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)

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...

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 ...

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 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.

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.

Manifestações neoplásicas em rinofaringe / Neoplasic manifestation in rhinopharynx

As neoplasias primárias de nasofaringe são raras na infância e adolescência. Sintomas como obstrução nasal persistente, cefaleia, epistaxes recorrentes e abaulamento paranasal ou de palato são comuns à maioria delas e podem ser confundidos com outras doenças infecciosas ou alérgicas.As neoplasias mais frequentes na faixa etária pediátrica são os rabdomiossarcomas, os linfomas não Hodgkin, o estesioneuroblastoma, o carcinoma de nasofaringe e o angiofibroma juvenil. A biópsia ou ressecção da lesão quando possível, seguida de tratamento quimio e radioterápico, quando indicado, compõem o tratamento destas neoplasias.

Frequency, presentation and management of juvenile nasopharyngeal angiofibroma

To determine frequency, clinical presentation and management of juvenile nasopharyngeal angiofibroma [JNA] at Civil Hospital Karachi. A descriptive study. Department of Otorhinolaryngology - Head and Neck Surgery, Dow Medical College, Civil Hospital Karachi and Dow University of Health Sciences from, January 2002 to December 2008. This study included 54 cases of nasopharyngeal angiofibroma over a period of 7 years. Follow up period ranges from 1 month to 7years. All patients were male with an age range of 14 - 17yrs. Epistaxis and nasal obstruction were the two most common presenting symptoms. Majority of our patients 39 [72%] had stage III a disease, stage II disease as seen in 13 [24%] cases while 2 [4%] cases presented in stage I disease according to Fisch classification. Surgery was done in all patients. Tumor recurrence was seen in 4 [7%] of cases. Angiofibroma usually presents in adolescent males. The triads of nasal obstruction, nasopharyngeal mass and recurrent epistaxis indicate the presence of the neoplasm. Surgery is the treatment of choice. Lateral rhinotomy is the most common approach. The sublabial endoscopic assisted approach is cosmetically better than other approaches and has definite advantages. Long term outcome for our patients treated by endoscopic assisted sublabial technique is required to further strengthen our view

Angiomyofibroblastoma Arising from the Posterior Perivesical Space: a Case Report with MR Findings

Angiomyofibroblastoma is a rare benign soft tissue neoplasm that predominantly occurs in the genital region of middle-aged women. We present a case of an angiomyofibroblastoma that involved the posterior perivesical space in a 48-year-old woman. We have documented the magnetic resonance imaging features of this case.

Tuberous sclerosis complex: advances in diagnosis, genetics, and management

Tuberous sclerosis complex (TSC) is an autosomal dominant multisystem neurocutaneous syndrome characterized by the development of multiple hamartomas distributed throughout the body, skin, brain, heart, kidneys, liver, and lungs. Two-thirds of patients represent sporadic mutations. The classic triad is seizures, mental retardation, and cutaneous angiofibromas. However, the full triad occurs in only 29 por cento of patients; 6por cento of them lack all three of them. Two tumor suppressor genes responsible for TSC have been identified: TSC1 gene on chromosome 9 and TSC2 on chromosome 16. This article highlights the most recent significant advances in the diagnosis and genetics of TSC, along with a discussion on the limitations and the usefulness of the revised 1998 clinical criteria for the tuberous sclerosis complex. The [quot ]ash leaf[quot ] macule often comes in other shapes, such as round; most are polygonal, usually 0.5 cm to 2.0 cm in diameter, resembling a thumbprint. Since the death of its describer, Thomas Fitzpatrick, we call each a [quot ]Fitzpatrick patch.[quot ] Special attention is paid in this work to TSC treatment options, including therapeutic trials with rapamycin, also known as sirolimus. LEARNING OBJECTIVE: After completing this learning activity, participants should familiar with tuberous sclerosis complex, its cutaneous signs and systemic findings stratified by patient age, its genetics, and the potential for meaningful therapeutic intervention.

Recurrent Juvenile Nasopharyngeal Angiofibroma Treated with Gamma Knife Surgery

Radiosurgery has been rarely applied for juvenile nasopharyngeal angiofibroma (JNA) and cumulative reports are lacking. The authors report a case of successful treatment of recurred JNA with gamma knife surgery (GKS). A 48-yr-old man was presented with right visual acuity deterioration and brain magnetic resonance images (MRI) disclosed a 3 cm-sized intraorbital mass in the right orbit. He underwent a right fronto-temporal craniotomy and the mass was subtotally removed to preserve visual function. Histological diagnosis confirmed JNA in typical nature. However, the vision gradually worsened to fail four years after operation. MRI then showed regrowth of the tumor occupying most of the right orbit. GKS was done for the re-curred lesion. A dose of 17 Gy was delivered to the 50% isodose line of tumor mar-gin. During the following four-year follow-up period, the mass disappeared almost completely without any complications. Usually JNA can be exclusively diagnosed by radiological study alone. So this report of successful treatment of JNA with GKS may provide an important clue for the novel indication of GKS.

Cellular angiofibroma of the vulva: case report with clinicopathological and immunohistochemistry study

CONTEXTO: O angiofibroma celular da vulva é um tumor raro que foi inicialmente descrito em 1997. Ocorre em mulheres de meia-idade (média de idade: 47 anos), apresentar pequeno tamanho (< 3 cm) e margem bem circunscrita. RELATO DE CASO: Descrevemos um caso em mulher de 51 anos de idade cujo diagnóstico pré-operatório foi confundido com cisto de glândula de Bartholin. A neoplasia era bem delimitada e constituída por três componentes característicos: células fusiformes formando pequenos fascículos, numerosos vasos sangüíneos e tecido adiposo entremeado às células fusiformes. As células do estroma eram positivas para vimentina e negativas para CD34, proteína S-100, actina e desmina. O diagnóstico diferencial deste distinto tumor inclui angiomixoma agressivo, angiomiofibroblastoma, lipoma, tumor fibroso solitário, perineurioma, e leiomioma.