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.

Melanoma mucoso nasal: una enfermedad impredecible / Mucosal melanoma of the nasal cavity: an unpredictable disease

RESUMEN El melanoma mucoso maligno es un tumor neuroectodérmico de menos del 5% de las neoplasias nasosinusales. Es un tumor de mal pronóstico clásicamente su promedio de supervivencia a cinco años es cercano al 28% y 17,5% a diez años, siendo aún peor cuando existe metástasis en cuello. Se presentan a continuación dos casos de melanoma mucoso en cavidad nasal y senos paranasales, el primero mujer 58 años con melanoma septal llevada a septotomía abierta más quimioterapia y radioterapia adyuvante, con recurrencia en cuello submaxilar derecho tratada con vaciamiento supraomohioideo actualmente sin recurrencia; el segundo hombre de 40 años con antecedente de resección melanoma septal quien presenta nuevo melanoma región etmoidal izquierda luego de su primera cirugía y es llevado a resección endoscópica transnasal sin tratamiento adyuvante por decisión del paciente.

ABSTRACT Sinonasal mucosal melanoma is a neuroectodermic tumor account less 5% of nasosinusal tumors. It has a poor prognosis with a five-year average survival near 28% and 17.5% to 10 years; if neck metastasis is even worst. Here we present two clinical cases. First one a woman 58 years old with septal melanoma who receives an open septectomy with chemotherapy and radiotherapy with submaxillary recurrence treated with supraomohyoid neck dissection. The second case, a man 40 years old with previous septal melanoma surgery who has an ethmoid sinus recurrence and receives transnasal endoscopic surgery without adjuvant therapy by patient desire.

Sinonasal mucoepidermoid carcinoma: a case report and literature review / Carcinoma mucoepidermoide nasosinusal: reporte de caso y revisión de la literatura

We report on a patient who presented at our clinic with a growth of the right palate of twenty years duration. A biopsy of the area was performed and the lesion was diagnosed as low-grade mucoepidermoid carcinoma (MEC). Due to the size of the tumor, it was decided to begin treatment with radiotherapy followed by chemotherapy. Once the initial treatment was completed, the lesion was reevaluated and surgery followed by reconstruction was recommended. The Patient rejected the recommended treatment and opted to enrolled in a pain management, palliative care program.

Reportamos sobre un paciente que se presentó con un crecimiento del paladar derecho de veinte años de evolución. Se realizó una biopsia del área y se diagnosticó la lesión como carcinoma mucoepidermoide (MEC) bajo grado. Debido al tamaño del tumor, se decidió comenzar el tratamiento con radioterapia seguida de quimioterapia. Una vez que el tratamiento inicial se completó, la lesión fue reevaluada y se recomendó la cirugía seguida de reconstrucción. El paciente rechazó el tratamiento recomendado y optó por seguir un tratamiento de manejo del dolor, programa de cuidados paliativos.

Melanoma primário de mucosa do trato nasossinusal: relato de 18 pacientes e análise de 1077 pacientes na literatura / Primary mucosal melanoma of the sinonasal tract: report of 18 patients and analysis of 1077 patients in the literature

O presente estudo tem por objetivo relatar as características, fatores prognósticos e desfecho de tratamento de 18 casos de melanoma da mucosa nasossinusal e conduzir uma revisão da literatura. MÉTODO: Entre 1995 e 2005, 18 pacientes consecutivos diagnosticados com melanoma da mucosa do trato nasossinusal e tratados em nossa instituição. Para encontrar as principais casuísticas de estudos relacionados, conduzimos uma revisão da literatura em PubMed e Scopus. No total, encontramos 35 casuísticas, incluindo 1.077 pacientes com melanoma da mucosa nasossinusal. RESULTADOS: Encontramos 16 homens e 2 mulheres. A idade de apresentação foi 51-80 anos (mediana de 58 anos). Todos os pacientes sofreram excisão cirúrgica seguida de radioterapia com ou sem quimioterapia. A mediana de sobrevida foi de 15 meses e a sobrevida geral de 5 anos foi de 23 por cento. Sob análise univariada, estadiamento e remissão completa após tratamento inicial foram importantes fatores prognósticos. Entretanto, sob análise multivariada, somente estágio da doença teve significância estatística. CONCLUSÃO: Melanoma nasossinusal é um tumor raro e agressivo com alto fracasso locorregional e distante e pobre desfecho de tratamento. Entretanto, em uma revisão da literatura, encontramos significativa melhora em termos de sobrevida de 5 anos para casuísticas recentes comparadas com casuísticas previamente relatadas.

The present study aimed at reporting on the characteristics, prognostic factors and treatment outcomes of 18 cases of nasosinusal mucosa melanoma, and do a literature review on the subject. METHODS: between 1995 and 2005, 18 patients consecutively diagnosed with nasosinusal mucosa melanoma were managed in our institution. We reviewed the literature in PubMed and Scopus in order to find the main series from studies associated with this topic. We found a total of 35 series, involving 1,077 patients with nasosinusal mucosa melanoma. RESULTS: we found 16 men and 2 women, with age at presentation between 51 and 80 years (median of 58 years). All these patients were submitted to surgical excision followed by radiotherapy with or without chemotherapy. The survival median was 15 months, and the 5-year general survival had a percentage value of 23 percent. Considering our univariate analysis: tumor staging and complete remission after initial treatment were deemed relevant prognostic factors. Nonetheless, considering the multivariate analysis, only disease stage was statistically significant. CONCLUSION: nasosinusal melanoma is a rare and aggressive tumor, with high loco-regional and distant failure rates, and poor treatment outcomes. Notwithstanding, in a literature review we found significant improvements considering the 5-year survival for recent series when compared to previously reported ones.

Teratocarcinosarcoma rinosinusal: reporte de un caso / Rhinosinusal teratocarcinosarcoma: a case report

Teratocarcinosarcoma rinosinusal es una neoplasia maligna infrecuente y rara, que combina elementos de teratoma y carcinosarcoma. Se compone de epitelio benigno o maligno (fibroblastos), mesénquima (cartílago, hueso o músculo liso) y elementos neurales. Hasta el año 2008 se han reportado un total de 63 casos de ubicación rinosinusal. Presentamos el caso de un teratocarcinosarcoma de fosa nasal, en un hombre de 67 años de edad, que consultó por obstrucción nasal rápidamente progresiva. La lesión se resecó completamente por abordaje endoscópico. El diagnóstico fue confirmado por histopatología e inmunohistoquímica. Se realizó tratamiento complementario con radioterapia, sin evidencia de recidiva al año después de la cirugía.

Rhinosinusal teratocarcinosarcoma is a rare malignant neoplasm, which combines teratoma and carcinoma elements. It is composed of benign or malignant epithelium (fibroblasts), mesenchymatic (cartilage, bone or smooth muscle) and neural elements. Until 2008, a total of 63 cases of rhinosinusal location have been reponed. We present the case of a nasal cavity teratocarcinosarcoma ín a 67 year old man that presented with rapidly progressing nasal obstruction. The lesion was completely resected by endoscopic approach. Diagnosis was confirmed by histological pathology and immunohistochemistry Additional treatment by radiotherapy was administered, with no evidence of recurrences a year after surgery.

Tumores nasossinusais raros: série de casos e revisão de literatura / Rare nasosinusal tumors: case series and literature review

Os tumores nasossinusais são patologias pouco freqüentes na prática clínica. Aproximadamente 0,8 por cento de todos os cânceres humanos localizam-se nessa região. Apesar de rara, a neoplasia nasossinusal manifesta-se habitualmente através de sintomas inespecíficos e comuns a inúmeras patologias inflamatórias. Este estudo se propõe a descrever uma série de casos de tumores nasossinusais não-epiteliais raros, incluindo estesioneuroblastoma, granuloma central de células gigantes, plasmocitoma extramedular, hemangiopericitoma sinonasal, neurofibroma e fibroma cemento-ossificante, diagnosticados no Hospital Geral de Fortaleza, SESA/SUS. Faz-se uma breve revisão de literatura de cada patologia, salientando-se a necessidade do diagnóstico anatomopatológico preciso para condução adequada de cada caso.

Tumors of the nasal cavity and paranasal sinuses are unusual pathologies found in clinical practice. Approximately 0.8 percent of all human cancers are located in this area. Despite being rare, nasosinusal neoplasms usually manifest through nonspecific symptoms that are common to numerous inflammatory pathologies. The aim of this study is to describe a series of rare nasosinusal tumors, including esthesioneuroblastomas, central giant cell granulomas, extramedullary plasmocytomas, nasosinusal hemangiopericytomas, neurofibromas and cemento-ossifying fibromas, diagnosed at the Fortaleza General Hospital. We, hereby, briefly review each of the aforementioned pathologies, stressing the need for a precise histological diagnosis for proper treatment in each case.

Resecciones craneales / Craneofacial resections

Evaluar la resección craneofacial como procedimiento de abordaje rutinario en pacientes que presentan tumores en los senos paranasales, sobre todo en aquellas lesiones que afectan el piso de la fosa craneal anterior y media. Se describen 8 pacientes diagnosticados y tratados en el Hospital Oncologíco "Padre Machado" y en el Intituto Médico La Floresta, Caracas, desde el año 1999 al 2005, se describen características de los pacientes, del tumor, extensión de la invasión, tipo de resección y reconstrucción, márgenes de resección, morbilidad y mortalidad post operatorias. No hubo mortalidad en el presente estudio. Las complicaciones post-operatorias ocurrieron en 25 por ciento de los casos. Infección de herida quirúrgica en 12,5 por ciento. Dentro de las complicaciones en el procedimiento a nivel del sistema nervioso central, el neumoencéfalo y el absceso cerebral representó el 37,5 por ciento. La sobrevida promedio a 2 años es de 70 por ciento. Las resecciones craneofaciales son un procedimiento seguro, con baja mortalidad y morbilidad generalmente asociada a co-morbilidad y antecedentes médicos del paciente, que permiten resecar adecuadamente con márgenes oncológicos lesiones localisadas en los senos paranasales próximos o con compromiso de la fosa cerebral anterior o media; donde es importante el manejo multidisciplinario de estas lesiones.

Linfoma näo-Hodgkin em cavidade nasal e seios paranasais: relato de 2 casos / Non-Hodgkin's lymphomas of the paranasal sinuses and nasal cavity: report of 2 cases

Os autores apresentam 2 casos de Linfoma nao-Hodgkin de cavidade nasal e seios paranasais, patologia esta que necessita um diagnóstico preciso para um tratamento adequado, uma vez que características histopatológicas convencionais nao sao definitivas.

Advances in the management of cancer of the paranasal sinuses

Tumores malignos dos seios paranasias säo incomuns e geralmente diagnosticados em estádios avançados da doença. Os fatos mais inportantes que ocorreram nesta área nos últimos 20 anos foram: 1) acumulou-se significativo número de informaçöes epidemiológicas com respeito a alguns dos fatores de risco envolvidos na etiologia destes tumores, 2) desenvolvimento da tomografia computadorizada de alta resoluçäo e ressonância nuclear magnética, que permitem excelente avaliaçäo da extensäo tumoral antes do tratamento, 3) sistema de estadiamento que permite a comparaçäo de resultados do tratamento, 4) a cirurgia craniofacial é método seguro e potencialmente curativo para alguns casos selecionados que eram consideraçöes inoperáveis ou eram operados inadequadamente há apenas alguns poucos anos, 5) preservaçäo do conteúdo orbitário quando isso é oncologicamente seguro, 6) avanços em reabilitaçäo protética permiten melhores resultados funcionais e estéticos, 7) desenvolvimento de técnicas de transferências de tecido com anastomose microvascular tem estimulado o desenvolvimento de ressecçöes mais radicais, 8) terapêutica combinada incluindo cirurgia, radioterapia e quimioterapia tem mostrado melhora nos resultados a curto prazo