Locally Advanced Staged Juvenile Nasopharyngeal Angiofibroma Treated with Endoscopic Endonasal Approach and Angio-embolization at a Tertiary Institution in Jamaica / Angiofibroma nasofaríngeo juvenil en etapa localmente avanzada tratado con un enfoque endonasal endoscópico y angioembolización en una institución terciaria en Jamaica

ABSTRACT Locally advanced staged juvenile nasopharyngeal angiofibroma (JNA) traditionally treated with external approaches has been associated with significant morbidities such as blood loss with subsequent blood transfusions, scars, impaired speech, poor swallowing, recurrence and prolonged hospital stays. This case report describes our initial experience in a patient with a locally advanced JNA treated with endonasal endoscopic approach and angio-embolization at Kingston Public Hospital, Jamaica, with subsequent benefits.

RESUMEN El angiofibroma nasofaríngeo juvenil (ANJ) en etapa localmente avanzada, tratado tradicionalmente con enfoques externos, se ha asociado a morbilidades significativas, tales como pérdida de sangre con transfusiones subsecuentes de sangre, cicatrices, trastornos del habla, pobre deglución, recurrencia y estancias hospitalarias prolongadas. Este reporte de caso describe nuestra experiencia inicial con un paciente con ANJ localmente avanzada tratado con un enfoque endonasal endoscópico y angioembolización en el Hospital Público de Kingston, Jamaica, con beneficios subsiguientes.

Angiomiofibroblastoma-like, un tumor excepcional: presentación de un caso clínico y diagnóstico diferencial / Angiomyofibroblastoma-like, an exceptional tumor: presentation of a clinical case and differential diagnosis

Presentamos un caso de Angiomiofibroblastoma-like (AML), en el que la ecografía fue importante para determinar la detección, localización y extensión local. Hallazgos: La ecografía demostró una lesión paratesticular sólida hipoecogénica de bordes bien definidos lo que sugirió lesión benigna extratesticular. La histología evidenció una neoplasia mesenquimal benigna tipo mixoide en cuyo diagnóstico diferencial se incluyen el Angiomixoma superficial (AMS), Angiomixoma agresivo (AMA), Angiomiofibroblastoma (AMF) y el AML. En base a estos hallazgos se realizó una revisión de tumores de similares características en la literatura, llegando finalmente al diagnóstico de AML cuya localización paratesticular sólo ha sido descrita en muy pocos casos hasta la fecha. Conclusión: La ecografía es útil para localizar los tumores paratesticulares y determinar su extensión local. No obstante, para llegar al diagnóstico definitivo es necesario realizar un estudio histológico e inmunohistoquímico de la tumoración.

We present a case of a paratesticular Angiomiofibroblastoma-like (AML) tumor in which ultrasound was important to give the specific location and local extension of the lesion. Findings: Ultrasound revealed a hypoechoic paratesticular lesion with well-defined borders, suggesting an extratesticular benign lesion. The histology showed a benign mesenchymal myxoid-type neoplasm. The differential diagnosis included superficial Angiomyxoma (AMS), Aggressive angiomyxoma (AMA), Angiomyofibroblastoma (AMF) and AML. Based on these findings, a review of similar tumors was carried out and ultimately led to the diagnosis of paratesticular AML. This location has been described only in a few cases in the literature. Conclusion: Ultrasound is useful to locate paratesticular tumors and determine their local extension. However, a definitive diagnosis still requires a histological and immunohistochemical study.

Angiofibroma de fosa temporal: reporte de un caso / Temporal fossa angiofibroma: a case report

RESUMEN El angiofibroma nasofaríngeo es el tumor benigno más frecuente de la nasofaringe, representando el 0,05% del total de las neoplasias de cabeza y cuello. Los angiofibromas en localizaciones distintas a la nasofaringe son entidades raras. Ellos son descritos esporádicamente en la literatura, ubicándose principalmente en el seno maxilar. En este artículo presentamos un caso de fibroangioma extranasofaríngeo localizado en fosa temporal derecha seguido de una revisión de literatura.

ABSTRACT Nasopharyngeal angiofibroma is the most common benign tumor of the nasopharynx, representing 0.05% of total neoplasms of the head and neck. Extranasopharyngeal angiofibromas are rare entities described sporadically in the literature, being located mainly in the maxillary sinus. We present a case of an extra-nasopharyngeal fibroangioma located in the right temporal fossa followed by a literature review.

Fibroangioma nasofaríngeo juvenil: experiencia de 15 años en el Hospital Barros Luco Trudeau / Juvenile nasopharyngeal fibroangioma: the experience of 15 years in Hospital Barros Luco Trudeau

Introducción: El fibroangioma nasofaríngeo juvenil es un tumor vascular benigno localmente agresivo, que afecta casi exclusivamente la nasofaringe de adolescentes de sexo masculino. Su manejo es complejo dada su extensión, naturaleza vascular y sus frecuentes recurrencias. Objetivo: Mostrar la experiencia de 15 años en fibroangioma juvenil en nuestro centro. Material y método: Estudio descriptivo retrospectivo de los pacientes con diagnóstico de ingreso de fibroangioma nasofaríngeo juvenil al Servicio de Otorrinolaringología del Hospital Barros Luco Trudeau entre los años 1997 y 2011, caracterizando al grupo de estudio en cuanto a características clínico-demográficas, vasos aferentes, relación entre etapa tumoral y vascularización, manejo terapéutico, complicaciones y recurrencias. Resultados: Se obtuvo un total de 20 pacientes, todos de sexo masculino, con un promedio de edad de 13,9 años. El síntoma de presentación más frecuente fue la epistaxis a repetición y obstrucción nasal presente en el 90% y 80%, respectivamente. Todos los pacientes se estudiaron con tomografia computarizada y recibieron embolización arterial preoperatoria. La mayoría de los tumores fueron de tipo II (65%) y III (20%), según clasificación de Radkowski. La técnica quirúrgica más empleada fue abierta (57,8%). Radioterapia en un caso. El vaso aferente principal fue la maxilar interno ipsilateral en el 100%. Todos los fibroangiomas etapa III eran además irrigados por la arteria carótida interna. Se encontró 20% de persistencia y 15% de recidiva. Conclusión: Nuestros resultados concuerdan con la gran mayoría de las series publicadas en la literatura. Epistaxis recurrente, obstrucción nasal y tumor nasal unilateral deben hacernos sospechar de esta patología en un adolescente masculino. El tratamiento de elección es la cirugía con embolización preoperatoria. La vía de abordaje endoscópica presenta menor morbilidad posoperatoria en pacientes con estadios I y II de Radkowski. Todos los fibroangiomas con compromiso intracraneano, presentan irrigación también del sistema carotideo interno.

Introduction: Nasopharyngeal Fibroangioma is a locally aggressive benign vascular tumor. Its management is complex given its size, vascular nature and its frequent recurrences. Aim: To show the experience of 15 years in Juvenile Fibroangioma in our center. Material and method: Retrospective descriptive study of patients admitted with a diagnosis of Juvenile Fibroangioma Nasopharyngeal in the Department of Otolaryngology Hospital Barros Luco Trudeau between 1997 and 2011. Results: A total of 20 patients was obtained. The most common presenting symptom was recurrent epistaxis and nasal obstruction present in 90% and 80% respectively. The most common surgical technique was open (57.8%). Radiotherapy in one case. The main afferent vessel was the ipsilateral internal maxillary in 100%. All Fibroangioma stage III were also supplied by the internal carotid artery. 20% of persistence and 15% of recurrence was found. Conclusion: Recurrent epistaxis, nasal obstruction and unilateral nasal tumor should raise the suspicion of this disease in a male teenager. The treatment of choice is surgery with preoperative embolization. The route of endoscopic approach has less postoperative morbidity in patients with stage I and II of Radkowski. All Fibroangioma with intracranial commitment, have also the internal carotid irrigation system.

Endoscopic endonasal approach for mass resection of the pterygopalatine fossa

OBJECTIVES: Access to the pterygopalatine fossa is very difficult due to its complex anatomy. Therefore, an open approach is traditionally used, but morbidity is unavoidable. To overcome this problem, an endoscopic endonasal approach was developed as a minimally invasive procedure. The surgical aim of the present study was to evaluate the utility of the endoscopic endonasal approach for the management of both benign and malignant tumors of the pterygopalatine fossa. METHOD: We report our experience with the endoscopic endonasal approach for the management of both benign and malignant tumors and summarize recent recommendations. A total of 13 patients underwent surgery via the endoscopic endonasal approach for pterygopalatine fossa masses from 2014 to 2016. This case group consisted of 12 benign tumors (10 juvenile nasopharyngeal angiofibromas and two schwannomas) and one malignant tumor. RESULTS: No recurrent tumor developed during the follow-up period. One residual tumor (juvenile nasopharyngeal angiofibroma) that remained in the cavernous sinus was stable. There were no significant complications. Typical sequelae included hypesthesia of the maxillary nerve, trismus, and dry eye syndrome. CONCLUSION: The low frequency of complications together with the high efficacy of resection support the use of the endoscopic endonasal approach as a feasible, safe, and beneficial technique for the management of masses in the pterygopalatine fossa.

Onychogryphosis in tuberous sclerosis complex: an unusual feature

Abstract Onychogryphosis is an acquired nail plate change. It often affects the toenail and is characterized by an opaque, yellow-brownish nail plate that is distorted, grossly thickened, elongated, and partly curved resembling a ram's horn. Tuberous sclerosis complex is a multisystem disorder associated with high rates of mental retardation, autism, cognitive impairment, behavioral problems, or seizures. Nail disease can also be associated, which is a concern to patients due to pain and nail distortion. We reported a typical tuberous sclerosis complex patient with distinctive clinical features of a ram's horn nails, which presented a great challenge to surgical treatment and nail restoration.

Nasopharyngeal angiofibroma treated with radiotherapy.

Nasopharyngeal angiofibroma is a rare, highly vascular, benign, locally aggressive tumor, affecting boys of adolescent age. The aggressiveness and high vascularity makes surgery and even a biopsy difficult in majority of cases. Although surgery is the treatment of choice in early cases, considerable debate exists regarding the treatment of advanced disease with intracranial extension. Radiotherapy provides a good response and also avoids surgery-associated morbidity. We are herewith reporting a case of nasopharyngeal angiofibroma who showed complete hemostasis and improvement in vision to radiotherapy.