Manejo multidisciplinario del carcinoma adenoide quístico de cabeza y cuello de alto grado / Multidisciplinary management of head and neck high grade adenoid cystic carcinoma

Introducción: El carcinoma adenoide quístico es un tumor epitelioide de la cabeza y el cuello poco común, ocurre más frecuentemente en el las glándulas salivares menores del paladar duro; se presenta sobretodo en la sexta década de la vida, exhibe un crecimiento lento, con recurrencia local frecuente y presencia de metástasis a distancia. La probabilidad de invasión perineural de este tumor es alta. El tratamiento actual consiste en cirugía más radioterapia adyuvante ya que la terapia combinada se ha asociado a mejores desenlaces en supervivencia. Objetivo: Presentar el caso de una paciente con diagnóstico de esta enfermedad, manejo y la conducta postoperatoria. Diseño: Reporte de Caso. Materiales y métodos: Se presenta el caso de una paciente de 34 años quien consultó por masa en lengua de 3 años de evolución, refiriendo disartria, otalgia izquierda y disfagia; en quien se diagnosticó carcinoma adenoide quístico, grado histológico 2 e invasión perineural, realizándose posteriormente glosectomía total y hemimandibulectomía izquierda. Resultados: Adecuada evolución, radioterapia postoperatoria con resultado postoperatorio a los 6 meses satisfactorio, periodo en el cual logra emisión de palabras comprensibles, con adecuada comunicación. Conclusiones: Ante la sospecha de un carcinoma adenoide quístico, se debe realizar biopsia, para definir celularidad y grado histológico del tumor, así como la presencia de invasión perineural. El tratamiento debe tener un enfoque multidisciplinario para lograr el mejor desenlace posible. El seguimiento debe ser cercano por lo menos en los 10 primeros años, dado la alta tendencia de este tumor a recidivar.

Introduction: The adenoid cystic carcinoma is a rare epithelioid head and neck tumor, which occurs more frequently in minor salivary glands of the hard palate and mainly during the sixth decade of life, showing slow growth rate, with frequent local recurrence and distant metastases. The probability of perineural invasion is high. Current treatment involves surgery plus adjuvant radiotherapy because of this has been associated with better outcomes in survival. Objective: To present the case of a patient diagnosed with this disease, management and postoperatory conduct. Design: Case Report. Materials and methods: We present the case of a 34 years old who consulted for mass on her tongue of three years of evolution, referring dysarthria, left otalgia and dysphagia; in whom adenoid cystic carcinoma, histological grade 2 and perineural invasion was diagnosed and subsequently a left hemimandibulectomy and total glossectomy were performed. Results: Adequate evolution, postoperative radiotherapy with satisfactory postoperatory outcome at 6 months, when achieves emission of understandable words, with proper communication. Conclusions: In a suspected adenoid cystic carcinoma, a biopsy should be performed in order to define cellularity and histological grade of the tumor and the presence of perineural invasion. Treatment should have a multidisciplinary approach to achieve the best possible outcome. Monitoring should be close at least the first 10 years, given the high propensity of this tumor to recur.

Carcinoma mucoepidermoide de las glándulas salivares en Brasil: resultados clinicopatológicos y una breve revisión / Mucoepidermoid carcinoma of the salivary glands in Brazil: clinicopathological outcomes and a brief review

The biological features and the clinical behavior of the mucoepidermoid carcinoma are varied and not known yet. The aim of present paper was to analyze the potential prognostic factors affecting the survival of patients diagnosed with primary mucoepidermoid carcinoma of head and neck. A retrospective study was conducted in 16 patients treated between 1990 and 2008 in the General Hospital of Riberirao Preto, USP Medicine School, Brazil. The following variables were studied: age, sex, anatomical location, tumor size, clinical stage, histological degree, relapse, metastasis, involved surgical edges ant treatment on the clinical-pathological results. The survival curves were designed using the Kaplan-Meier method and the statistic analysis was made using the log-rank test. The 68.7 percent of patients was of male sex, all patients were between 13 and 83 years old. The 75 percent of tumors was located in the great salivary glands, the 56.3 percent in the parotid glands ones, the mucoepidermoid carcinomas of low degree and of II stage were the 37.5 percent. The surgical resection was carried out in all patients. The follow-up period in present study fluctuates between 6 and 217 months. The general rate of 5- y years or 10-years survival was of 85.6 percent whereas the rates of disease-free survival were of 81.8 percent at 5 years and of 68.2 percent at 10 years. The were statistically significant influences of the tumor size (p = 0.05), presence of metastasis (p = 0.04) and of the primary anatomical location (p = 0.04) on the rates of disease-free survival. The results obtained show the significance of the primary anatomical location of the tumor, of its size and of the presence of metastasis in the survival of mucoepidermoid carcinomas(AU)

Las características biológicas y el comportamiento clínico del carcinoma mucoepidermoide son muy variados y aún poco conocidos. El propósito de este estudio fue analizar los factores pronósticos que puedan afectar la supervivencia de los pacientes con diagnóstico de carcinoma mucoepidermoide primario de cabeza y cuello. Se realizó un estudio retrospectivo de 16 pacientes tratados entre 1990 y 2008 en el Hospital General de Ribeirao Preto, Escuela de Medicina USP, Brasil. Se estudiaron las variables: edad, sexo, localización anatómica, tamaño del tumor, estadio clínico, grado histológico, recidiva, metástasis, bordes quirúrgicos comprometidos y tratamiento, sobre los resultados clínico-patológicos. Las curvas de supervivencia fueron construidas utilizando el método de Kaplan-Meier y el análisis estadístico fue realizado mediante la prueba del log-rank. Se constató 68,7 por ciento de pacientes del sexo masculino, todos los pacientes comprendidos en las edades entre 13 y 83 años. El 75 por ciento de los tumores se localizó en las glándulas salivales mayores, 56,3 por ciento en parótida, los carcinomas mucoepidermoides de bajo grado y estadio II con 37,5 por ciento. La resección quirúrgica fue realizada en todos los pacientes. El período de seguimiento en este estudio varió entre 6 y 217 meses. La tasa general de supervivencia, tanto a los 5 como a los 10 años fue de 85,6 por ciento, mientras que las tasas de supervivencia libre de enfermedad fueron de 81,8 por ciento a los 5 años y de 68,2 por ciento a los 10 años. Se demostró la existencia de influencias estadísticamente significativas del tamaño del tumor (p = 0,05), presencia de metástasis (p = 0,04), y de la localización anatómica primaria (p = 0,04) sobre las tasas de supervivencia libre de enfermedad. Los resultados obtenidos demuestran la importancia de la localización anatómica primaria del tumor, de su tamaño y de la presencia de metástasis, en la supervivencia de los carcinomas mucoepidermoides(AU)

Treatment results and prognostic analysis of 54 patients with adenoid cystic carcinoma originated from the major salivary glands / 中华放射肿瘤学杂志

Objective Adenoid cystic carcinoma (ACC), a rare malignancy in head and neck region, is predominately found in the salivary glands. Our study is to retrospectively analyze the treatment outcomes and prognostic factors of ACC originated from the major salivary glands. Methods A total of 54 patients diagnosed as ACC were treated in our institution, including 24 cases originated from the parotid gland and 30 from the submandibular or sublingual gland. According to the records, 26 patients received surgery alone and 28 were treated with surgery followed by radiotherapy with a median dose of 58 Gy (range, 50 -65 Gy). Results The Follow-up rate was 94%, and 15 patients from postoperative radiotherapy group and 20 from surgery alone group were followed up more then 5 years. The 5-year overall survival rate, local-regional control rate, distant metastasis rate, and disease-free survival rate were 97%, 71%, 13% and 69%, respectively. Lung metastasis, occurred in 7 patients, was the most common distant failure. Fifteen recurrences were observed, including 13 in surgery alone group and 2 in postoperative radiotherapy group. The 5-year local-regional control and disease-free survival rates were 90% and 85% for patients treated with postoperative radiotherapy, 54% and 55% for those treated with surgery alone. Univariate and multivariate analyses showed that postoperative radiotherapy was the only prognostic factor of local-regional control and survival rates. Other parameters such as nerve involvement did not significantly influence the treatment results. Conclusions Postoperative radiotherapy can improve the prognosis of ACC originate from the major salivary glands compared with surgery alone. Distant metastasis is an obstacle in curing the disease, which indicates the value of systemic treatment.

Cystic Sebaceous Adenoma of the Parotid Gland: A Case Report

Cystic sebaceous adenomas are rare neoplasms that can arise in salivary glands. Among the salivary glands, the most commonly reported location is the parotid gland where it must be differentiated from other intraparotid masses. Unfortunately, its imaging features are not well-known as a result of its rarity. We report a case of cystic sebaceous adenoma that manifested as a gradually enlarging mass within the parotid gland of a 60-year-old man.

Cystic Sebaceous Adenoma of the Parotid Gland: A Case Report

Cystic sebaceous adenomas are rare neoplasms that can arise in salivary glands. Among the salivary glands, the most commonly reported location is the parotid gland where it must be differentiated from other intraparotid masses. Unfortunately, its imaging features are not well-known as a result of its rarity. We report a case of cystic sebaceous adenoma that manifested as a gradually enlarging mass within the parotid gland of a 60-year-old man.

Acinic Cell Carcinoma Arising from Unusual Location: 3 Case Reports

Most acinic cell carcinomas arise within the parotid gland: extraparotid origin is rare. We encountered three cases of extraparotid acinic cell carcinoma arising in the buccal or palatal region, or the submandibular gland. All three tumors presented as a painless, slow-growing mass. CT imaging indicated that they were well defined, homogeneously enhanced, round masses. In one case, sonography demonstrated relatively homogeneous low echogenicity.

Pleomorphic Adenoma Arising from Heterotopic Salivary Gland Tissue in the Neck: A Case Report

We report a rare case of pleomorphic adenoma arising from heterotopic salivary gland tissue in the upper neck. Although it is difficult to differentiate this condition from lymph node diseases-including metastasis- on the basis of radiologic findings alone, it should be included in the differential diagnosis of a solitary unilateral solid cervical mass, particularly one in the upper neck.