Experiencia en cirugía endoscópica de angiofibroma nasofaríngeo juvenil / Endoscopic surgery experience of juvenile nasopharyngeal angiofibroma

Resumen Introducción: El angiofibroma nasofaríngeo juvenil (ANJ) es un tumor benigno poco frecuente, altamente vascularizado y localmente agresivo, encontrado casi exclusivamente en pacientes masculinos adolescentes. Se presentan con epistaxis recurrente y obstrucción nasal. Objetivo: Presentar la experiencia en el tratamiento quirúrgico endoscópico exclusivo para los ANJ del equipo de rinología del Hospital del Salvador. Material y Método: Estudio descriptivo retrospectivo de corte transversal con revisión de fichas clínicas entre enero de 2011 a junio de 2017 con tratamiento quirúrgico endoscópico exclusivo para ANJ. Resultados: 16 pacientes con edad promedio de 17,2 años, 81% se presentó con obstrucción nasal y epistaxis. Todos fueron embolizados 48 o 24 horas previo a la cirugía. El tiempo quirúrgico promedio fue de 199 minutos. El sangrado estimado fue de 831 ml en promedio, con sólo un paciente con requerimientos de transfusión. El 71% no requirió taponamiento nasal anterior. El requerimiento de hospitalización fue de 4,6 días. Sólo un paciente ha tenido recurrencia al año de control. Conclusión: Los resultados en pacientes con ANJ tratados en el Hospital del Salvador reafirman el éxito de la técnica endoscópica exclusiva versus abordajes abiertos convencional, ya que presentan mejores resultados.

Abstract Introduction: The juvenile nasopharyngeal angiofibroma (ANJ) is a benign, infrequent and highly vascularized tumor. It is locally aggressive, found almost only in adolescent male patients. The classical clinical presentation is recurrent epistaxis and nasal obstruction. Aim: To review the experience of exclusive endoscopic surgery for patients with ANJ by the rhinology team of Hospital del Salvador. Material and Method: Retrospective, cross sectional, descriptive study with research of medical records of patients with exclusive endoscopic surgery treatment between January 2011 and June 2017. Results: 16 patients with a mean age of 17.2 years, 81% had nasal obstruction and epistaxis. All of them were embolized 48 to 24 hours prior surgery. Mean surgical time was 199 minutes. Estimated bleeding was 831 ml among all patients, with only one requiring blood transfusions, while 71% did not need nasal packing. Average length of hospital stay was 4.6 days. Only one patient had a recurrence after one year of surgery. Conclusion: Results of patients with ANJ treated in Hospital del Salvador reassert the success of the exclusive endoscopic surgery versus traditional open approaches, showing better results.

Master Regulators of Epithelial-Mesenchymal Transition and WNT Signaling Pathways in Juvenile Nasopharyngeal Angiofibromas.

Juvenile nasopharyngeal angiofibroma (JNA) is a rare fibrovascular benign tumor showing an invasive growth pattern and affecting mainly male adolescents. We investigated the role of epithelial-mesenchymal transition (EMT) and WNT signaling pathways in JNA. Gene expression profiles using nine JNA paired with four inferior nasal turbinate samples were interrogated using a customized 2.3K microarray platform containing genes mainly involved in EMT and WNT/PI3K pathways. The expression of selected genes (BCL2, CAV1, CD74, COL4A2, FZD7, ING1, LAMB1, and RAC2) and proteins (BCL2, CAV1, CD74, FZD7, RAF1, WNT5A, and WNT5B) was investigated by RT-qPCR (28 cases) and immunohistochemistry (40 cases), respectively. Among 104 differentially expressed genes, we found a significantly increased expression of COL4A2 and LAMB1 and a decreased expression of BCL2 and RAC2 by RT-qPCR. The immunohistochemistry analysis revealed a low expression of BCL2 and a negative to moderate expression of FZD7 in most samples, while increased CAV1 and RAF1 expression were detected. Moderate to strong CD74 protein expression was observed in endothelial and inflammatory cells. A significant number of JNAs (78%) presented reduced WNT5A and increased WNT5B expression. Overall, the transcript and protein profile indicated the involvement of EMT and WNT pathways in JNA. These candidates are promising druggable targets for treating JNA.

Fibroangioma nasofaríngeo juvenil. Experiencia en 89 pacientes / Juvenile nasopharyngeal angiofibroma. An experience of 89 patients

El fibroangioma nasojuvenil (FANJ) es un tumor benigno, localmente invasivo, que se presenta en varones púberes y adolescentes. Se manifiesta clínicamente por insuficiencia ventilatoria nasal progresiva y epistaxis. Con el objetivo de describir las características clínico quirúrgicas se realizó un estudio observacional, retrospectivo, transversal de los FANJ intervenidos quirúrgicamente en el período enero 2000 a diciembre 2017 en el Hospital de Pediatría Juan P. Garrahan. Resultados: se incluyeron 89 pacientes con diagnóstico de fibroangioma nasojuvenil intervenidos quirúrgicamente. El 71% de los pacientes menores de 14 años. El síntoma predominante fue epistaxis en el 91% de los casos. El 80,1% (72/89) estaban incluidos en los estadios III y IV de Chandler presentando digitaciones a fosa pterigomaxilar y senos paranasales. El 29,2% (26/89) de los casos presentaba invasión a endocráneo. En el 96% (86/89) se realizó embolización prequirúrgica de las ramas nutricias del tumor. Los abordajes quirúrgicos más utilizados fueron: la vía transantral en el 43,8% (39/89), abordaje de Le Fort 1 en el 21,3% (19/89), endoscópico en el 12,3% (11/89), abordaje combinado con neurocirugía en el 12,3% (11/89) de los pacientes. La recurrencia fue de 33,7% y no hubo pacientes fallecidos. Conclusiones: El fibroangioma nasojuvenil es una patología tumoral que requiere para su resolución un equipo multidisciplinario en instituciones con infraestructura de alta complejidad. El abordaje quirúrgico debe seleccionarse de acuerdo a la localización y extensión tumoral, la efectividad de la embolización previa, la edad del paciente y la experiencia del equipo quirúrgico (AU)

Juvenile nasopharyngeal angiofibroma (JNA) is a benign, locally invasive tumor, occurring in pubertal and adolescent males. Clinically the tumor manifests with progressive nasal obstruction and epistaxis. With the aim to describe the clinical and surgical features, an observational, retrospective, cross-sectional study was conducted in patients with JNA who underwent surgery at Hospital de Pediatría Juan P. Garrahan between January 2000 and December 2017. Results: Overall, 89 patients diagnosed with JNA who underwent surgery were included; 71% were under 14 years of age. The main symptom was epistaxis occurring in 91% of the cases. Of all patients, 80.1% (72/89) were in Chandler stages III and IV with extension into the pterygomaxillary fossa and paranasal sinuses. Intracranial invasion was found in 29.2% (26/89) of the cases. In 96% (86/89), preoperative embolization of the feeding branches of the tumor was performed. The most commonly used surgical approaches were: The transantral approach in 43.8% (39/89), Le Fort 1 in 21.3% (19/89), endoscopic approach in 12.3% (11/89), and an approach combined with neurosurgery in 12.3% (11/89) of patients. Recurrence rate was 33.7% and none of the patients died. Conclusions: Management of JNA a multidisciplinary team at a tertiary care institution. The surgical approach should be selected according to tumor location and extension, effectiveness of previous embolization, the patient age, and expertise of the surgical team (AU)

Resection of a juvenile nasoangiofibroma by Le Fort I osteotomy: Experience with 40 cases.

PURPOSE: The aim of this study was to determine the rate of success and complications of juvenile nasoangiofibroma resection by Le Fort I osteotomy. MATERIAL AND METHODS: Data were obtained from the medical records of 40 patients with a diagnosis of juvenile nasoangiofibroma confirmed by anatomopathological examination. All tumors were resected by Le Fort I osteotomy between 1983 and 2010. The data obtained were gender, age, symptoms, sites of invasion, preoperative embolization, routes of surgical access, duration of surgery, complications, need for transfusion, relapses, and follow-up time. RESULTS: All patients were male, ranging in age from 7 to 27 years. The most common symptom was nasal obstruction, and central nervous system (CNS) invasion was present in 27.5% of cases. Craniotomy was associated with Le Fort I osteotomy in only one case. The mean duration of surgery was 216 min. Complications occurred in 15% of cases, with intraoperative bleeding being the most frequent one. Relapses occurred in 5% of cases. The mean follow-up was 48.8 months. CONCLUSION: Exclusively surgical treatment by Le Fort I access proved to be a safe and effective method for the treatment of nasoangiofibromas, permitting the removal of tumors even in patients with extension to the CNS, with a low rate of complications and relapses.

Angiofibroma juvenil nasofaríngeo / Juvenile nasopharyngeal angiofibroma

Se presenta el caso de un angiofibroma juvenil nasofaríngeo, en un paciente del consultorio médico No. 9, que pertenece al Policlínico Integral Docente Carlos Manuel Portuondo de Marianao. Se reconoce la importancia de realizar una historia clínica detallada, así como un minucioso examen físico, por el médico de familia y el otorrinolaringólogo del área de salud, que incluyó la rinoscopia posterior, para poder llegar al diagnóstico de esa patología, y realizar la extirpación precoz del angiofibroma, mediante el proceder quirúrgico. Se concluyó que el diagnóstico clínico se correspondió con el histopatológico, y que la conducta quirúrgica temprana es resolutiva en la afección

It is presented a case of juvenile nasopharyngeal angiofibroma in a patient with this pathology, from the clicial practice No. 9, in Comprehensive Teaching Polyclinic Carlos Manuel Portuondo in Marianao. It recognizes the importance of a thorough clinical history and careful physical examination by the family physician and the otolaryngologist in this health area, including a posterior rhinoscopy, to diagnose this disease and to achieve early removal of the angiofibroma, by a surgical procedure. It was concluded that the clinical diagnosis corresponded to the histopathological diagnosis, and that early surgical treatment is resolute in this condition

Nasoangiofibroma juvenil: una revisión actualizada del diagnóstico, clasificación y tratamiento / Juvenile nasopharingeal angiofibroma: a current review of diagnosis, clasification and treatment

Objetivo: Realizar una recopilación de datos acerca del nasoangiofibroma juvenil (NAF) permitiendo aclarar conceptos en cuanto al diagnóstico, clasificación y aproximación del manejo. Diseño: Revisión de la literatura. Método: Se recolectaron datos históricos, anatómicos, etiológicos, las múltiples clasificaciones adoptadas hasta el momento, los distintos tratamientos propuestos y las indicaciones sugeridas según la Rinología actual. Conclusiones: El NAF es un tumor de naturaleza benigna, ampliamente vascularizado, de comportamiento agresivo dada su propiedad de erosión y extensión, con pronóstico favorable cuando su diagnóstico y tratamiento es temprano. La embolización es un aspecto fundamental en el adecuado control vascular e intraquirúrgico de la lesión. El manejo endoscópico hadisminuido la morbilidad, la estancia hospitalaria y las complicaciones generadas por los abordajes externos. Ocasionalmente se requiere de abordajes quirúrgicos combinados o el uso de terapéuticas ablativas adicionales. El nasoangiofibroma juvenil requiere de un manejo interdisciplinario, pues plantea un reto diagnóstico, terapéutico intervencionista y quirúrgico.

Objective: To collect data about Juvenile Nasopharyngeal Angiofibroma (JNA), clarifying concepts about diagnosis, classification and management approach. Design: Review of the literature. Method: Data were collected historical, anatomical, etiological, multiple classifications adopted so far, the various proposed treatments and the indications suggested by the current Rhinology. Conclusions: The NAF is a benign tumor, extensively vascularized, aggressive behavior because of its ownership of erosion and extension, with a favorable prognosis when diagnosis and treatment is early realized. Embolization is a fundamental aspect of proper control and intraoperative vascular injury. Endoscopic treatment has decreased the morbidity, hospital stay and complications caused by external approaches. Occasionally requires combined surgical approaches or the use of additional ablative therapy. The Juvenile angiofibroma requires interdisciplinary management; it poses diagnostic, interventional and surgical challenge therapy.

Nasoangiofibroma juvenil: nuestra experiencia en los últimos 10 años y revisión de la literatura / Juvenile nasopharyngeal angiofibroma: our experience in the last 10 years and review of the literature

Introducción: El nasoangiofibroma juvenil es un tumor benigno con comportamiento de agresividad local, dadas las importantes epistaxis que provoca, la frecuente recidiva local y la complejidad terapéutica que se presenta ante esta patología; se presenta exclusivamente en hombres adolescentes y corresponde al 0,5 por ciento-0,05 por ciento de todos los tumores de cabeza y cuello. Objetivo: Determinar el manejo y las características del paciente con el diagnóstico de nasoagiofibroma juvenil que acude a nuestro servicio. Evaluar necesidad de transfusión sanguínea. Exponer las complicaciones encontradas. Material y método: Se realizó un trabajo retrospectivo observacional de corte transversal de pacientes con diagnóstico de nasoangiofibroma juvenil (NAJ), confirmado por estudios histológicos en la Cátedra de Otorrinolaringología de la Facultad de Ciencias Médicas de la Universidad Nacional de Asunción, desde enero de 1998 hasta septiembre de 2008. Resultados: Con un total de 45, de los cuales 44 recibieron tratamiento quirúrgico. El rango de edad estaba comprendido entre 7 a 34 años, con una mediana de 16 años y un promedio de 17 años, todos de sexo masculino. Se presentaron con mayor frecuencia en estadio Fisch II, rango de evolución sintomática de 1 a 60 meses, con una mediana de 7 meses de evolución de epistaxis recurrente, obstrucción nasal y rinorrea. La mayoría de grupo sanguíneo O Rh+, provenientes del Departamento Central, recibiendo como tratamiento la resección endoscópica. Siete pacientes presentaron recidivas, con una mediana de 7 meses desde el tratamiento. En 58,8 por ciento de los procedimientos quirúrgicos fue necesaria la transfusión de derivados sanguíneos. Conclusión: Los pacientes que acuden al servicio con el diagnóstico de nasoangiofibroma juvenil, son sometidos a tratamiento quirúrgico en la totalidad de los casos. En la mayoría de los casos se requirió algún tipo de transfusión sanguínea. No hubo complicaciones por...

Introduction: The juvenile Nasopharyngeal Angiofibroma is a benign tumor with behavior of local aggressiveness, given the important epistaxis that it provokes, the frequent local recidiva and the therapeutic complexity that one presents before this pathology; he appears exclusively in teen men and corresponds to 0,5 percent-0,05 percent of all the tumors of head and neck. Aim: To determine the managing and the characteristics of the patient with juvenile Nasopharyngeal Angiofibroma diagnosis that comes to our service. To evaluate need of blood transfusion. To expose the opposing complications. Material and method: There realized a retrospective work observacional of patients' transverse court (cut) with diagnosis of juvenile Nasopharyngeal Angiofibroma (NAJ), confirmed by histological studies in the Service of Otolaryngology of the Asuncion's National University, from January, 1998 until September, 2008. Results: With a whole of 45, of which 44 received surgical treatment. The range of age was understood (included) between (among) 7 to 34 years, with a median of 16 years and an average of 17 years, all of masculine sex. Appellants presented with major frequency in estadio Fisch II, range of symptomatic evolution from 1 to 60 months, with a median of 7 months of evolution of epistaxis, nasal obstruction and rinorrea. The majority of blood group O Rh +, from the Central department, receiving as treatment the resection endoscopic. 7 patients presented recidivas, with a median of 7 months from the treatment. In 58,8 percent of the surgical procedures there was necessary the transfusion of blood derivatives. Conclusion: The patients who come to the service with juvenile nasopharyngeal angiofibroma diagnosis, are submitted to surgical treatment in the totality of the cases. In most cases there was needed some type of blood transfusion. There were no complications for the surgical procedure.

Angiofibroma nasofaríngeo juvenil: A propósito de un caso / Juvenile nasopharyngeal angiofibroma: a case presentation

Se presenta el caso de un adolescente de 16 años de edad, raza blanca, del sexo masculino, con antecedentes de salud relativa, que se valoró en la consulta externa del Hospital Pediátrico Provincial Docente "Pepe Portilla" de Pinar del Río, Cuba, por presentar un cuadro obstructivo nasal de 2 años de evolución, que se acompañó en los últimos meses de epistaxis ligeras, además presentaba rinolalia, ronquidos nocturnos, apnea del sueño y buen estado general; al examen físico se encontró en la rinoscopia posterior un tumor rojizo, duro al tacto, no ulcerado, fijo, que ocupa toda la nasofaringe, posteriormente se realizó Tomografía Axial Computarizada, estableciéndose el diagnóstico de Angiofibroma Nasofaríngeo Juvenil y se procedió a la exéresis quirúrgica. Los autores presentan las experiencias derivadas de la atención del caso, y revisan la literatura médica sobre el tema con especial referencia al diagnóstico y tratamiento.

A Caucasian-16 year old boy presenting a relative health history was assessed at an out-patient office in "Pepe Portilla" Pediatric Hospital. Pinar del Rio. Cuba. It was observed a nasal obstruction with 2 years of evolution being associated to light epitasis in the last months as well as rhinolalia, night snores, sleeping apnea and a good general condition. At physical examination it was found a non- ulcer and hard - reddish mass occupying the whole nasopharynx. Later it was performed a CTA that showed a Juvenile Nasopharyngeal Angiofibroma and the surgical procedure was carried out. Authors present the experiences obtained with this case and make a review of the medical literature giving emphasis to diagnosis and treatment.

Tratamento cirúrgico de nasoangiofibroma sem embolização / Surgical Treatment of Non-embolized Patients with Nasoangiofibroma

Nasoangiofibroma juvenil (NAFJ) é um tumor incomum que se localiza na região do forame esfenopalatino. A cirurgia combinada à embolização pré-operatória tem sido a opção terapêutica mais empregada nos pacientes com NAFJ sem invasão intracraniana. O objetivo desse estudo é avaliar a viabilidade do tratamento cirúrgico do nasoangiofibroma em pacientes estágios I- III de Fisch, sem uso de embolização pré- operatória. MATERIAL E MÉTODO: Estudo descritivo, retrospectivo, utilizando-se dados de revisão de prontuário de quinze pacientes com NAFJ estágio I a III de Fisch submetidos à cirurgia sem embolização pré-operatória, entre os anos de 2000 e 2005. RESULTADOS: Dos quinze pacientes, sete pacientes foram submetidos à cirurgia endoscópica, quatro via transmaxilar, três via endoscópica e transmaxilar e um via transmaxilar e transpalatina. Seis pacientes necessitaram de hemotransfusão no intra-operatório, com média geral de 1.3 bolsa/paciente. Nenhum caso de mortalidade ou morbidade significativa foi registrado. Onze dos quinze pacientes foram acompanhados por tempo médio de doze meses com taxa de recidiva de 27 por cento. Quatro pacientes perderam seguimento. CONLUSÃO: A ressecção de NAFJ classes I- III foi realizada com segurança em pacientes não-embolizados, com taxa de sangramento intraoperatório, ocorrência de complicações e taxa de recorrência próximas dos valores pesquisados na literatura para pacientes embolizados.

Juvenile nasopharyngeal angiofibroma (JNA) is an uncommon tumor of the sphenopalatine foramen. Surgery combined with preoperative embolization has been the treatment of choice for JNA patients without intracranial invasion. This study aims to assess the viability of surgically treating non-embolized patients with JNA (types I-III according to Fisch). MATERIAL AND METHOD: This is a retrospective, descriptive study based on the medical records of 15 patients with histologically confirmed JNA (Fisch’s types I- III), who underwent surgical treatment without pre-op embolization in our institution between 2000 and 2005. RESULTS: Seven of the fifteen patients were approached endoscopically, four through the transantral approach, three were treated with the combined transmaxillary and endoscopic approach, and one with the combined transmaxillary and transpalatal approach. Six patients required intraoperatory blood transfusion, averaging volumes of 1.3 unit/patient. There were no cases of death or significant morbidity. Eleven of the fifteen patients were followed for an average of twelve months and 27 percent of them relapsed. Four patients did not comply with the follow-up scheme. CONCLUSION: Resection of JNF types I-III was safely completed in non-embolized patients. The observed levels of intraoperative bleeding, occurrence of complications, and rates of recurrence were close to those seen in embolized patients as found in the literature.