Giant cell angiofibroma of gingiva in tuberous sclerosis complex: a case report and literature review.

BACKGROUND: Tuberous sclerosis complex (TSC) is a rare, complex genetic disorder characterized by hamartomas and neoplastic lesions in various organ systems. With the development of radiology and gene testing, the diagnostic criteria for TSC were updated in 2012 at the International Consensus Conference. Intraoral fibromas have long been associated with TSC. However, the incidence of giant cell angiofibroma (GCA) in TSC patients is extremely rare. Here, we report the first case of GCA in the gingival tissue of a patient with TSC. CASE PRESENTATION: A 41-year-old woman first visited the Department of Oral and Maxillofacial Surgery, Chonnam National University Dental Hospital, complaining of gingival enlargement. Clinical examination revealed several manifestations associated with TSC, including intraoral fibromas, facial angiofibromas, dental enamel pits, ungual fibromas, "confetti" skin lesions, hypomelanotic macules, and a shagreen patch. Intraoral examination revealed a 6.0 × 5.0 cm gingival overgrowth on the left mandible. Surgical excision was performed, and subsequent histopathological examination confirmed the diagnosis of GCA. There was no evidence of recurrence within the 24- months of surgery. CONCLUSIONS: We report the first case of GCA in the gingival tissue of a patient with TSC. This report would contribute to an improved understanding of this rare disease. However, further case reports are necessary to clarify the relationship between GCA and TSC.

Challenges and innovations in managing a complex giant bilateral juvenile nasopharyngeal angiofibroma with intracranial extension.

Juvenile nasopharyngeal angiofibroma (JNA) is a highly vascular, benign and locally aggressive tumour that predominantly affects adolescent males. Recognised for its persistence and propensity to recur, patients usually present with gradual development of symptoms such as epistaxis and nasal obstruction over several months to years. Diagnosis typically combines clinical assessments and radiographic studies, often involving preoperative angiography to identify feeder vessels and facilitate embolisation, reducing intraoperative bleeding during surgical interventions. A comprehensive approach to treatment, considering both tumour characteristics and patient well-being, is crucial, particularly when dealing with cases involving intracranial extension. Surgical excision remains the primary treatment for angiofibroma, though radiotherapy is considered for cases with intracranial extension. This case report outlines a case involving a young man in his 20s with a large bilateral JNA extending into the intracranial area. The patient underwent preoperative embolisation followed by surgical resection using a nasofrontomaxillary swing approach with a bifrontal craniotomy window. This alternative approach provided enhanced exposure to address the involvement of the infratemporal fossa, anterior and middle skull base. Postoperatively, residual intracranial tumour was managed with radiation therapy. Over a 2-year follow-up, the patient remains asymptomatic, with a minor postradiation reduction in the intracranial component's size.

Validation of the Index for Facial Angiofibromas: Data analysis from a randomized controlled trial of sirolimus gel treatment in patients with tuberous sclerosis complex.

The Index for Facial Angiofibromas (IFA), a novel scoring system for angiofibromas, has been validated in patients with tuberous sclerosis complex (TSC). The objective of this analysis was to further validate the IFA using data from a clinical trial of topical sirolimus in patients with TSC. This was an analysis of photographs from a Phase III trial conducted in Japan (NCT02635789). Patients (n = 62) were randomized 1:1 to receive sirolimus or placebo gel for 12 weeks. Changes in angiofibromas were independently assessed using the primary composite endpoint, the Facial Angiofibroma Severity Index (FASI), and the IFA. Thresholds for a clinically meaningful change in IFA score were evaluated using receiver operating characteristic (ROC) analysis. The IFA scores had good-to-excellent inter-assessor reliability, very high intra-assessor reliability, and could be used to evaluate the distribution of disease severity at baseline. High correlations were observed between the categorized change from baseline in IFA scores and the primary composite endpoint (Kendall's coefficient of concordance, W = 0.8655, p < 0.0001), and between the change from baseline in IFA and FASI scores (Kendall's coefficient of concordance, W = 0.745, p < 0.0001). By ROC analysis, an optimal IFA cut-off point of 1.667 was determined to distinguish patients with markedly improved or improved angiofibromas from those with slightly improved or unchanged angiofibromas (area under the curve 0.937) as determined by the primary composite endpoint. The IFA score is potentially clinically useful because of its high validity and reliability. A decrease in score from baseline of ≥1.667 may be considered clinically meaningful.

Para testicular cellular angiofibroma (AF)/angiomyofibroblastoma (AMF)-like tumor: A case report of a rare diagnosis in an elderly male.

Cellular angiofibroma (AF)/Angiomyofibroblastoma (AMF)-like tumor is a rare benign mesenchymal neoplasm. It is very challenging to distinguish benign versus malignant mass radiologically. It is of paramount importance to distinguish Cellular Angiofibroma (CAF) microscopically from its differential diagnoses. A 64-year-old man presented with scrotal swelling. Pathological examination showed features of cellular AF/AMF- like tumor, which shows positivity for CD34, with negativity for S-100 Protein, smooth muscle actin and desmin.

Early-onset juvenile nasopharyngeal angiofibroma (JNA): a systematic review.

BACKGROUND: Juvenile Nasopharyngeal Angiofibroma (JNA) is a fibrovascular tumor of the nasopharynx that classically presents in adolescent males. The reported mean age of onset is between 13 and 22 years old [1-6]. Significant androgen stimulation is hypothesized to explain the strong predisposition for JNA to present in young adolescent males. However, considerable variability in age at diagnosis exists with rare involvement of very young patients incongruent with typical male pubertal growth patterns. OBJECTIVE: The purpose of this systematic review is to identify cases of early-onset JNA (EOJNA), (defined as age < 10 years) in the literature and to examine the disease characteristics and treatments used in this patient group. A case of a 7 year old boy with EOJNA at our institution is also described and presented. METHODS: We searched Embase, Cochrane database and MEDLINE from 1996 to February 2021 for studies that reported cases of EOJNA. Relevant clinico-demographic data, disease severity and treatment outcomes were recorded and analyzed using descriptive statistics. We compared our findings with reported means for JNA in all ages. RESULTS: We identified 29 studies containing a total of 34 cases of EOJNA. The vast majority (31/34) of patients were males and the mean age of diagnosis was 8.15 years old. The most common presenting symptoms were nasal obstruction (65.2%) and epistaxis (60.9%). Patients were most commonly Radkowski stage II (39.4%) and III (39.4%). Primary treatment modalities included open surgery (66.7%), endoscopic surgery (24.2%), and radiotherapy (9.1%). Recurrence was evident in 30%. Radkowski stage and type of treatment did not differ significantly within the EOJNA group (p = 0.440 and p = 0.659, respectively). CONCLUSION: This systematic review suggests that rare cases of EOJNA have distinct disease characteristics. Patients in this cohort appeared to have more advanced disease and higher recurrence rates when compared with reported averages. We hope that this review prompts increased clinical awareness of this potentially more aggressive subtype of JNA. As more cases of EOJNA are reported, a more powered statistical analysis of this cohort would be feasible.

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.

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.

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)

Angiomiofibroblastoma vulvar. Reporte de un caso / Angiomyofibroblastoma of the Vulva. A case report

Introducción: El angiomiofibroblastoma es una lesión mesenquimatosa benigna, nodular, bien circunscrita, poco frecuente, localizada principalmente en la vulva, se presenta casi exclusivamente en mujeres de mediana edad. Clínicamente son tumores de crecimiento lento, acompañados de dolor, que con frecuencia tiende a diagnosticarse como un quiste de Bartolino, hidroceles del canal de Nuck y angiomixoma agresivo. El tratamiento de elección es la exéresis quirúrgica. Caso clínico: Se presenta el caso de una mujer de 49 años de edad quien acudió a consulta por presentar una masa de 3 años de evolución con crecimiento progresivo en región vulvar, que se acompañaba de intenso dolor y ardor limitando sus actividades diarias. A la evaluación clínica se encuentra masa de gran tamaño que abarca labio mayor y menor izquierdo de la vulva. El diagnóstico tras la exéresis quirúrgica y estudio patológico fue angiomiofibroblastoma. Discusión: Aunque esta patología es una entidad poco frecuente, el diagnóstico correcto del angiomiofibroblastoma, evitará que se confunda con otras lesiones de mayor riesgo y que se realice por tanto, un tratamiento excesivo e inadecuado, ya que este tumor se cura con la excisión simple. Conclusión: Aunque el angiomiofibroblastoma es una entidad poco frecuente, su reconocimiento y correcto diagnóstico es importante ya que el tratamiento estriba en una resección simple, con excelente pronóstico y sin recurrencia, por lo que debe ser considerado como parte del repertorio de diagnósticos clínicos cuando una mujer consulta por una lesión vulvar...(AU)

Tuberous sclerosis complex diagnosed from oral lesions / Complexo esclerose tuberosa diagnosticado a partir de lesoes orais

CONTEXT: Tuberous sclerosis complex (TSC) is a genetic disease in the group known as neurocutaneous syndromes, with dominant autosomal inheritance. It is characterized by skin and adnexal lesions and central and peripheral nervous system tumors, with neurological and psychiatric findings. It may affect the heart, kidneys, eyes, face, bones, lungs, stomach and dentition. CASE REPORT: We present the case of a 66-year-old man with dermatological signs that included hypopigmented maculae, confetti-like lesions, shagreen plaque, angiofibromas on nasolabial folds, neck and back, nail dystrophy and periungual fibromas on fingers and toes. An electroencephalogram produced normal results, but magnetic resonance imaging showed a nodular image measuring 1.2 x 1.0 cm close to the Monro foramen, which was similar to cerebral parenchyma and compatible with a subependymal giant-cell astrocytoma. A conservative approach was taken, through control imaging examinations on the lesion for seven years, with absence of any expansive process or neurological symptoms. Abdominal ultrasonography revealed a solid, heterogenic and echogenic mass with a calcified focus, measuring 4.6 x 3.4 cm, in the rightkidney, compatible with angiomyolipoma. The patient was treated by means of complete nephrectomy because of malignant areas seen on histopathological examination and died one month after the procedure. This case report illustrates the importance of oral clinical findings such as dental enamel pits and angiofibromas in making an early diagnosis of TSC, with subsequent screening examinations, treatment and genetic counseling. .

CONTEXTO: O complexo esclerose tuberosa é uma doença genética pertencente ao grupo das facomatoses, de herança autossômica dominante, caracterizada por lesões acometendo pele e anexos, além de tumores do sistema nervoso central e periférico, com presença de achados neurólogicos e psiquiátricos, podendo acometer coração, rins, olhos, dentes, mucosa oral e outros órgãos. RELATO DE CASO: Apresentamos o caso de um paciente do sexo masculino, 66 anos de idade, que apresentava diversos sinais dermatol ógicos, como máculas hipopigmentadas, lesões em confete, placas tipo shagreen, angiofibromas nas regiões cervical, nasolabial e dorsal, distrofias ungueais e fibromas nos dedos das mãos e pés. Embora o encefalograma não tenha mostrado alterações, a ressonância nuclear magnética apresentou imagem nodular com aspecto semelhante ao parênquima cerebral, medindo 1.2 x 1.0 cm e próxima ao forame de Monro, compatível com astrocitoma subependimário de células gigantes. Abordagem conservadora foi escolhida por meio de controle imaginológico da lesão por sete anos, com ausência de sintomas neurológicos ou processos expansivos. A ultrassonografia de abdômen total revelou massa medindo 4.6 x 3.4 cm, com áreas ecogênicas e heterogêneas, apresentando focos de calcificação no rim direito, com padrão semelhante ao de um angiomiolipoma. O paciente foi submetido a nefrectomia total, devido à presença de áreas de malignidade ao exame histopatológico, e evoluiu para óbito um mês após o procedimento. Este relato de caso ilustra a importância de achados clínicos orais, tais como lesões no esmalte dentário e angiofibromas, para o diagnóstico precoce dessa doença e posterior rastreamento, tratamento ...

Nasopharyngeal angiofibroma: Our experience and literature review

Introduction: Juvenile nasopharyngeal angiofibroma is a rare, highly vascular, and histologically benign tumor, generally observed in male adolescents. It shows very aggressive behavior due to local invasiveness and is associated with various symptoms. Juvenile nasopharyngeal angiofibroma originates in the sphenopalatine forame, causing epistaxes and nasal obstruction. Objective: To retrospectively describe our experience in the diagnosis and treatment of patients with juvenile nasopharyngeal angiofibroma. Scientific drawing: Retrospective, descriptive study conducted after approval from the Ethics Committee of the Federal University of Sergipe (protocol 0114.0.107.000 -11). Methods: We analyzed findings in 20 patients who underwent surgery between 2004 and 2011. Factors analyzed include patient age and gender, symptoms, stages, treatment, length of surgery, intraoperatory bleeding, postoperative need for nasal tampons, hospitalization time, complications, and tumor recurrence. Results: Patients were aged 10-29 years. All patients were treated surgically, including 17 who underwent endoscopic surgery. The mean operation time was 120 min, and the mean bleeding volume was 300 mL. Seventeen patients required clamping of the external carotids and tumor embolization. Conclusion: Endoscopic surgery alone or with other conventional techniques was safe for the treatment of angiofibromas of different stages...

Nasoangiofibroma juvenil en un paciente adulto masculino / Juvenile nasopharyngeal angiofibroma in an adult male

El nasoangiofibroma juvenil es un tumor infrecuente, que comprende el 0,05 por ciento de los tumores de cabeza y cuello, histológicamente es benigno, pero localmente se comporta como invasivo, tiene una predilección especial por la nasofaringe y pacientes adolescentes de sexo masculino. Es un tumor altamente vascular, la epistaxis recurrente y la obstrucción nasal son los dos síntomas más comúnmente referidos por los pacientes y puede comprometer la vida secundaria al sangrado o la extensión intracraneana. Aunque generalmente se presenta en adolescentes de sexo masculino, se han descrito algunos casos en mujeres y adultos. Este artículo presenta un caso inusual de nasoangiofibroma juvenil en un paciente masculino de 39 años, quien se presenta al servicio de urgencias del Hospital Central de la Policía por presencia de epistaxis aguda abundante, que lo lleva a choque hipovolémico, requiriendo transfusión, estabilización, arteriografía con embolización y resección de la lesión.

The Juvenile Nasopharyngeal Angiofibroma is a rare tumor, which comprises 0.05% of head and neck tumors, histologically benign, but locally is invasive, has a special predilection for the nasopharynx and male adolescent patients, is highly vascular, the recurrent epistaxis and nasal obstruction are the two most common symptoms reported by patients. These tumors can compromise the life secondary to bleeding or intracranial extension. Although it usually occurs in male adolescents have been few cases in women and adults, this article presents an unusual case of juvenile nasoangiofibroma in a 39 years old male patient, who presents to the emergency department of the Central Police Hospital by the presence of abundant and acute epistaxis, which leads to hypovolemic shock, requiring transfusion, stabilization, arteriography with embolization and resection of the lesion.

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.

Experiência de sete anos em pacientes com angiofibroma nasofaríngeo juvenil / A seven-year experience with patients with juvenile nasopharyngeal angiofibroma

O angiofibroma nasofaríngeo juvenil (ANJ) é um raro tumor em adolescentes masculinos originário na nasofaringe. OBJETIVOS: Apresentar a experiência do tratamento do ANJ em pacientes do Serviço de Otorrinolaringologia entre 2001 e 2008. MATERIAL E MÉTODOS: Foram revisados de prontuários do Serviço de Otorrinolaringologia os dados demográficos, apresentação clínica, métodos de investigação e tratamento de 16 pacientes. DESENHO DO ESTUDO: estudo descritivo, retrospectivo, de corte transversal. Resultados: Todos os pacientes são do gênero masculino e a média de idade ao diagnóstico foi de 16,8 anos (variação de 9 a 23 anos), sendo mais de 56 por cento deles com estádio II de Fisch. Embolização pré-operatória foi realizada em 10 pacientes (62,5 por cento). Todos os 16 pacientes foram submetidos à ressecção cirúrgica. Dois pacientes (66,7 por cento) que não foram submetidos à embolização pré-operatória necessitaram de transfusão sanguínea. O índice de recidiva foi de 43,75 por cento e o índice de cura foi 93,75 por cento. CONCLUSÕES: Embolização pré-operatória diminui a perda sanguínea intra-operatória. O índice de recidiva foi relacionado ao estadiamento avançado do tumor ao diagnóstico e à não-realização de embolização pré-operatória. Cirurgia associada à embolização pré-operatória são os principais tratamentos do ANJ. Todos os pacientes devem ter estudos de imagem pré-operatórios, especialmente tomografia computadorizada, para auxiliar no planejamento cirúrgico e no seguimento.

Juvenile nasopharyngeal angiofibroma (JNA) is a rare tumor in adolescent males. It originates in the nasopharynx. AIM: to present the experience of JNA management at an Otorhinolaryngology Service between 2001 and 2008. MATERIALS AND METHODS: Demographical data, clinical presentation, investigations as well as the treatment of sixteen JNA patients were reviewed and collected from medical records from the ORL Service. DESIGN: Cross-sectional, retrospective and descriptive study. Results: All JNA patients were male. The average age at diagnosis was 16.8 years (range 9-23 years). More than 56 percent of the patients were classified as Fisch II. Preoperative embolization was carried out in ten (62.5 percent) patients. All 16 patients were submitted to primary surgical resection. Two patients (66.7 percent) who didn't receive preoperative embolization required intraoperative blood transfusion. The overall recurrence rate was 43.75 percent and the cure rate was 93.75 percent. CONCLUSION: Preoperative embolization minimizes intraoperative blood loss. The recurrence rate was related to advanced tumoral stage at diagnostic and the lack of preoperative embolization. Surgery combined with preoperative embolization is the major treatment for JNA. All the patients should undergo preoperative imaging studies, especially CT, to assist in surgical planning and follow-up.