Angiomiofibroblastoma vulvoperineal, presentación de un caso / Vulvoperineal angiomyofibroblatoma, a case report

An angiomyofibroblastoma is a benign tumor that may present itself in the genital tract of a reproductive age woman. Despite it being a benign neoplasia, unable to be invasive, it can reach large sizes, producing discomfort, pain, and anatomical deformation. A late diagnosis may result in increased surgical difficulty for resection. The following is the clinical case of a woman with a vulvo-perineal tumor of 6 years of evolution, with a final diagnosis of angiomyofibroblastoma, in which complete resection of the lesion was achieved with good cosmetic results. The highlight of this tumor is its slow, silent, but progressive growth, leading to diagnoses that are often confused and late, with a consequent delay in treatment. Among the common differential diagnoses are the Bartholin gland cyst and the aggressive angiomyxoma.

El angiomiofibroblastoma es un tumor benigno que puede presentarse en el tracto genital de la mujer en edad reproductiva. A pesar de ser una neoplasia benigna, sin capacidad invasora, puede alcanzar grandes tamaños, produciendo molestia, dolor y alteración importante de la anatomía. Si el diagnóstico es tardío, aumenta la dificultad quirúrgica en su resección. A continuación, se presenta el caso clínico de una mujer con un tumor vulvoperineal de 6 años de evolución, con diagnóstico final de angiomiofibroblastoma, en el que se logró la resección completa de la lesión con un buen resultado estético. La importancia de este tumor está dada por su crecimiento lento, silencioso, pero progresivo, llevando a que su diagnóstico sea en muchas ocasiones confuso y tardío, con el consecuente retraso en el tratamiento. Dentro de los diagnósticos diferenciales comunes se encuentran el quiste de la glándula de Bartholino y el angiomixoma agresivo.

Hamartoma fibrovascular facial grave en paciente con esclerosis tuberosa (ET). Resolución de caso problema. Nuestra experiencia / Severe facial fibrovascular hamartoma in a patient with tuberous sclerosis (TS). Problem case resolution. Our experience

La ET es un trastorno multisistémico autosómico dominante que se caracteriza por displasia celular y tisular en varios órganos (cerebro, corazón, piel, ojos, riñones, pulmones) que constituyen una fuente importante de morbilidad y mortalidad. Las manifestaciones comunes incluyen tubérculos corticales, nódulos subependimarios, astrocitomas subependimarios de células gigantes, convulsiones, rabdomiomas cardíacos, AML renales, hamartomas retinianos, linfangioleiomiomatosis pulmonar, angiofibromas faciales, manchas de hojas de ceniza, parches de Shagreen, discapacidad intelectual y trastorno del espectro autista. Se presenta a continuación la resolución de un caso problema grave de una paciente que llega a la consulta al hospital público, con severa incapacidad para mantener la permeabilidad de la válvula nasal externa, a expensas de formación harmartomatosa grave, de años de evolución, fétida y sangrante, decidiéndose tomar conducta quirúrgica urgente y agresiva dada las condiciones de la lesión, la poca colaboración de la paciente y el contexto familiar de la misma que presenta además trastornos conductuales asociados a manifestaciones neurológicas de la enfermedad (retraso madurativo)

ET is an autosomal dominant multisystem disorder characterized by cellular and tissue dysplasia in several organs (brain, heart, skin, eyes, kidneys, lungs) that constitute a major source of morbidity and mortality. Common manifestations include cortical tubercles, subependymal nodules, subependymal giant cell astrocytomas, seizures, cardiac rhabdomyomas, renal AML, retinal hamartomas, pulmonary lymphangioleiomyomatosis, facial angiofibromas, ash leaf spots, shagreen patches, intellectual disability, and autism spectrum disorder. This paper presents the resolution of a serious problem case of a patient who attends the consultation of a public hospital, with severe inability to maintain the patency of the external nasal valve, at the expense of severe harmartomatous formation, of many years of evolution, fetid and bleeding, deciding to undertake urgent and aggressive surgical conduct given the conditions of the lesion, the lack of collaboration of the patient and the family context of the same, which also presents behavioral disorders associated with neurological manifestations of the disease (maturational delay).

Surgical approach analysis of endoscopic resection of juvenile nasopharyngeal angiofibroma / 临床耳鼻咽喉头颈外科杂志

Objective:To investigate the surgical approach for the resection of juvenile nasopharyngeal angiofibroma（JNA） under nasal endoscopy. Methods:The clinical data of 87 patients undergoing endoscopic resection of nasopharyngeal fibroangioma were retrospectively analyzed. We classified JNA according to tumor site, size, invasion scope and anatomic position relationship between tumor and midline of pupil. Three endoscopic surgical approaches were selected according to the classification, and the postoperative symptoms, complications and recurrence were investigated and analyzed. Results:The tumor resection rate of 87 cases by nasal endoscopic surgery was 100%. Thirty-five cases were approached through the middle nasal passage（small tumors located in the nasal sinuses and pterygopalatine fossa）, forty-five cases were approached through the lateral wall of the nasal cavity（tumor invaded the pterygopalatine fossa but did not exceed the midline of the pupil） , and seven cases were approached via the lateral wall of nasal cavity + ipsilateral anterior wall of maxillary sinus（tumor invaded the infratemporal fossa beyond the midline of pupil or invaded the cavernous sinus and the middle cranial fossa epidural）, Postoperative patients with nasal congestion, nasal bleeding, headache, dizziness, vision loss and other symptoms showed varying degrees of improvement. No surgical death or intracranial infection occurred. The postoperative follow-up was 6-78 months, and the recurrence rate was 3.44%. Conclusion:Endoscopic resection of nasopharyngeal fibroangioma is the main treatment method for JNA. Selecting suitable endoscopic approach to resect JNA, To maximize the advantage of nasal endoscopic equipment according to the inherent anatomical space of the human nasal cavity, In order to achieve the purpose of JNA resection, reduce intraoperative and postoperative complications, reduce the recurrence rate and improve the prognosis.

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.

Tumores de Fosa Pterigopalatina: Tipos de abordajes quirúrgicos y complicaciones / Pterygopalatine Fossa Tumors: Types of surgical approaches and complications

INTRODUCCIÓN. La fosa pterigopalatina es una zona anatómica de difícil acceso, que al presentar masas tumorales genera un alto riesgo de morbimortalidad en población juvenil y adulta, que precisa determinar las complicaciones asociadas a cirugía. OBJETIVO. Evaluar los tipos de abordaje quirúrgico, complicaciones e identificar la estirpe histológica de los tumores de fosa pte-rigopalatina. MATERIALES Y MÉTODOS. Estudio transversal retrospectivo. Población y muestra conocida de 29 Historias Clínicas de pacientes con hallazgos de imagen e histopatológico de tumores con invasión a fosa pterigopalatina divididos en dos grupos: A) resección de masa tumoral y B) biopsia de masa tumoral, operados en el Hospital de Especialidades Carlos Andrade Marín en el período de enero 2017 a diciembre de 2020. RESULTADOS. El 82,76% (24; 29) fueron hombres, con promedio de edad de 30,6 años. El 82,76% (24; 29) de las masas tumorales se originaron en nasofaringe; no se reportaron casos primarios. El tumor más frecuente fue el Angio-fibroma Nasofaringeo Juvenil 68,97% (20; 29), seguido por los tumores malignos con el 20,69% (6; 29), siendo usual el carcinoma adenoideo quístico. En el 62,07% (18; 29) el tumor invadió Fosa Infratemporal y en el 44,83% (13; 29) hacia esfenoides. En el grupo A, el abordaje quirúrgico empleado en el 20,83% (5; 24) fue mediante técnica abierta y en el 79,17% (19; 24) con técnica endoscópica, tanto uni 31,58% (6; 19) como multiportal 68,42% (13; 19). La complicación fue la hiposensibilidad facial en el 12,5% (3; 24), todos en abordajes abiertos. CONCLUSIÓN. Se evaluó los tipos abordaje quirúrgico y se identificó la estirpe histológica de los tumores de fosa pterigopalatina

INTRODUCTION. The pterygopalatine fossa is an anatomical area of difficult access, which when presenting tumor masses generates a high risk of morbimortality in the juvenile and adult popula-tion, which needs to determine the complications associated with surgery. OBJECTIVE. To eva-luate the types of surgical approach, complications and identify the histologic type of pterygopala-tine fossa tumors. MATERIALS AND METHODS. Retrospective cross-sectional study. Population and known sample of 29 Clinical Histories of patients with imaging and histopathological findings of tumors with invasion to pterygopalatine fossa divided into two groups: A) resection of tumor mass and B) biopsy of tumor mass, operated at the Carlos Andrade Marín Specialty Hospital in the period from january 2017 to december 2020. RESULTS. The 82,76% (24; 29) were men, with an average age of 30,6 years. 82,76% (24; 29) of the tumor masses originated in nasopharynx; no primary cases were reported. The most frequent tumor was juvenile nasopharyngeal angiofibroma 68,97% (20; 29), followed by malignant tumors with 20,69% (6; 29), being usual the adenoid cystic carcinoma. In 62,07% (18; 29) the tumor invaded the Infratemporal Fossa and in 44,83% (13; 29) into the sphenoid. In group A, the surgical approach used in 20,83% (5; 24) was by open technique and in 79,17% (19; 24) by endoscopic technique, both uni 31,58% (6; 19) and multiportal 68,42% (13; 19). The complication was facial hyposensitivity in 12,5% (3; 24), all in open approaches. CONCLUSION. The types of surgical approach were evaluated and the histologic type of pterygo-palatine fossa tumors was identified.

A rare case of Angiofibroma mimicking Fibrous Epulis in Posterior Gingival Mucosa

@#Angiofibromas are highly vascular, locally aggressive but histologically benign tumors. A 40-year-old woman complaint of pedunculated mass in the right gingiva of the posterior region since 7 months earlier. Surgical excisional biopsy under general anesthesia was performed. Histopathological examination showed an angiofibroma. Angiofibromas are localized in the superficial soft tissue and the gingiva could have a similar clinical appearance as an epulis. Definitive diagnosis was determined through histopathological analysis.

Anestesia en el angiofibroma juvenil: un reto al anestesiólogo / Anesthesia in juvenile angiofibroma: an challenge for the anesthesiologist

Introducción: La anestesia para el angiofibroma juvenil es una de las intervenciones más complejas dentro de la otorrinolaringología, pues el sangramiento incoercible causa hipovolemia aguda la cual se produce en un breve período y lleva al shock hipovolémico y a la muerte de no tratarse adecuadamente. Objetivo: Realizar una actualización sobre el perioperatorio de la anestesia en el angiofibroma juvenil. Desarrollo: Se debe minimizar las pérdidas sanguíneas a toda costa. La mejor asociación fue la hipotensión inducida, la hemodilución hipovolémica y el predepósito de sangre autóloga, con la angiografia y embolización arterial selectiva del tumor. Conclusiones: La exéresis quirúrgica del angiofibroma juvenil es una intervención de alto riesgo. El equipo de trabajo constituye un elemento primordial. La asociación de hipotensión inducida, hemodilución hipovolémica y predepósito de sangre autóloga son los pilares fundamentales para la mejor evolución de estos pacientes(AU)

Introduction: Anesthesia for juvenile angiofibroma is one of the most complex interventions within otolaryngology, since incoercible bleeding causes acute hypovolemia, which occurs in a short period and leads to hypovolemic shock and death if not treated properly. Objective: To carry out an update about the perioperative period of anesthesia in juvenile angiofibroma. Development: Blood losses must be minimized at all costs. The best association was induced hypotension, hypovolemic hemodilution, and autologous blood predeposit, with angiography and selective arterial embolization of the tumor. Conclusions: Surgical removal of juvenile angiofibroma is a high-risk intervention. The work team is an essential element. The association of induced hypotension, hypovolemic hemodilution, and autologous blood predeposit are the fundamental pillars for the best evolution of these patients(AU)

Cellular angiofibroma: A rare vulvar tumor case report angiofibroma celular: Um caso raro de tumor vulvar

Abstract Cellular angiofibroma (CA)is a rare benign mesenchymal tumor. In women, it occurs mainly in the vulvovaginal region, with vulvar location in 70% of the cases. Its clinical presentation is nonspecific and similar to several other vulvar tumors of different cellular origins. Thus, its histological and immunohistochemical features allow distinction fromother tumors. Cellular angiofibromas have good prognosis, despite some risk of relapse. The authors present the case of a 49-year-old woman with a bulky right vulvar lesion, for which the preoperative diagnosis was a Bartholin cyst, but the histological and immunohistochemical evaluation yielded a CA.

Resumo O angiofibroma celular é um tumor mesenquimatoso benigno e raro. Nasmulheres, surge principalmente na região vulvo-vaginal, com localização vulvar em 70% dos casos. A sua apresentação clínica é inespecífica e semelhante a vários outros tumores vulvares de diferentes origens celulares. Assim, são as suas características histológicas e imunohistoquímicas que permitem a diferenciação entre eles. Os angiofibromas celulares têm bom prognóstico,embora apresentemalgumrisco de recidiva. Os autores apresentamo caso de umamulher de 49 anos de idade comuma lesão vulvar direita volumosa, cujo diagnóstico pré-operatório era de quisto da glândula de Bartholin, mas cujo exame histológico e imunohistoquímico revelaram tratar-se de um angiofibroma celular.

Angiofibroma nasoseptal: reporte de un caso y revisión / Nasal septal angiofibroma: case report and review

RESUMEN Paciente de 32 años que acudió a consultas por epistaxis intermitente. A la exploración, se evidenció una masa proveniente del septum en fosa nasal izquierda no sospechosa, que a la biopsia demostró ser un angiofibroma. Se describe el abordaje terapéutico de un angiofibroma extranasofaríngeo septal y una revisión de la literatura.

ABSTRACT A 32-year-old patient attended our department for intermittent epistaxis. Upon examination, a non-suspicious mass coming from the septum was found in the left nostril, which at biopsy proved to be an angiofibroma. We describe the therapeutic approach of a septal extranasopharyngeal angiofibroma and a review of the literature.

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.

Nasal septum angiofibroma: a rare condition with an unusual onset

The characteristics of extra-nasopharyngeal angiofibromas tend to be different from angiofibromas of the nasopharynx according to patient gender, patient age, prevalence, affected site, pathogenesis, and clinical and epidemiological features. We report a case of an extra-nasopharyngeal angiofibroma in a 28-year-old man referred to the ENT Clinic for right-sided epistaxis, airflow impairment and nasal swelling. The right nostril was completely occluded works by a reddish-yellow mass that bled easily. The computed tomography scan revealed an “inhomogeneous solid lesion in the nasal fossa”. With the patient under general anesthesia, the formation in the anterior portion of the right side of the nasal septum was removed up to its vascular base. Although electrical cauterization efficiently controlled the bleeding, we abraded the sub-perichondral area to prevent further bleeding as well as recurrence. The histological exam report confirmed the diagnosis of angiofibroma. As in our case, epistaxis is commonly the presenting sign of angiofibroma. Yet its onset was peculiar, given that the bleeding started with a low impact trauma. The nasal swelling was also a relevant feature as well as the breathing impairment. Although uncommon, nasal septal angiofibromas should considered in patients with epistaxis.

Use of Topical Rapamycin as Maintenance Treatment after a Single Session of Fractionated CO₂ Laser Ablation: A Method to Enhance Percutaneous Drug Delivery

Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous disorder with an incidence of approximately 1 in 5,000 to 10,000 live births. TSC has various clinical manifestations such as multiple hamartomas in systemic organs, including the skin. Angiofibromas are the most common skin lesions in patients with TSC. Although benign, angiofibromas develop in childhood and puberty, and can be psychosocially disfiguring for patients. Skin lesions in TSC, specifically angiofibromas, have no significant risk of malignant transformation after puberty; thus, they require no treatment if not prominent. However, the presentation of TSC is important owing to its impact on patient cosmesis. Surgical treatment and laser therapy are the mainstream treatments for angiofibromas. Although the evidence is limited, topical mammalian target of rapamycin inhibitors such as sirolimus (rapamycin) are effective in facial angiofibroma treatment. We describe an adult patient with an angiofibroma who had an excellent response to treatment with topical rapamycin after a single session of carbon dioxide (CO₂) laser ablation. The patient showed no sign of relapse or recurring lesions for a year. CO₂ laser ablation may serve as a new paradigm of treatment for angiofibromas in TSC. Since the selection of laser devices can be limited for some institutions, we suggest a rather basic but highly effective approach for angiofibroma treatment that can be generally applied with the classic CO₂ device.

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 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)

Osteotomia Le Fort I para tratamento cirúrgico do angiofibroma juvenil nasofaríngeo: relato de três casos / Le Fort I osteotomy for surgical treatment of juvenile nasopharyngeal angiofibroma: report of three cases

O angiofibroma juvenil nasofaríngeo é um tumor benigno altamente vascularizado, de crescimento lento, mas localmente invasivo e destrutivo. De baixa prevalência, geralmente se localiza na nasofaringe de indivíduos jovens do gênero masculino. As características clínicas mais comuns são obstrução nasal e epistaxe. Possui diagnóstico baseado, principalmente, nos achados clínicos e imaginológicos, já que a biópsia é dificultada pelas características vasculares da lesão. Seu tratamento de escolha é a excisão cirúrgica, que pode ser realizada por diversas técnicas: via endoscópica, degloving médio-facial, via transpalatina, rinotomia lateral ou através da Osteotomia Le Fort I. O objetivo deste trabalho é relatar a utilização da Osteotomia Le Fort I como acesso para o tratamento cirúrgico de três pacientes do gênero masculino portadores de extensos angiofibromas localizados na nasofaringe que procuraram atendimento no ambulatório de cirurgia buco-maxilo facial do Hospital Universitário Presidente Dutra (HUUFMA)... (AU)

Juvenile nasopharyngeal angiofibroma is a slow-growing, highly invasive and vascularized benign tumor. With low prevalence rate, it is usually located in the nasopharynx of young men. The most common clinical characteristics are nasal obstruction and epistaxis. Its diagnosis is based mainly on the clinical and imaging findings, for the biopsy is hindered due to the vascular characteristics of the lesion. Thus, the treatment of choice is its surgical excision, which can be performed by many techniques: endoscopic, medial-facial degloving, transpalatine, lateral rhinotomy or through the Le Fort I Osteotomy. This paper aims to depict the use of Le Fort I Osteotomy approach for the surgical treatment of three male patients with extensive angiofibroma located in nasopharynx who sought care at the Oral and Maxillofacial Surgery Department of Presidente Dutra University Hospital.... (AU)

Abordaje endoscópico transeptal de tumores benignos de cavidades paranasales / Transseptal endoscopic approach of benign tumors of paranasal sinuses

RESUMEN La cirugía endoscópica nasosinusal es un procedimiento frecuente en la práctica otorrinolaringológica, y en la última década se han introducido nuevas técnicas para ayudar a la resección de tumores en regiones tradicionalmente consideradas de difícil acceso. Una de estas técnicas es el abordaje transeptal, que permite el abordaje de la pared anterior y lateral del seno maxilar, así como el trabajo a cuatro manos para el abordaje de estos tumores. El objetivo de esta revisión es describir el abordaje endoscópico transeptal, como técnica complementaria en la cirugía endoscópica de tumores nasales benignos, específicamente de papiloma invertido y angiofibroma nasofaríngeo juvenil.

ABSTRACT Endoscopic sinus surgery is a frequent procedure in Otorhinolaryngology practice, in the last decade new techniques have been introduced to help the resection of tumors in regions traditionally considered of difficult access. One of these techniques is the trans-septal approach, which allows the approach of the anterior and lateral wall of the maxillary sinus, as well as four-handed work for approach of these tumors. The aim of this review is to describe the transeptal endoscopic approach as a complementary technique in the endoscopic surgery of benign nasal tumors, specifically inverted papilloma and juvenile nasopharyngeal angiofibroma.

Estado actual del tratamiento del angiofibroma nasal juvenil / Current status of the nasopharyngeal angiofibroma treatment

El angiofibroma nasal juvenil es un tumor benigno de presentación infrecuente, que afecta a varones prepuberes y púberes predominantemente, altamente vascularizado lo que explica el sangrado nasal importante que acompaña la sintomatología de estos pacientes, y que puede devenir en complicaciones fatales durante el acto operatorio. La localización anatómica donde se origina este tumor y la relación que establece durante su crecimiento con otras estructuras en la base de cráneo explican la complejidad que implica tratar a este tipo de pacientes. La presente revisión tiene por objeto presentar el estado actual del tratamiento de estos tumores. El tratamiento de elección es la cirugía, con una tendencia cada vez mayor a realizar abordajes menos invasivos, dejando otras modalidades como la radioterapia para aquellos casos inoperables. (AU)

The nasopharyngeal angiofibroma is an infrequent benign tumor that presents in male patients, predominantly preadolescents, highly vascularized which explains the high volume nasal bleeding during the course of the disease, that can even cause fatal outcomes during surgery. The anatomic location where this tumor originates and the following compromise of adyacent structures in the skull base explains the complexity of the treatment of this tumor. The following article review the state of the art of the treatment offered to the patients published in the literature. The election treatment is surgery, with a growing tendency to offer minimally invasive techniques, and recommending other modalities, such as radiotherapy, for advanced tumors not amenable to surgical resection. (AU)

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.