lesión tumoral con argón plasma, seguido de colocación de un stent en Y (traqueal, bronquial izquierdo y bronquial derecho) y, posteriormente, realizar un tratamiento definitivo con radioterapia externa con técnica de intensidad modulada a dosis de 60 Gy en fraccionamiento de 2 Gy. Tres meses después del tratamiento, el paciente se presenta sin evidencia radiológica de adenopatías en la base del cuello o axila, con un stent endotraqueal permeable, sin estenosis y con una disminución del volumen tumoral, por lo que fue posible el retiro del stent.

Adenoid cystic carcinomas are extremely rare tumors, for which surgery is the mainstay of therapy; however, the surgical approach may be contradictory depending on tumor size, involvement of adjacent structures or concurrent diseases. In these cases, definitive external beam radiation therapy can offer adequate tumor control and symptom relief. The present case report describes a patient with an irregular tumor that surrounds the circumference of the trachea, causing a decrease in the tracheal lumen, in whom it was decided to perform a volume reduction of tumor with argon plasma, followed by placement of a Y-stent (tracheal, left bronchial and right bronchial), and subsequent definitive treatment with external radiotherapy with intensity modulated technique at doses of 60 Gy in 2 Gy fractionation. Three months after treatment, the patient presents without radiological evidence of lymphadenopathy at the base of the neck or axilla, with a patent endotracheal stent, with no stenosis and a decrease in tumor volume, for which the removal of the Stent was feasilble.

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.

Síndrome de Brooke-Spiegler asociado a carcinoma basocelular: reporte de caso / Brooke-Spiegler syndrome associated with basal cell carcinoma: case report

El síndrome de Brooke-Spiegler (SBS) es una entidad rara, autosómica dominante, que ocurre por mutaciones del gen CYLD, el cual funciona como supresor de tumores. Se presenta el caso de una mujer de 50 años de edad, con historia de aparición de lesiones características de tricoepiteliomas que predominaban en nariz, región interciliar y mentón, que iniciaron desde los 14 años de edad. Desde hace 5 años refiere aumento del tamaño de lesiones en alas nasales, y una lesión en punta nasal de 2 años de evolución. Al realizarse una correlación clínica e histológica, asociada a los antecedentes familiares de la madre y hermano de la paciente, se concluyó que el cuadro clínico era compatible con tricoepitelioma múltiple familiar, una variante especial del SBS, en este caso asociado a carcinoma basocelular, que aunque no es un hallazgo común, se ha visto que se puede presentar en esta enfermedad. El diagnóstico preciso de SBS requiere de una correlación clínico-histológica, y se debe hacer un seguimiento clínico cercano para detectar cambios en las lesiones en piel, que puedan indicar una transformación maligna

Brooke-Spiegler syndrome (BSS) is a rare autosomal dominant condition that occurs due to mutations in the CYLD gene, which functions as a tumor suppressor gene. The case of a 50-year-old woman with a history of characteristic trichoepitheliomas predominantly in the nose, glabella and chin that began at 14 years of age is presented. She reports an increase in the size of the nasal ala lesions for the past 5 years, and the appearance of a new lesion in the nasal tip 2 years ago. When performing a clinical and histological correlation, associated with family history in both the mother and brother, it was concluded that the diagnosis was compatible with multiple familial trichoepithelioma, a special variant of BSS, associated in this case with basal cell carcinoma, that although not a common finding, has been seen to coexist in this disease. The diagnosis of BSS requires a clinical and histological correlation, and a close clinical follow-up must be performed to detect changes in the skin lesions that may indicate malignant transformation

Immunohistochemical expression of OCT4 and CD44 in major and minor salivary gland neoplasms

Abstract The aim of this study was to identify tumor parenchyma cells exhibiting immunohistochemical profile of stem cells by evaluating the immunoreactivity of OCT4 and CD44 in a number of cases of salivary gland neoplasms. The sample consisted of 20 pleomorphic adenomas, 20 mucoepidermoid carcinomas, and 20 adenoid cystic carcinomas located in major and minor salivary glands. The expression of OCT4 and CD44 was evaluated by the percentage of positive cells and the intensity of expression. All studied cases showed positive expression of OCT4 and CD44 and higher values than the control groups. For OCT4, luminal and non-luminal cells were immunostained in the case of pleomorphic adenomas and adenoid cystic carcinomas. Moreover, the immunoreactivity of CD44 was particularly evident in the non-luminal cells of these lesions. In mucoepidermoid carcinomas, there was immunoreactivity for both markers in squamous and intermediate cells and absence of staining in mucous cells. For both markers, a significantly higher immunostaining was verified in neoplasms located in the major salivary glands compared with lesions in minor salivary glands (p<0.001). In the total sample and in minor salivary glands, malignant neoplasms exhibited higher immunoreactivity for OCT4 than pleomorphic adenoma. A significant moderate positive correlation (r = 0.444 and p ≤ 0.001) was found between OCT4 and CD44 immunoexpression in the total sample. The high expression of OCT4 and CD44 may indicate that these proteins play an important role in identifying tumor stem cells.

Neoplasias orofaciais em crianças e adolescentes / Orofacial neoplasms in children and adolescents

Objetivo:Este trabalho analisou os casos de neoplasias orofaciais em crianças e adolescentes, diagnosticadas pelo Laboratório de Patologia Oral da Universidade Federal de Pernambuco no período de março de 2000 a março de 2019. Métodos: Tratou-se de um estudo retrospectivo das neoplasias mais prevalentes diagnosticada no serviço. Os dados foram tabulados e analisados através do programa Statistical Package for the Social Sciences(SPSS) na versão 24.0. Resultados:Dos 6.766 casos diagnosticados, 101 foram de neoplasias, destes 52,5% foram no sexo feminino. A média de idade entre os pacientes foi de 12,6 anos. Em relação ao tipo de biópsia, a excisional foi mais prevalente e em 71,3% dos casos os diagnósticos histopatológicos confirmaram as hipóteses diagnósticas clínicas. A neoplasia benigna não odontogênica mais comum foi o papiloma oral com 30 casos (29,7%). Entre os tumores benignos de origem odontogênica, os odontomas foram os mais prevalentes com 16 casos (15,8%). Foram diagnosticados apenas dois tipos de neoplasias malignas: o carcinoma adenoide cístico e o leiomiossarcoma. Conclusão:Levantamentos epidemiológicos de lesões orofaciais são importantes para determinar a sua prevalência, além de contribuir com a determinação das características na população estudada, fornecendo ao cirurgião-dentista uma base sólida para o diagnóstico e manejo clínico das neoplasias nesses indivíduos.

Aim:To analyze the cases of orofacial neoplasms in children and adolescents, diagnosed by the Oral Pathology Laboratory of the Federal University of Pernambuco from March 2000 to March 2019. Methods: Treatment of a retrospective study of the most prevalent neoplasms diagnosed at the laboratory. Data were tabulated and analyzed using the Statistical Package for Social Sciences (SPSS), version 24.0. Results:Of the 6,766 cases diagnosed, 101 were neoplasms, 52.5% of these were not female. The average age among the patients was 12.6 years. Regarding the type of biopsy, tooth extractions were more prevalent, and in 71.3% of the cases, the histopathological diagnoses confirmed the clinical diagnostic hypotheses. The most common non-odontogenic benign neoplasm was the oral papilloma, found in 30 cases (29.7%). Among the benign tumors of odontogenic origin, the odontomas were the most prevalent in 16 cases (15.8%). Only two types of malignant neoplasms were diagnosed: adenoid cystic carcinoma and leiomyosarcoma. Conclusion:Epidemiological surveys of orofacial lesions are important to determine their prevalence and aid in defining the characteristics in the studied population, providing the dentist with a continuous basis for the diagnosis and clinical treatment of neoplasms in these individuals.

Expression of thymidylate synthase in salivary adenoid myoepithelial cells and its clinical significance / 南方医科大学学报

OBJECTIVE@#To evaluate the expression of thymidylate synthase (TS) in myoepithelial cells (MECs) of salivary adenoid tissues and explore its clinical significance.@*METHODS@#Immunohistochemical staining EnVision method was used to detect the expression of TS, P63, Calponin, CK5/6 and S-100 in 32 salivary gland specimens, including 10 non-neoplastic and salivary inflammation specimens, 11 mixed tumor specimens, 5 basal cell carcinoma specimens and 6 adenoid cyst carcinoma specimens. The specificity and sensitivity of TS as a specific molecular marker of salivary muscle epithelial cells were evaluated in comparison with P63, Calponin, CK5/6 and S-100.@*RESULTS@#The expression pattern of TS in all the salivary gland tissue specimens was identical with that of p63. TS and P63 both showed strong immunohistochemical expressions in MECs of salivary adenoid tissue specimens. Calponin, CK5/6, and S-100 showed cytoplasmic/membranous expressions in the MECs. In addition, TS exhibited weak or moderate cytoplasmic expression in a few salivary gland epithelial cells, cancer cells and scattered stromal cells, with negative expression in the cell nuclei. The expression of TS in the MECs of all the salivary adenoid specimens was highly consistent with those of P63, Calponin, CK5/6 and S-100 (>0.05) Except for CK5/6 expression in Salivary inflammation and Salivary gland specimens. Kappa>0.75. The specificity and sensitivity of TS as a molecular marker of MECs were both 100%.@*CONCLUSIONS@#TS is a new specific marker of MECs for differential diagnosis of salivary gland tumors.

Cervical lymph node metastasis in adenoid cystic carcinoma of the salivary glands: A clinicopathologic study / 北京大学学报(医学版)

OBJECTIVE@#To investigate the incidence and clinicopathologic features of cervical lymph node metastasis in salivary gland adenoid cystic carcinoma (AdCC).@*METHODS@#Retrospective analysis was made in 798 AdCC patients who underwent tumor resection during January, 2001 to January, 2019 in Peking University School and Hospital of Stomatology, especially the clincopathologic characteristics of 82 cases with lymph node metastasis.@*RESULTS@#In the study, 82 of the 798 patients were identified with cervical lymph node metastasis, which was confirmed by histopathological examination. The palate, the region of sublingual gland and mouth floor, and the parotid were the frequently involved primary sites for AdCCs. The general incidence rate of lymph node metastasis was approximately 10%. The submandibular gland, the region of sublingual gland and mouth floor, and the mobile tongue were the most frequent sites of lymph node metastasis with the incidence rates of 20.8%, 16.1%, and 15.1%, respectively; while lymph node metastasis was uncommon in the tumors which were the origin from the palate and parotid, with incidence rates of 6.1% and 3.4%, respectively. Most AdCC cases (70.7%) showed the classic "tunnel-style" metastatic pattern of occurrence, and the level I and II regions were the most frequently involved areas. The 5-year and 10-year overall survival rates of the patients with lymph node metastasis were 77.4% and 20.6% respectively, while the 5-year and 10-year overall survival rates of the patients with no lymph node metastasis were 83.5% and 57.6%, respectively. The univariate analysis demonstrated that statistically significant differences in the overall survival for the presence of lymph node metastasis (P<0.001). In the meantime, the 5-year disease-free survival rate of the patients with lymph node metastasis also showed statistically significant differences to that of the AdCC patients with no lymph node metastasis. In addition, the primary site and histological grade were significantly associated with lymph node metastasis, and the high-grade solid growth pattern was identified as a strong predictor for the occurrence of lymph node metastasis.@*CONCLUSION@#Cervical lymph node metastasis has a high tendency of occurrence in submandibular gland and tongue-mouth floor complex, and the high-grade solid growth pattern could be taken as a strong predictor for the occurrence of lymph node metastasis, which correlates to poor prognosis of AdCC patients. A selective neck dissection should be considered as a management in such patients.