Plasmocitoma extramedular solitario rinosinusal: reporte de caso y revisión de literatura / Solitary extramedullary plasmacytoma of the rhinosinusal cavity: a case report and literature review

Resumen El plasmocitoma extramedular solitario (PES) es una neooplasia maligna infrecuente caracterizada por una proliferación aislada de células plasmáticas monoclonales en tejido extramedular. La localización más frecuente es en cabeza y cuello con predominio en el territorio rinosinusal, sin embargo, estas lesiones malignas representan menos del 1% de los tumores de esta región anatómica. El diagnostico requiere una alta sospecha clínica, análisis histopatológico acucioso, estudios serológicos y exámenes radiológicos sistémicos de acuerdo a los criterios diagnósticos establecidos en la literatura internacional. Se analiza el caso de un paciente masculino con un PES que se presentó como un tumor de fosa nasal derecha y obstrucción nasal de meses de evolución con hallazgos clínicos e imagenológicos inespecíficos. El diagnóstico definitivo se realizó mediante biopsia endoscópica nasal y estudio histopatológico. El tratamiento fue abordado de manera multidisciplinaria entre otorrinolaringología, hematología y radiooncología. De acuerdo a las guías internacionales, se decidió realizar radioterapia localizada con buen resultado clínico precoz. El PES requiere un abordaje multidisciplinario para lograr un diagnóstico y tratamiento oportuno, siendo imprescindible la exclusión del mieloma múltiple debido a las diferencias terapéuticas y en pronóstico clínico. El tratamiento puede realizarse con radioterapia y/o cirugía, siendo la radioterapia el pilar de tratamiento.

Abstract Solitary extramedullary plasmacytoma (SEP) is a rare malignant neoplasm characterized by isolated proliferation of monoclonal plasma cells in extramedullary tissue. The most frequent location is in the head and neck with a predominance in the rhinosinusal territory; however, these malignant lesions represent less than 1% of the tumors in this anatomical region. The diagnosis requires a high clinical suspicion, careful histopathological analysis, serological studies and systemic radiological examinations according to the diagnostic criteria established in the international literature. We analyze the case of a male patient with SEP that presented as a tumor of the right nostril and nasal obstruction of months of evolution with nonspecific clinical and imaging findings. The definitive diagnosis was made by nasal endoscopic biopsy and histopathological study. The treatment was approached by multidisciplinary teamwork. According to international guidelines, it was decided to perform localized radiotherapy with good early clinical results. SEP requires a multidisciplinary approach to achieve a timely diagnosis and treatment, being essential exclusion of multiple myeloma due to the therapeutic differences and prognosis. Treatment can be done with radiation therapy and/or surgery; radiation therapy is the mainstay of treatment.

Does evaluation of tumor volume or/both origination site better guide to optimal surgery for inverted papilloma? / A avaliação do volume do tumor e/ou dos seus locais de origem fornece a melhor orientação para a técnica cirúrgica ideal do papiloma invertido?

Abstract Introduction Sinonasal inverted papilloma is noted for its high rate of recurrence. Staging systems aid to reduce recurrence and avoid excessive surgeries by guiding the selection of the optimal surgical approach. Objective To evaluate the effectiveness of different endoscopic approaches in inverted papilloma by assessing tumor origin site and tumor volume. Methods Krouse classification system that is based on tumor volume was used for staging; furthermore, tumor origin sites were grouped as lateral nasal wall, medial wall and other walls of maxillary sinus. The main treatment method for all patients was endoscopic sinus surgery. Endoscopic extended middle meatal antrostomy, endoscopic Caldwell-Luc and endoscopic medial maxillectomy were the additional surgery types performed in different combinations. Results Fifty-five patients (42 male) with a mean 54.9 ± 14.4 years of age were included. 37 patients were diagnosed with advanced stage inverted papilloma (67.2 %). Recurrence was observed in 12 patients (21.8 %). In early stage lateral nasal wall origination, no recurrence was observed in the simple tumor resection group (0/10). In early stage medial wall origination, no recurrence was observed in the extended middle meatal antrostomy group (0/8). In advanced stage medial wall origination, the recurrence rates of extended middle meatal antrostomy, extended middle meatal antrostomy + endoscopic Caldwell- Luc and endoscopic medial maxillectomy were 100.0 %, 53.8 % and 13.6 %, respectively (p = 0.002). In advanced stage other walls of maxillary sinus origination, recurrence rates of extended middle meatal antrostomy + endoscopic Caldwell-Luc and endoscopic medial maxillectomy were 20 % and 16.6 %, respectively (p = 0.887). Conclusion Tumor origin site, tumor stage and surgery types show an impact on recurrence. Despite the fact that tumor origin site singly could lead to appropriate selection of the surgery type in most cases, tumor stage carries substantial importance in selection of surgery type for sinonasal-inverted papilloma. An operation plan regarding both tumor volume and tumor origin site may aid surgeons in selecting optimal endoscopic surgical method to avoid recurrence or excessive surgeries.

Resumo Introdução O papiloma invertido nasossinusal é conhecido por sua alta taxa de recorrência. Os sistemas de estadiamento ajudam a reduzir a recorrência e evitar cirurgias excessivas e orientam a seleção da abordagem cirúrgica ideal. Objetivo Avaliar a eficácia de diferentes abordagens endoscópicas no papiloma invertido, de acordo com o local de origem e o volume do tumor. Método Para o estadiamento, usou-se o sistema de classificação de Krouse, baseado no volume do tumor; além disso, os tumores foram agrupados de acordo com seus locais de origem: parede nasal lateral, parede medial e outras paredes do seio maxilar. O principal método de tratamento para todos os pacientes foi a cirurgia endoscópica nasossinusal. Foram feitos, em diferentes combinações, os seguintes tipos de cirurgia: antrostomia estendida do meato médio, Caldwell-Luc endoscópica e maxilectomia medial endoscópica. Resultados Foram incluídos 55 pacientes (42 homens) com média de 54,9 ± 14,4 anos. Trinta e sete pacientes foram diagnosticados com papiloma invertido avançado (67,2%). Foi observada recorrência em 12 pacientes (21,8%). No estágio inicial com origem na parede nasal lateral, não foi observada recorrência no grupo de ressecção simples de tumor (0/10). No estágio inicial com origem na parede medial, não foi observada recorrência no grupo de antrostomia estendida do meato médio (0/8). Com tumor em estágio avançado com origem na parede medial, as taxas de recorrência na antrostomia estendida do meato médio, antrostomia estendida do meato médio + Caldwell-Luc endoscópica e maxilectomia medial endoscópica foram de 100,0%, 53,8% e 13,6%, respectivamente (p = 0,002). No tumor em estágio avançado em outras paredes do seio maxilar, as taxas de recorrência na antrostomia estendida do meato médio + Caldwell-Luc endoscópica e maxilectomia medial endoscópica foram de 20% e 16,6%, respectivamente (p = 0,887). Conclusão O local de origem do tumor, o estágio do tumor e os tipos de cirurgia mostram impacto na recorrência. Apesar da consideração de que na maioria dos casos o local de origem do tumor pode, de forma isolada, orientar a seleção apropriada do tipo de cirurgia, o estágio do tumor tem importância substancial na seleção do tipo de cirurgia para papiloma invertido nasossinusal. Um planejamento cirúrgico considerando tanto o volume quanto o local de origem do tumor pode ajudar os cirurgiões a selecionar o tipo ideal de cirurgia endoscópica para evitar recorrências ou remoções excessivas.

Sinonasal inverted papilloma: rate of recurrence and malignant transformation in 44 operated patients / Papiloma invertido nasossinusal: taxa de recorrência e transformação maligna em 44 pacientes operados

Abstract Introduction: Although sinonasal inverted papillomas are benign lesions, they are locally aggressive and have a potential malignant transformation ranging from 5% to 15%, with a high recurrence rate. Objective: The aim of this article is to describe the rate of recurrence and malignant transformation in patients with a diagnosis of inverted papilloma who underwent surgery in a tertiary hospital in São Paulo. Methods: We performed a retrospective analysis of patients diagnosed with sinonasal papilloma who had undergone surgery in a tertiary hospital in São Paulo, between August 1998 and August 2017. A patient chart review was conducted to assess data of patients' demographics, tumors characteristics, follow-up appointments, recurrence and malignancy. Inverted papillomas were analyzed and classified under the Krouse staging system. Results: A total of 69 surgeries were performed in patients with diagnosis of sinonasal papilloma. Inverted papilloma was the most prevalent subtype (49 cases ‒ 80.33%), followed by exophytic papilloma (6 cases ‒ 9.84%) and by oncocytic papilloma (6 cases - 9.84%). The recurrence rate was 34.09% for inverted papilloma (15/44) and the mean time of recurrence was 24.6 months. Malignant transformation occurred in 6 patients (13.64%). Three of these patients presented carcinoma in the first surgery and three patients developed carcinoma during the follow-up. Conclusion: The high recurrence rate and malignancy potential allow us to consider inverted papillomas as aggressive tumors. In a tertiary hospital in São Paulo the recurrence rate the mean time to recurrence is 24.6 months. The recurrence after 10 years implies was 34.09% and the need for long-term follow up. It is possible that the high recurrence rate and the high malignant transformation rate we found are due to the large number of tumors discovered at an advanced stage (most of them staged T3 and T4), secondary to poor access to health system, in developing countries.

Resumo Introdução: Embora os papilomas invertidos nasossinusais sejam lesões benignas, eles são localmente agressivos e apresentam uma potencial transformação maligna que varia de 5% a 15%, com alta taxa de recorrência. Objetivo: Descrever a taxa de recorrência e transformação maligna em pacientes com diagnóstico de papiloma invertido submetidos à cirurgia em um hospital terciário em São Paulo. Método: Análise retrospectiva dos pacientes diagnosticados com papiloma nasossinusal submetidos a cirurgia em um hospital terciário em São Paulo, entre agosto de 1998 e agosto de 2017. Uma revisão de prontuários dos pacientes foi realizada para avaliar dados demográficos, características dos tumores, consultas de seguimento, recorrência e malignidade. Os papilomas invertidos foram analisados e classificados de acordo com o sistema de estadiamento de Krouse. Resultados: Foram realizadas 69 cirurgias em pacientes com diagnóstico de papiloma nasossinusal. O papiloma invertido foi o subtipo mais prevalente (49 casos - 80,33%), seguido pelo papiloma exofítico (6 casos - 9,84%) e pelo papiloma oncocítico (6 casos - 9,84%). A taxa de recidiva foi de 34,09% para o papiloma invertido (15/44) e o tempo médio de recorrência foi de 24,6 meses. Transformação maligna ocorreu em seis pacientes (13,64%); três desses pacientes apresentaram carcinoma na primeira cirurgia e três pacientes desenvolveram carcinoma durante o seguimento. Conclusão: A alta taxa de recorrência e o potencial de malignidade nos permitem considerar os papilomas invertidos como tumores agressivos. Em um hospital terciário de São Paulo, a taxa de recorrência foi de 34,09% e o tempo médio de recorrência de 24,6 meses. A recorrência após 10 anos implica na necessidade de acompanhamento a longo prazo. É possível que a alta taxa de recorrência e a alta taxa de transformação maligna que encontramos sejam devidas à grande extensão dos tumores (a maioria deles nos estágios T3 e T4), decorrente do acesso precário ao sistema de saúde nos países em desenvolvimento.

Surgical outcomes of sinonasal inverted papilloma: a 17 year review / Resultados cirúrgicos do papiloma invertido nasossinusal: uma revisão de 17 anos

Abstract Introduction: Inverted papillomas represent one of the most common benign neoplasic lesions located in the sinonasal tract. Owing to the local erosive behavior, tendency to recur and the potential for malignant transformation, surgical management of inverted papillomas is often challenging. Objective: This study aimed to analyze the surgical outcomes of patients with inverted papillomas, according to the Krouse staging and the different surgical approaches. Methods: Retrospective study of patients diagnosed with sinonasal inverted papillomas who underwent surgical treatment between 2000 and 2016 at a tertiary referral hospital. Cases with follow-up less than 12 months were excluded. The rate and the time of recurrence were the main outcomes. Values of p < 0.05 were considered statistically significant. Results: Thirty-six cases with mean age of 60 years, predominantly male (72%), were included. The follow-up period ranged from 1 to 16 years, with an average of 4.5 years. Krouse T1 Stage corresponded to 11.1%; T2 occurred in 50% of cases; while T3 and T4 Stages accounted for 30.6% and 8.3% of patients, respectively. Most cases were approached by an endoscopic technique alone (83.3%), with a recurrence rate of 13.3%. Patients treated via a combined or open approach revealed a recurrence of 16.7%. No differences in the recurrence rate were reported when comparing endoscopic surgery with the open or combined techniques. Krouse Stage T3 had a significant association with inverted papillomas recurrence (p = 0.023). All inverted papilloma relapses occurred up to 2 years post-operatively. One case of malignant transformation was recorded (2.7%). Conclusion: Endoscopic surgery did not increase the recurrence rates and can be a safe and efficient alternative to open or combined techniques. The recurrence of inverted papillomas seem to be related to the persistence of the disease and tend to occur early after primary surgery. Krouse T3 Stages may be associated with a higher recurrence of inverted papillomas.

Resumo Introdução: Os papilomas invertidos são uma das lesões neoplásicas benignas mais comuns no trato nasossinusal. Devido ao seu comportamento localmente agressivo, tendência para recidivar e potencial de malignização, o tratamento cirúrgico dos papilomas invertidos constitui frequentemente um desafio. Objetivo: Analisar os resultados cirúrgicos dos papilomas invertidos segundo o estadiamento de Krouse, bem como avaliar as diferentes abordagens cirúrgicas. Método: Estudo retrospectivo de pacientes com diagnóstico de papiloma invertido nasossinusal submetidos a tratamento cirúrgico entre 2000 e 2016 em hospital terciário. Casos com acompanhamento inferior a 12 meses foram excluídos. A taxa e o tempo de recidiva foram os principais resultados analisados desfechos avaliados. Valores p < 0,05 foram considerados estatisticamente significativos. Resultados: Foram incluídos 36 casos, com média de idade de 60 anos, predominantemente do sexo masculino (72%). O período de acompanhamento variou de 1 a 16 anos, em uma média de 4,5 anos. Relativamente ao estadiamento, 11,1% dos pacientes foram classificados como estadio Krouse T1, 50% como T2, 30,6% como T3 e 8,3% como T4. A maioria dos casos foi tratada exclusivamente por cirurgia endoscópica (83,3%), com taxa de recidiva de 13,3%. Pacientes tratados com uma técnica combinada ou aberta apresentaram recidiva de 16,7%. Não foram observadas diferenças quanto à taxa de recidiva entre os casos abordados por via endoscópica e os casos tratados com técnica aberta ou combinada. Verificou-se uma associação significativa entre o estadio T3 de Krouse e recidiva de papilomas invertidos (p = 0,023). Todas as recidivas de papilomas invertidos foram observadas até dois anos após a cirurgia. Um caso de transformação maligna foi registrado (2,7%). Conclusão: A cirurgia endoscópica não aumentou as taxas de recidiva e pode ser uma alternativa segura e eficiente às técnicas abertas ou combinadas. Os casos de recidiva do papiloma invertido parecem estar relacionados com a persistência da doença e tendem a ocorrer precocemente após a cirurgia primária. Os estadios T3 de Krouse podem estar associados a uma maior recidiva.

Tumores fibroóseos de cavidades paranasales: experiencia en el Hospital Clínico de la Universidad de Chile y revisión de la literatura / Fibro-osseous tumors of paranasal cavities: experience in the Hospital Clínico de la Universidad de Chile and review of the literature

Los tumores de cavidades paranasales presentan una baja frecuencia. Dentro de éstos, entre los benignos destacan las lesiones fibroóseas que se caracterizan por el reemplazo de hueso normal por estroma celular fibroso. Dentro de estas lesiones se describen osteoma, displasia fibrosa y fibroma osificante. Se revisan 3 casos de pacientes del Hospital Clínico de la Universidad de Chile y se presenta una revisión bibliográfica en cuanto a las lesiones fibroóseas, su clínica, diagnóstico, imagenología y tratamiento.

The tumors of paranasal cavities present a low frequency. Among the benign tumors are fibro-osseous lesions characterized by the replacement of normal bone by fibrous cell stroma. Osteoma, fibrous dysplasia, and ossifying fibroma are described within these lesions. Three cases of patients from the Hospital Clínico de la Universidad de Chile are reviewed and a bibliographic review is presented regarding the fibro-osseous lesions, their clinical features, diagnosis, imaging and treatment.

Enfrentamiento de pacientes Testigo de Jehová en cirugía otorrinolaringológica: a propósito de un caso de resección de tumor rinosinusal / Jehovah's Witnesses patients in otorhinolaryngological surgery: a case regarding a sinonasal tumor resection

Las cirugías de cabeza y cuello presentan un alto riesgo de sangrado que puede significar el uso de transfusiones sanguíneas. Existen casos en las que éstas son rechazadas, como sucede con los Testigos de Jehová. Se expone el caso de una paciente Testigo de Jehová con un tumor rinosinusal con alto riesgo de sangrado que consultó por epistaxis recurrente. Se evidencia un tumor ocluyendo la fosa nasal derecha de aspecto vascular a la rinoscopía y la tomografía computarizada. Múltiples aferencias de la arteria esfenopalatina y etmoidales se observaron en una angiografía cerebral. Previo a la resección, se embolizó la arteria maxilar. Durante la cirugía, se contó con un sistema de recuperación de sangre autóloga, hemodilución e infusión de ácido tranexámico. Se ligó la arteria etmoidal anterior derecha vía externa con apoyo endoscópico y luego se resecó el tumor vía endoscópica. La biopsia reveló un carcinoma sinonasal escamoso. Existen alternativas terapéuticas en pacientes que rechacen el uso de hemoderivados. Destacan medidas preoperatorias como la embolización endovascular, intraoperatorias como el uso de agentes hemostáticos, técnicas quirúrgicas y anestésicas. Es importante analizar todas las opciones disponibles de forma multidisciplinara y junto con el paciente, para determinar la conducta más adecuada a seguir.

Head and neck surgeries have a high risk of bleeding, and therefore could require the use of blood transfusions. There are cases for which blood transfusions are not an acceptable option, as is the case for Jehovah's Witnesses. We present the case of a Jehovah's Witness with a sinonasal tumor with a high risk of bleeding, who presented with recurrent epistaxis. Rhinoscopy and computed tomography revealed a vascular-like tumor occluding the right nasal cavity. Cerebral angiography showed afferents of the sphenopalatine and ethmoidal arteries leading to the tumor. Prior to the resection, the maxillary artery was embolized. During surgery, we relied on an autologous blood recovery system, hemodilution and tranexamic acid. Right anterior ethmoidal artery ligation was performed by an endoscopic assisted external approach. The tumor was resected endoscopically The biopsy revealed a squamous sinonasal carcinoma. There are therapeutic alternatives for patients who cannot receive blood products. There are preoperative measures such as endovascular embolization, intraoperative measures such as the use of hemostatic agents and specific surgical or anesthetic techniques. It's important to analyze all of the available options in a multidisciplinary team approach, and to take into consideration the patient's preferences, in order to determine the best surgical conduct.

Sinonasal organising haematoma - a little known entity / Hematoma nasossinusal em organização - uma entidade pouco conhecida

Abstract Introduction: Sinonasal organising haematoma is a recently described, rare, benign inflammatory condition, which closely resembles malignancy in its clinical presentation. Objective: To describe the clinical features of organising haematoma and to review the evolution of surgical options successfully used. Methods: A retrospective review of charts of all patients with a histopathological diagnosis of sinonasal organising haematoma was performed. Results: Six (60%) of the 10 patients were male with a mean age of 47.4 years. All patients had unilateral disease with recurrent epistaxis as the presenting symptom. Maxillary sinus was the most commonly involved sinus. There was no history of trauma in any of the patients. Hypertension (80%) was the most commonly associated comorbidity. Contrast-enhanced CT scan of the paranasal sinuses showed heterogeneous sinus opacification with/without bone erosion. Histopathological examination was diagnostic. Complete endoscopic excision was done in all patients resulting in resolution of the disease. Conclusion: Awareness of this relatively new clinical entity and its evaluation and treatment is important for otolaryngologists, maxillofacial surgeons and pathologists alike. Despite the clinical picture of malignancy, histopathological features of benign disease can safely dispel such a diagnosis.

Resumo Introdução: Hematoma nasossinusal em organização é uma condição inflamatória benigna rara, recentemente descrita, que se assemelha a lesões malignas em sua apresentação clínica. Objetivo: Descrever as características clínicas do hematoma em organização e analisar a evolução das opções cirúrgicas usadas com sucesso. Método: Foi feita a revisão retrospectiva dos prontuários de todos os pacientes com diagnóstico histopatológico de hematoma nasossinusal em organização. Resultados: Seis (60%) dos 10 pacientes eram do sexo masculino, com média de 47,4 anos. Todos os pacientes apresentavam doença unilateral com epistaxe recorrente como sintoma de apresentação. O seio maxilar era o mais comumente afetado. Não havia histórico de trauma em qualquer dos pacientes. Hipertensão (80%) foi a comorbidade mais comumente associada. A tomografia computadorizada dos seios paranasais com contraste mostrou opacificação heterogênea do seio com/sem erosão óssea. O exame histopatológico foi diagnóstico. A excisão endoscópica completa foi feita em todos os pacientes, resultou na resolução da doença. Conclusão: A conscientização a respeito dessa entidade clínica relativamente nova e sua avaliação e tratamento são importantes para os otorrinolaringologistas, cirurgiões buco-maxilo-faciais e patologistas. Apesar do quadro clínico de malignidade, as características histopatológicas da doença benigna podem descartar com segurança esse diagnóstico.

Cirugía endoscópica nasal en el tratamiento de papiloma invertido: a propósito de 18 casos / Endoscopic sinus surgery in the management of inverted papilloma: an analysis of 18 cases

RESUMEN Introducción El papiloma invertido nasosinusal es un tumor benigno con tendencia a la recidiva, y con riesgo de malignización. El tratamiento habitual es la extirpación quirúrgica. Objetivo Describir y analizar las características clínicas, epidemiológicas, y resultados del tratamiento quirúrgico por vía endoscópica. Material y método Estudio retrospectivo, descriptivo, incluyendo pacientes con papiloma invertido nasosinusal sometidos a extirpación quirúrgica endoscópica entre los años 2012 y 2016. Se analizaron variables epidemiológicas, clínicas, complicaciones quirúrgicas, recurrencias y transformación maligna. El grado de extensión se evaluó con tomografia computarizada según la clasificación de Krouse. Resultados La serie está constituida por 18 pacientes, con una edad promedio de 57,4 años. La clínica se caracterizó por obstrucción nasal unilateral 72,2% (13/18 pacientes), rinorrea 33,3% (6/18 pacientes), hiposmia 27,8% (5/18 pacientes) y epistaxis 27,8% (5/18 pacientes). Según la clasificación de Krouse, las lesiones iniciales se describen como estadio T1 en 7/18, T2 en 5/18, T3 en 5/18 y T4 en 1/18 pacientes. En el período de seguimiento, se observa recurrencia en 4 pacientes en un período promedio de seguimiento de 5,6 meses, y un caso de transformación maligna. No hubo complicaciones quirúrgicas, excepto un caso de epífora por obstrucción de la vía nasolagrimal. Conclusiones El abordaje endoscópico fue efectivo para el tratamiento de papilomas invertidos nasosinusales.

ABSTRACT Introduction Sinonasal inverted papillomas are benign tumors with a tendency to recur and a potential for malignant transformation. Surgical resection is the treatment of choice. Aim To describe and analyze the clinical characteristics, epidemiology, and surgical results from an endoscopic endonasal approach. Material and method Retrospective and descriptive study of all patients who underwent endoscopic sinus surgery for the treatment of a sinonasal inverted papilloma between the years 2012 and 2016. Variables analyzed include demographics, signs and symptoms, surgical complications, recurrence rate and malignant transformation. Tumor extension was evaluated with the Krouse staging system on computed tomography imaging. Results This series includes 18 patients, with a mean age of 57.4 years. Clinically, patients presented with unilateral nasal obstruction 72.2% (13/18 patients), followed by rhinorrhea 33.3% (6/18 patients), hyposmia 27.8% (5/18 patients) and epistaxis 27.8% (5/18 patients). Based on the Krouse staging system, 7/18 were T1, 5/18 were T2, 5/18 were T3 and 1/18 was T4 on initial evaluation. On follow-up, 4 patients presented with a recurrence at a mean period of 5.6 months, and one patient exhibited malignant transformation. No surgical complications were observed except for one case of epyphora as a consequence of nasolacrimal obstruction. Conclusions The endoscopic approach was effective for the treatment of sinonasal inverted papillomas.

Carcinoma adenoide quístico de cavidad nasal y senos paranasales: experiencia de 5 años en Clínica Las Condes de Santiago de Chile / Adenoid cystic carcinoma of nasal cavity and paranasal sinuses: 5 years experience in las Condes Clinic of Santiago of Chile

RESUMEN Introducción: El carcinoma adenoide quístico es una neoplasia originada en glándulas exocrinas de todo el cuerpo, principalmente en glándulas salivales mayores. En cavidad nasal y senos paranasales es poco frecuente y se caracteriza por presentar una alta frecuencia de recurrencia y de metástasis a distancia posterior a su remisión y a pesar de su tratamiento. Objetivo: Describir características de una serie de casos de pacientes con carcinoma adenoide quístico de cavidad nasal y senos paranasales operados. Material y método: Estudio descriptivo-retrospectivo. Período enero de 2012 y enero de 2017. La información se obtuvo a partir de las fichas electrónicas de la Clínica Las Condes. Se describen procedencia, edad y sexo, características clínicas, hallazgos anatomopatológicos, métodos diagnósticos, tratamiento, evolución. Resultados: Total de 5 pacientes operados, 3 mujeres y 2 hombres. La edad promedio fue de 63 años, con rango de 33 años a 90 años de edad. 100% de los casos sin factores asociados. La presentación clínica más frecuente fue la obstrucción nasal unilateral, seguida por el dolor facial. Dos pacientes se presentaron con diagnóstico inicial, dos por recurrencia y uno por persistencia. En tres casos el tumor se origina de seno maxilar y en dos en seno etmoidal. Todos presentaron enfermedad avanzada, etapa IV y III. Tres pacientes histológicamente fueron de bajo grado y dos de alto grado. En los cinco casos el tratamiento primario fue quirúrgico, en el 60% endoscópico, 20% abierto y 20% combinado. En cuatro casos se usó radioterapia posoperatoria y en tres de éstos, quimioterapia concomitante. Todos se encuentran sin signos de recidiva tumoral en último control. Conclusiones: El carcinoma adenoide quístico de cavidad nasal y senos paranasales es bastante infrecuente, su incidencia es menor a 1/100.000 casos por año. Es más frecuente en mujeres entre 40 y 50 años. Se identifica más con su origen en el seno maxilar (50%) y de patrón cribiforme. Clínicamente se presenta en estadíos avanzados ya que en etapa precoz es asintomático o presenta clínica inespecífica inflamatoria. El diagnóstico se realiza con biopsia complementada con imagenología. El tratamiento más utilizado es la cirugía endoscópica o abierta asociado a radioterapia posoperatoria, a pesar de la cual, se presentan con alta recurrencia a largo plazo.

ABSTRACT Introduction: Adenoid Cystic Carcinoma is a neoplasm originating in exocrine glands throughout the body, mainly in the major salivary glands. In the nasal cavity and paranasal sinuses is rare and characterized by a high frequency of recurrence and distant metastasis after remission and despite its treatment. Aim: To describe characteristics of a series of patients with operated adenoid cystic carcinoma of the nasal cavity and paranasal sinuses. Material and method: Descriptive-retrospective study. Period January 2012 and January 2017. The information was obtained from the electronic tabs of the Las Condes Clinic. It describes origin, age and sex, clinical characteristics, anatomopathological findings, diagnostic methods, treatment, evolution. Results: Total 5 patients operated, 3 women and 2 men. The average age was 63 years, ranging from 33 years to 90 years of age. 100% of the cases without associated factors. The most frequent clinical presentation was unilateral nasal obstruction, followed by facial pain. 2 patients presented with initial diagnosis, 2 due to recurrence and 1 due to persistence. In 3 cases the tumor originates from the maxillary sinus and in 2 in the ethmoidal sinus. All had advanced disease, stage IV and III. 3 patients were histologically low grade and 2 high grade. In all 5 cases, the primary treatment was surgical, 60% endoscopic, 20% open and 20% combined. In 4 cases, postoperative radiotherapy was used and in 3 of this concomitant chemotherapy. All are without signs of tumor recurrence in the last control. Conclusion: Adenoid Cystic Carcinoma of the nasal cavity and paranasal sinuses is quite infrequent; its incidence is less than 1/100,000 cases per year. It is more common in women between 40 and 50 years. It is identified more with its origin in the maxillary sinus (50%) and cribriform pattern. Clinically it presents in advanced stages since at an early stage, it is asymptomatic or it presents nonspecific inflammatory clinic. Diagnosis is performed with biopsy supplemented with imaging. The most commonly used treatment is endoscopic or open surgery associated with postoperative radiotherapy, despite which, they present with high recurrence in the long term.

Manejo endoscópico de osteoma etmoidal con extensión orbitaria: a propósito de un caso / Endoscopic manegement of frontoethmoidal osteoma: on case subject

El osteoma es el tumor más frecuente de los senos paranasales, habitualmente asintomático debido a su lento crecimiento, sin embargo, pueden desarrollarse síntomas dependiendo del tamaño, localización y extensión, con potencial compromiso de órbita y cerebro. La cirugía está indicada en casos sintomáticos pudiendo realizarse abordaje externo, endoscópico o combinado. Presentamos un caso de osteoma etmoidal con compromiso orbitario resuelto, manejado por medio de la cirugía endoscópica nasal, con apoyo de navegación.

The osteoma is the most common tumor of the paranasal sinuses, is usually asymptomatic because of their slow growth, however, may develop symptoms depending on the size, location and extent, with potential compromise of orbit and brain. Surgery is indicated in symptomatic cases, with external, endoscopic or combined approach. We present a case of ethmoidal osteoma with orbital involvement managed by endoscopic image guided surgery.

Resección de osteoma fronto-naso-maxilar: desafío anatómico con asistencia de estereolitografía y sistema piezoeléctrico / Resection of fronto-naso-maxillary osteoma: anatomic challenge assisted with stereolithography and piezoelectric system

Los osteomas fronto-etmoidales son los tumores benignos más frecuentes de los senos paranasales, pudiendo evolucionar con complicaciones por compresión de estructuras adyacentes. Se presenta el caso de una paciente de 63 años de edad, que consultó por aumento de volumen nasofrontal, cefalea y diplopía. Tras el examen clínico, se evidenció una asimetría facial con lateralización del bulbo ocular derecho y exoftalmo. La tomografía de los senos paranasales mostró una lesión que ocupa parcialmente el seno frontal, etmoidal y parte de la cavidad nasal. Los cuidados anatómicos y planificación quirúrgica se desarrollaron en un modelo estereolitográfico mientras que la cirugía de exéresis total se realizó con ayuda del sistema piezoeléctrico. El examen histológico confirmó el diagnostico de osteoma. Se obtuvo una reconstrucción exitosa, recuperando totalmente simetría y función ocular.

Fronto-ethmoidal osteomas are the most frequent benign tumors of the paranasal sinuses, and may evolve with complications by compression of adjacent structures. The case is a 63-year-old female patient, who consulted about increased nasofrontal volume, headache and diplopia. After the clinical exam, she presented a facial asymmetry with lateralization of the right eyeball and exophthalmus. Computed tomography of the sinuses showed a lesion that partially occupies the frontal sinus, ethmoidal sinus and part of the nasal cavity. The anatomical care and surgical planning was developed in a stereolithographic model while the total excision surgery was performed with the help of the piezoelectric system. Histological examination confirmed the diagnosis of osteoma. A successful reconstruction is obtained, fully recovering symmetry and ocular function.

Utilidad de imagen de banda estrecha en un caso de melanoma mucoso de septum nasal / Utility of narrow band Imaging in a nasal septum mucosal melanoma case

En los últimos años, se está utilizando la fibrolaringoscopía con imagen de banda estrecha (NBI) como técnica novedosa para observar el patrón específico de microvas-cularización de una lesión concreta a evaluar. Es conocida por su utilidad en el diagnóstico de otras lesiones de vías aerodigestivas superiores, fundamentalmente laríngea y digestiva. Los melanomas mucosos son tumores infrecuentes, que suelen localizarse a nivel del área rinosinusaly que comportan un manejo y pronóstico distinto con respecto a los melanomas cutáneos. Se presenta el caso clínico de una paciente mujer con anamnesis, exploración y fibrolaringoscopía con imagen de banda estrecha, compatible con melanoma mucoso de fosa nasal izquierda. El tratamiento realizado fue quirúrgico, sin necesidad de tratamiento coadyuvante, y no presenta evidencia de enfermedad al año postseguimiento.

In recent years, it is being used fibrolaryngoscopy with narrowband image (NBI) as a novel technique to observe the specific pattern of microvasculature of a particular lesion. NBI is known for its usefulness in the diagnosis of other lesions of the upper aerodigestive tract, (primarily laryngeal and digestive lesions). Mucosal melanomas are rare tumors, which are usually located at the level of rhino-sinusal area and involving a different prognosis and management regarding cutaneous melanomas. We report a female patient case with anamnesis, clinical examination and NBI compatible with mucosal melanoma of left nostril. Surgical treatmentwas performed without adjuvant therapy, and there is no evidence of disease at one year post-monitoring.

Schwannoma nasal / Nasal Schwannoma

Introdução: O Schwannoma é uma neoplasia benigna com origem nas células de Schwann presentes na bainha de nervos autossômicos periféricos. Localiza-se cranial ou extracranial. Na cabeça e pescoço constitui 30 a 45% dos casos extracraniais, sendo que 4% deles estão localizados no nariz e seios paranasais. Relato do caso: Paciente feminina, 37 anos, realizou tomografia de crânio após queda para investigar trauma cranioencefálico. Esta identificou velamento de seios paranasais à esquerda. O achado motivou a investigação da hiposmia e obstrução nasal à esquerda de longa data. À rinoscopia anterior, observou-se lesão expansiva polipoide ocupando a fossa nasal, não permitindo a passagem do nasofibroscópio. Tomografia computadorizada evidenciou lesão ocupando totalmente a fossa nasal esquerda, velamento do seio maxilar e células etmoidais anteriores e posteriores à esquerda. Realizou-se exérese endoscópica da lesão, que tinha provável origem no recesso esfenoetmoidal. A biópsia transoperatória com congelação observou lesão inflamatória. A confirmação do diagnóstico de Schwannoma foi feita pelo anatomopatológico e imuno-histoquímica, que evidenciou alta reatividade à proteína S-100. Discussão: As lesões intranasais unilaterais apresentam inúmeras possibilidades diagnósticas. Os sintomas são inespecíficos e os exames de imagem pouco esclarecedores. Neste relato, a tomografia sugeriu pólipo de Killian ou papiloma invertido pela unilateralidade da lesão. Porém, as características histopatológicas e a imunorreatividade à proteína S-100 auxiliaram na confirmação diagnóstica. Conclusão: O Schwannoma intranasal faz parte do diagnóstico diferencial das lesões intranasais. O exame histopatológico e a imunohistoquímica são de extrema importância para confirmação diagnóstica frente à inespecificidade dos sintomas e achados radiológicos (AU)

Introduction: Schwannoma is a benign neoplasm originating in the Schwann cells present in the sheath of peripheral autosomal nerves. It is located cranially or extracranially. In the head and neck it comprises 30 to 45% of extracranial cases, 4% of which located in the nose and paranasal sinuses. Case report: A 37-year-old female patient underwent skull tomography after a fall to investigate cranioencephalic trauma. This identified veiling of paranasal sinuses on the left. The finding motivated the investigation of long-standing hyposmia and nasal obstruction on the left. At anterior rhinoscopy, an expansive polypoid lesion occupying the nasal fossa was observed, not allowing the nasofibroscope to pass. Computed tomography revealed a lesion totally occupying the left nasal fossa, maxillary sinus veil, and anterior and posterior ethmoid cells on the left. Endoscopic excision of the lesion that was likely to originate in the sphenoethmoidal recess was performed. Intraoperative biopsy with freezing showed an inflammatory lesion. Confirmation of the Schwannoma diagnosis was made by pathology and immunohistochemistry that showed high reactivity to S-100 protein. Discussion: Unilateral intranasal lesions present numerous diagnostic possibilities. The symptoms are nonspecific and imaging exams are not very elucidating. In this report, CT scan suggested a Killian polyp or inverted papilloma due to the unilaterality of the lesion. However, the histopathological characteristics and immunoreactivity to the S-100 protein aided in the diagnostic confirmation. Conclusion: Intranasal Schwannoma is part of the differential diagnosis of intranasal lesions. Histopathological examination and immunohistochemistry are extremely important for diagnostic confirmation in the face of the non-specificity of symptoms and radiological findings (AU)

Pseudotumor inflamatorio nasosinusal: reporte de un caso / Inflammatory pseudotumor sinonasal: a case report / Pseu-dotumor inflamatório nasossinusal: relatório de um caso

El pseudotumor inflamatorio (PTI) es una entidad no neoplásica, de etiología desconocida, caracterizada por la proliferación de un infiltrado inflamatorio policlonal sobre un estroma de tejido conjuntivo. Pueden ser definidos como lesiones que clínica y radiológicamente simulan neoplasias. El pseudotumor inflamatorio puede causar dolor facial, obstrucción nasal, exoftalmos y discapacidad visual y con frecuencia causa la erosión y destrucción ósea. Los corticoesteroides, la radioterapia y la cirugía se han utilizado como modalidades de tratamiento, solos o en combinación.

The inflammatory pseudotumour (IPT) is a nonneoplastic entity of unknown origin, and is characterised by a proliferation of connective tissue and a polyclonal inflammatory infiltrate. May be defined as lesions that clinically and radiologically simulate neoplasms. Inflammatory pseudotumour sometimes causes facial pain, nasal obstruction, exophthalmos and visual impairment, and often causes bone erosion and destruction. Corticosteroids, radiotherapy and surgery have been used as treatment modalities either on their own or in combination.

O pseudo-tumor inflamatório (PTI) é uma entidade não neoplásica de etiologia desconhecida, caracterizada pela proliferação de um infiltrado inflamatório policlonal em um estroma do tecido conjuntivo. Podem ser definidos como lesões que simulam neoplasias clínica e radiologicamente. O pseudo-tumor Inflamatório pode causar dor facial, obstrução nasal, exoftalmia (olhos saltados), deficiência visual e freqüentemente provoca o desgaste e a destruição óssea. Os cortiçoesteroides, a radioterapia e a cirurgia têm sido usadas como modalidades de tratamento, por si só ou em combinação.

Glomangiopericitoma: reporte de un caso / Glomangiopericytoma: a case report

El glomangiopericitoma es un tumor nasosinusal extremadamente raro, más frecuente en pacientes de edad avanzada y en mujeres. Si bien su etiopatogenia es desconocida, se reconocen ciertos factores predisponentes tales como hipertensión arterial, embarazo, trauma y uso de corticoides. Para su diagnóstico, resulta necesario recurrir tanto a estudio por imágenes como a la histología y técnicas de inmunohistoquímica. Microscópicamente se caracteriza por un prominente crecimiento perivascular de células uniformes ovales o fusiformes, dispuestas en fascículos cortos intercalados con capilares de diámetros variables ramificados en "asta de ciervo" y presenta inmuno-rreactividad positiva para actina, factor XIII-A y vimentina principalmente. Clínicamente este tumor presenta un comportamiento generalmente benigno, pero con una elevada tasa de recurrencia. Presentamos el caso de una mujer de 71 años, con antecedentes de hipertensión arterial, que acudió a nuestro centro por rinorrea purulenta y epistaxis unilateral derecha de 5 años de evolución. Al examen se observa masa polipoidea en fosa nasal derecha con abundante vascularización, sin otros hallazgos al examen físico. La lesión es resecada en su totalidad mediante cirugía endoscópica. El estudio histológico e inmunohistoquímico son compatibles con glomangiopericitoma. La paciente evoluciona con remisión de su sintomatología y a los dos meses desde la resección no ha presentado evidencias de recurrencia.

Glomangiopericytoma is an extremely rare sinonasal tumor, more common among elderly and women. Although its etiology and pathogeny are unknown, there are certain predisposing factors, such as arterial hypertension, pregnancy, trauma and corticosteroids. To diagnose this tumor, it is necessary imaging, histopathologic and inmunohistochemical studies. At the microscopic study, it is characterized by a prominent perivascular growth of oval-shaped or fusiform cells, arranged in short fascicles separated by capillary vessels of variable diameters, with staghorn-like ramifications, and lmmunohistochemistry positive mainly for actin, XIII-A factor and vimentin. Clinically, this tumor has a generally benign behavior, but with high recurrence percentage. We present the case of a 71 years old woman, with history of hypertension, who present in our center with a 5 years history of purulent rhinorrhea and right unilateral epistaxis, at the physical exam there is a polypoid mass in the right nasal fossa with rich vascularization, with no other findings. This tumor was resected entirely with endoscopic surgery. Histology and immunohistochemistry were compatible witch gomangiopericytoma. The patient evolve with remission of her symptoms and with no signs of recurrence at two-month follow-up.

Abordaje endonasal endoscópico para neoplasias malignas de la base craneal / Endonasal endoscopic approach for malignant cranial base neoplasm

Introduction: Malignant cranial base neoplasm constitutes a heterogeneous group of lesions with common seat in this localization. In the last years endonasal endoscopic approach has been more used. Methods: It was carried out a descriptive retrospective study of the database of the Service of Neurosurgery of "Hermanos Ameijeiras" Hospital between September 2009 and September 2015 with the objective of determining the results of endoscopic treatment to these lesions. Results: They were treated a total of 12 patients with wicked lesions of the cranial base inside. Six of them corresponded to anterior cranial base: 5 carcinomas (2 indiferenciate carcinoma, 2 carcinomas of scamous cells and 1 adenocarcinoma) and a metastases of renal carcinoma. Other six corresponded to cordoma (3 patients) and hematopoyetic neoplasm (2 plasmocitomas and 1 linfoma). The age average was of 51,1 years with a masculine-feminine relationship of 1,1:1. Conclusions: Surgical resection according to oncological principles can be used with endoscopic technics that in fact are associate with less morbility, better vision, betercompartiments access, nasolacrimal system and medial canthal tendon, absence of facial scar, craniectomy and brain retraction.

Introducción: Las neoplasias malignas de la base craneal constituyen un grupo heterogéneo de lesiones con asiento común en esta localización. En los últimos años el abordaje endonasal endoscópico ha cobrado mayor auge. Métodos: Se realizó un estudio retrospectivo descriptivo de la base de datos del Servicio de Neurocirugía del Hospital "Hermanos Ameijeiras" durante el período comprendido entre septiembre de 2009 y septiembre de 2015 con el objetivo de determinar los resultados del tratamiento endonasal endoscópico a estas lesiones. Resultados: Fueron tratados un total de 12 pacientes con lesiones malignas de la base craneal, 6 de la base craneal anterior dentro de los cuales 5 correspondieron a carcinomas (2 carcinomas indiferenciados, 2 carcinomas de células escamosas y 1 adenocarcinoma) y una metástasis de carcinoma renal. De los 6 de fosa posterior 3 presentaron cordomas y 3 lesiones hematopoyéticas (2 plasmocitomas y 1 linfoma). El promedio de edad fue de 51,1 años con una relación masculino-femenino de 1,1:1. Conclusiones: La resección quirúrgica acorde a los principios oncológicos se puede realizar utilizando las técnicas endoscópicas que incluso se asocian a menor morbilidad, mayor visión, mejor acceso a determinados compartimentos, preservación del sistema nasolacrimal, del tendón cantal medial, ausencia de cicatriz facial, de craneotomía y de retracción cerebral.

Manejo endoscópico de papiloma invertido nasosinusal: experiencia de 15 años en Hospital Clínico Regional de Concepción / Endoscopic management of sinonasal inverted papilloma: 15 years experience in Concepción Clinical Hospital

Introducción: El papiloma invertido es uno de los tumores benignos más frecuentes de la región nasosinusal. Los principales problemas en su manejo son la recurrencia y transformación maligna. Su tratamiento es la extirpación quirúrgica. Objetivos: Describir y analizar las caracterfsticas cínicas y epidemiológicas, así como el resultado del tratamiento endoscópico practicado en el Hospital Clínico Regional de Concepción. Material y método: Se realizó un estudio retrospectivo, descriptivo, de todos los pacientes con diagnóstico de papiloma invertido nasosinusal operados vfa abordaje endoscópico entre los años 1997 y 2012. Se analizaron variables epidemiológicas y clínicas, incluyendo análisis de las recurrencias y transformación/asociación a cáncer. Resultados: Se encontraron 57 casos de papiloma invertido nasosinusal, 55 de los cuales se abordaron endoscópicamente, 65,5% hombres, 54,7 años en promedio de edad. La obstrucción nasal fue el principal síntoma. La mayor cantidad se concentró en estadio 2 (47,3%) y 3 (32,7%) de Krouse. Complicaciones relevantes 7,8%, las cuales fueron resueltas sin secuelas. Seguimiento promedio de 4,1 años, con una recurrencia de 5 (9,1%) casos, una persistencia (1,8%). Asociación con carcinoma escamoso en el 5,5% de los casos. Conclusión: La experiencia con la resección endoscópica en el papiloma invertido nasosinusal ha demostrado ser efectiva en nuestro medio, con baja tasa de complicaciones y recurrencias.

Introduction: The inverted papilloma is one of the most common benign tumors of the sinonasal region. The main problems in their management are the recurrence and malignant transformation. Their treatment is surgical removal. Aim: To describe and analyze the clinical and epidemiological characteristics, as well as the results of endoscopic treatment in a tertiary hospital. Materials and methods: A retrospective, descriptive study of all patients with diagnosis ofsinonasal inverted papilloma operated by endoscopic approach between 1997 and 2012 was carried out. Epidemiological and clinical variables were analyzed, including analysis of recurrences and transformation to / association with cancer. Results: 57 cases of sinonasal inverted papilloma were found, 55 of which were endoscopically resected, 65.5% male, 54.7 years old on average. Nasal obstruction was the main symptom. Most of them were in Krouse stage 2 (47.3%) and 3 (32.7%); 7.8% of major complications, which were resolved without sequels. The follow up average was 4.11 years, with a recurrence of 5 (9.1%) cases, and (1.8%) persistence. Association with squamous-cell carcinoma was found in 5.5% of cases. Conclusion: The clinical and epidemiological characteristics do not differ greatly from those reported in the literature. Experience with endoscopic resection in sinonasal inverted papilloma has proven effective in our clinical practice, with a low rate of complications and recurrences, this approach currently being the first choice in most cases.

Osteoma frontoetmoidal, otras aplicaciones del Draf III, a raíz de un caso clínico / Frontoethmoidal osteoma, other applications of Draf III on the grounds of a clinical case

Presentamos el caso de una mujer de 57 años, que consulta tras notar un aumento de volumen pétreo y de lento crecimiento en el reborde orbitario medial derecho, asociado a obstrucción nasal, anosmia y descarga posterior. La evaluación clínico radiológica evidenció un gran osteoma frontoetmoidal derecho con compromiso orbitario y de la lamela lateral de la lámina cribosa, y además, una rinosinusitis crónica con pólipos. Se realizó una resección endoscópica del tumor por medio de un Draf III y una cirugía endoscópica funcional del resto de las cavidades perinasales para el tratamiento de la rinosinusitis crónica. Describimos el caso y discutimos sus aspectos relevantes.

We report the case of a 57 year-old woman consulting because of a slow growing solid tumor in the right medial orbital rim associated with nasal obstruction, anosmia and posterior nasal drip. Clinical and radiological evaluation evidenced a large right frontoethmoidal osteoma involving the orbit and thelateral lamella of the cribriform plate, and chronic rhinosinusitis with polyps. A Draf III was performed to remove the osteoma and a bilateral functional endoscopic sinus surgery of the remaining sinuses was performed to treat thechronic rhinosinusitis. We described the case and discuss its relevant aspects.