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.

A comparison between Different Surgical Approaches of Nasopharyngeal Angiofibroma: A Cross-Sectional Observational Study in BSMMU, Bangladesh

Background: The Otolaryngologist is currently dealing with a dramatic problem the nasopharyngeal angiofibroma. The posterolateral wall of the nasal cavity, close to the superior margin of the sphenopalatine foramen, is where the tumor commonly first appeared.Material & Methods:The Otolaryngology & Head Neck Surgery department at Bangabandhu Shiekh Mujib Medical University, Dhaka Medical College Hospital, and Shaheed Suhrawardy Medical College Hospital in Dhaka undertook this retrospective cross-sectional study. The research was done from February 2012 to November 2012. The study’s overall sample size was 30.Results:The majority of individuals (54%) who have nasopharyngeal angiofibroma are between the ages of 16 and 20. the majority of patients have similar symptoms such epistaxis, nasal blockage, nasal discharge, anemia, and masses in the nasal cavity. At presentation, anaemia is present to various degrees in 90% of cases. The lateral rhinotomy method is employed (40%) more often than other approaches. Three blood units were to be transfused into a maximum of 36.66% of the patients throughout the surgery.Conclusion:Nasopharyngeal angiofibroma is currently a difficult issue for otolaryngologists. When endoscopy was used instead of open techniques, we discovered that the mean length of the procedure was shorter, which may be related to endoscopy’s decreased morbidity.

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 celular mesenquimatoso vulvar: reporte de un caso y revisión bibliográfica / Vulvar mesenchymal cell angiofibroma: A case report and literature review

Resumen: ANTECEDENTES: El angiofibroma celular (neoplasia mesenquitamosa) es un tumor poco frecuente conformado por células fusiformes y estructuras vasculares. Los angiofibromas celulares son neoplasias pequeñas, delimitadas, que pueden aparecer en mujeres de 40 a 50 años. CASO CLÍNICO: Paciente de 15 años, sin antecedentes patológicos de importancia para el padecimiento actual, que acudió al servicio médico debido a la aparición de una masa vulvar de crecimiento rápido, de 1 año de evolución. En la exploración física se observó una tumoración vulvar de 10 x 4 cm, en forma de herradura y en el contorno de la vulva se visualizó otra tumoración dependiente de la anterior, en el clítoris, de 7 x 5 cm, que simulaba un falo; ambas estaban adheridas a los planos profundos, sin causar dolor a la palpación, móviles, circunscritas, de consistencia blanda, con crecimiento de vello corporal en posición androgénica. Se llevó a cabo la resección del tumor; el procedimiento se inició con el acceso al labio mayor, sin afectar el meato uretral y el clítoris; se observó aumento de la vascularidad y la tumoración se disecó en su totalidad. El reporte histopatológico fue de angiofibroma celular. La evolución de la paciente fue favorable. CONCLUSIÓN: El angiofibroma celular de la vulva es una neoplasia mesenquimatosa con características clínicas, morfológicas e inmunohistoquímicas propias. El caso aquí expuesto es el primero reportado en una paciente adolescente.

Abstract: BACKGROUND: Cellular angiofibroma is a very rare tumor, it was described as a mesenchymal neoplasm, mainly made up of two components: spindle cells and vascular structures. Cellular angiofibromas are small-delimited malignancies and characteristically occur in women between 40 to 50 years of age. CLINICAL CASE: A 15-year-old female patient, with no pathological history of importance for the current condition, went to the medical unit due to the presence of a 1-year evolution vulvar mass, which was growing rapidly, 10 x 4 cm vulvar mass, horseshoe-shaped bearing the contour of the vulva, another tumor dependent on the previous in the clitoris area (7 x 5 cm), simulating a phallus, fixed to deep planes, not painful on palpation, mobile, circumscribed, of soft consistency, with growth of body hair in an androgenic position. In the surgical event, a tumor resection was performed, beginning an approach by the external part of the majors labia, respecting the urethral meatus and clitoris, an increase in vascularity was observed, dissecting the tumor in its entirety. The histopathological report was of cellular angiofibroma. The evolution of the patient was favorable. CONCLUSION: The cellular angiofibroma of the vulva is a mesenchymal neoplasm with the own clinical, morphological and immunohistochemical characteristics. The case presented is the first identity developed in an adolescent patient.

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.

Láser CO2 ablativo en angiofibroma facial por esclerosis tuberosa / Ablative laser CO2 in facial angiofibroma due to tuberous sclerosis

Se presenta el caso de un paciente de 25 años con múltiples pápulas y placas de aspecto fibroso, distribuidas principalmente en cara, además de cuero cabelludo, tórax y región periungueal pedia, asintomático. Se realizó el diagnóstico clínico e histopatológico de esclerosis tuberosa, enfermedad genética poco frecuente. Se utilizó láser CO2 modo ablativo en 2 ciclos de dos sesiones cada una, en lesiones cutáneas faciales (angiofibroma facial), con el objetivo de mejorar la apariencia y promover calidad de vida. Como resultado se evidenció mejoría de la apariencia y ausencia de recidiva con un método terapéutico moderno y aún inaccesible a todas las personas en nuestro país.

We present the case of a 25-year-old patient with multiple fibrous papules and plaques, mainly distributed on the face, as well as asymptomatic scalp, thorax and peduncle periungueal region. The clinical and histopathological diagnosis of tuberous sclerosis was performed, a rare genetic disease. CO2 laser was used ablative mode in 2 cycles of two sessions each, in facial skin lesions (facial angiofibroma), with the aim of improving the appearance and promoting quality of life. As a result, improvement in the appearance and absence of recurrence was evidenced by a modern therapeutic method that is still inaccessible to all people in our country.

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.

​Clinical Review Of Juvenile Nasopharyngeal Angiofibroma In Urban Tertiary Care Centre: A Retrospective Analysis

Introduction: The aim of this study was to review management, surgical approaches used, blood loss, complications, andrecurrence rate of juvenile nasopharyngeal angiofibroma (JNA) in our institution during 2002–2005 period.Materials and Methods: All patients referred for a JNA were included in the study. Medical files and imaging data wereretrospectively analyzed. Surgical management was then evaluated consecutive patients operated on from April 2002 to June2005. Case series with chart review. The study was conducted from data on patients operated from April 2002 to 2005 in MadrasMedical College and Government General Hospital.Results: A total of 42 patients were operated on, with a mean age of 16.8 years (range, 9–31 years). In the endoscopic approachblood loss was found to be <300 ml. With Weber Ferguson trans palatine extensive tumor with proptosis blood loss was >1000 mlembolization has reduced the intraoperative blood loss. Since they explored pterygopalatine fossa by removing,posterior partof medial wall and posterior wall completely, recurrence was rare.Conclusion: Progress in skull base anatomy, instrumentation, cameras, and surgical strategy allows for expansion of theindications for endoscopic removal of JNA. This approach may have a better outcome in terms of blood loss, hospital stay, andlocal sequelae. Still, an external approach should be considered only for selected cases due to a massive intracranial extensionor optic nerve or internal carotid artery entrapment by the tumor.

Low-temperature plasma radiofrequency in nasal endoscopic surgery for juvenile nasopharyngeal angiofibroma / 医学研究生学报

Objective The aim of this article was to investigate the effect of nasal endoscopic surgery (NES) using low-temperature plasma radiofrequency (LTPRF) in the treatment of juvenile nasopharyngeal angiofibroma (JNA). Methods We retrospectively analyzed the clinical data on 38 cases of JNA treated by NES+LTPRF (n = 26) or conventional surgical resection (control, n = 12) in Drum Tower Hospital of Nanjing University School of Medicine from January 2012 to December 2018. We compared the operative time, postoperative Lund-Mackay endoscopy scores (LMES), incidence of complications, postoperative recovery, rate of recurrence, and Visual Analogue Scale (VAS) scores between the two groups of patients. Results The rates of LMES gradesⅠ and Ⅱ were significantly higher while those of grades Ⅲ and Ⅳ remarkably lower in the NES+LTPRF than in the control group (P < 0.05). Compared with the controls, the patients of the NES+LTPRF group showed markedly shorter time of nasal mucosa epithelialization ([3.5 ± 0.1] vs [2.3 ± 0.1] mo, P < 0.05) and lower rates of postoperative complications (38.5% vs 7.7%, P < 0.05), recurrence (16.7% vs 3.8%, P < 0.05), and VAS scores at 3 days, 2 months, 6 months and 12 months after surgery (P < 0.05). Conclusion NES+LTPRF is an effective strategy for the treatment of juvenile nasopharyngeal angiofibroma and worthy of a wide clinical application.

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.

Nasoangiofibroma Juvenil vs Hemangioma Arteriovenoso, Reto diagnóstico / Nasal Juvenile Angiofibroma vs Arteriovenous Hemangioma, Diagnosis Challenge. Case report

El nasoangiofibroma juvenil es un tumor vascular benigno, localmente agresivo pero poco común, representa tan solo el 0,05% de todos los tumores de cabeza y cuello, con una incidencia de aproximadamente 1:150.000. Por otra parte los hemangiomas son neoplasias benignas de las células endoteliales, representa el 7% de los tumores vasculares y son lesiones poco común de cabeza y cuello, pero ¿Son una misma entidad?, ¿Se puede trazar una línea clara para diferenciarlos?. El objetivo del presente articulo es proporcionar información acerca de estas dos entidades clínicas y responder las preguntas previamente formuladas. Este es el caso clínico de un paciente del género masculino de 19 años de edad, quien consultó por cefalea hemicraneana izquierda, otalgia y sensación de opresión ocular ipsilateral de 7 días de evolución. La tomografía axial computarizada de cráneo mostró una masa de densidad de tejidos blandos que ocupa la coana izquierda, infiltra parcialmente la región posterior de la fosa nasal izquierda, erosiona la apófisis pterigoides y el suelo de la celda esfenoidal izquierda. Se realizó biopsia por medio de nasofibrolaringoscopia en el consultorio, previa a infiltración con lidocaína, se toman dos muestras de lesión en coana izquierda sin sangrado significativo; el reporte patológico informa hemangioma arteriovenoso, por lo que se decidió realizar embolización pre quirúrgica; posteriormente es llevado a procedimiento quirúrgico sin complicaciones, finalmente la biopsia excisional reporta nasoangiofibroma juvenil con estudio de inmunohistoquimica CD31 y CD34 reactivo en pared de estructurasvasculares. El nasoangiofibroma juvenil es el tumor más común de la nasofaringe en hombres adolescente, la ausencia de epistaxis recurrente unilateral no debe descartar el diagnostico..(AU)

Nasal Juvenile angiofibroma is a benign vascular tumor, locally aggressive but uncommon, it only represents 0,05% of all head and neck tumors, with an approximate incidence of 1:150-000. On the other hand hemangiomas are benign neoplasms of endothelial cells, they represent 7% of vascular tumors and they are uncommon lesions of the head and neck but Are they the same entity?, Can a clear line be drawn to differentiate them?. The objective of this article is to provide information about these two clinical entities and answer the questions previously asked. This is the clinical case of a 19 year old male, that consulted for left headache, left earache and left ocular oppression of 7 days of evolution. Brain axial constrast computed tomography showed a mass of soft tissue density that occupies the left coana, partially infiltrates the posterior region left nasal fossa, erodes the pterygoid process and the floor of left the sphenoid cell. Biopsy was performed by means of nasofibrolaryngoscopy in the office, previos to infiltration with lidocaine, two samples of lesion were taken in the left choana without significant bleeding; the pathological report informs arteriovenous hemangioma. it was decided to perform a pre-surgical embolization. After this, the surgical procedure was carried out without complications. Finally, the excisional biopsy reported a juvenil nasoangiofibroma with immunohistochemical study of CD31 and CD34 reactive in wall of vascular structures. Nasal Juvenile Nasoangiofibroma is the most common tumor of the nasopharynx in adolescent males, the absence of recurrent unilateral epistaxis should not rule out the diagnosis..(AU)

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)