Angiofibroma nasofaríngeo juvenil: estudio de 6 casos en el Hospital Carlos van Buren de Chile / Juvenile nasopharyngeal angiofibroma: study of 6 cases in the Carlos van Buren Hospital in Chile

Introducción: El angiofibroma nasofaríngeo juvenil es una neoplasia vascular benigna y localmente agresiva, que se desarrolla casi exclusivamente en adolescentes de sexo masculino. Sus manifestaciones clínicas habituales son epistaxis y obstrucción nasal. Objetivo: Dar a conocer la experiencia en el Servicio de Otorrinolaringología del Hospital Carlos van Buren de pacientes con angiofibroma operados por vía endoscópica y abierta entre los años 2008 y 2015. Material y método: Estudio descriptivo retrospectivo de pacientes con diagnóstico de angiofibroma nasofaríngeo juvenil que ingresaron al Servicio de Otorrinolaringología del Hospital Carlos van Buren entre los años 2008 y 2015. Resultados: Hubo un total de 6 casos. La edad de los pacientes fluctuó entre los 12 y los 29 años, el 100% fueron pacientes masculinos. Los síntomas de presentación más frecuente fueron epistaxis recurrente y obstrucción nasal, presentes en 5/6 de los pacientes. La totalidad de los casos fueron estudiados con TC, RM y angiografía. El manejo en todos los casos fue con embolización endovascular 48 horas previo a la resección. Conclusión: Los resultados obtenidos se correlacionan con la literatura. El abordaje endoscópico sigue siendo de elección. Este tiene como ventajas menores pérdidas sanguíneas intraoperatorias, una disminución del número días de hospitalización y las tasas de recurrencia.

Introduction: Juvenile nasopharyngeal angiofibroma is a benign vascular neoplasm, locally aggressive that develops almost exclusively in adolescent males. Its usual clinical manifestations are epistaxis and nasal obstruction. Aim: To show the experience in the Department of Otolaryngology Hospital Carlos van Buren of angiofibromas operated by endoscopic and open surgery between the years 2008 and 2015, and review of the literature. Material and Method: Retrospective descriptive study of patients diagnosed with juvenile nasopharyngeal angiofibroma admitted in the Department of Otolaryngology Hospital Carlos van Buren, Valparaiso between 2008 and 2015. Results: A total of 6 cases were identified. The age of patients ranged from 12 to 29 years. The most common presenting symptoms were recurrent epistaxis and nasal obstruction, both present in 5/6 of patients. All the cases were studied with CT, MRI and angiography. All cases had pre-surgical endovascular embolisation48 hours prior to excision. Conclusions: The results correlate with those seen in the literature. The endoscopic approach is the better option, because of its lower intraoperative blood loss, days of hospitalization and recurrence.

Molecular pathogenesis of juvenile nasopharyngeal angiofibroma in brazilian patients.

Juvenile nasopharyngeal angiofibroma (JNA) is a vascular tumor of the nasopharynx that accounts for 0.5% of all cancers of the head and neck. It primarily affects males aged 14-25 years. Of the many genes that mediate the development of JNA, GSTM1 has been most frequently associated with this vascular tumor. The loss of expression of GSTM1 (null genotype) is linked to the development of these tumors. The aim of this cross-sectional case study was to examine the prevalence of the GSTM1-null genotype in Brazilian patients with JNA. DNA was extracted from the leukocytes of blood samples from 10 patients. GSTM1 genotypes were analyzed using a PCR-based assay that was designed to identify the wild-type allele of GSTM1. All 10 patients (100%) were males, with a mean age of 17.8 years. The null genotype for GSTM1 was noted in 4 patients (40%)-1 (10%) at Fisch stage I, 1 (10%) at stage III, and 2 (20%) at stage II. No patient with this genotype had stage IV disease. There was no correlation between Fisch classification and GSTM1 genotype (P = .5695). The correlation between age at diagnosis and GSTM1 genotype was not significant (P = .728). The present findings indicate that there is evidence of an association between the GSTM1-null genotype and JNA in this studied Brazilian population.

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.

Orbital involvement in juvenile nasopharyngeal angiofibroma: prevalence and treatment.

PURPOSE: To assess the prevalence of orbital invasion by juvenile nasoangiofibroma and to discuss its surgical treatment. METHODS: A retrospective review of the medical records and tomographic scans of a case series of 19 patients with juvenile nasoangiofibroma was performed. All scans were reviewed by a radiologist and an orbital surgeon. The presence of the tumor was assessed in the pterygopalatine fossa, nasal cavity, nasopharynx, paranasal sinuses, inferior orbital fissure, orbit, and middle cranial fossa. RESULTS: The most common structures invaded were pterygopalatine fossa (100%), nasal cavity (94.7%), sphenoid sinus (84.2%), and nasopharynx (73.7%). The orbit was invaded in 6 (31.6%) patients. In 5 of these patients, the tumor extended in the orbit through the inferior orbital fissure. Four patients with orbital invasion were successfully operated with the Le Fort I approach. CONCLUSIONS: Orbital involvement is relatively common in the setting of juvenile nasoangiofibroma extension. The main route of orbital invasion is the inferior orbital fissure. The Le Fort I osteotomy is an adequate approach for managing juvenile nasoangiofibroma when it invades the orbit.

The biology and pathology of selected skull base tumors.

This paper describes the pathobiology of some of the more common skull base tumors. In addition to clinicopathologic features, emphasis is placed upon methods of diagnosis utilizing immunoperoxidase stains and molecular markers that may or may not impact upon prognosis.