Juvenile nasopharyngeal angiofibroma staging: An overview.

Staging of tumors is very important in treatment and surgical decision making, as well as in predicting disease recurrence and prognosis. This review focuses on the different available classifications of juvenile nasopharyngeal angiofibroma (JNA) and their impact on the evaluation, management, and prognosis of JNA. The literature was reviewed, and publications on JNA staging were examined. Our MEDLINE search of the entire English-language literature found no review article on the current available staging systems for JNA. In this article, we review the common JNA classification systems that have been published, and we discuss some of their advantages and disadvantages. The most commonly used staging systems for JNA are the Radkowski and the Andrews-Fisch staging systems. However, some newer staging systems that are based on advances in technology and surgical approaches-the Onerci, INCan, and UPMC systems-have shown promising utility, and they will probably gain popularity in the future.

Nasopharyngeal angiofibroma: a concise classification system and appropriate treatment options.

OBJECTIVES: To describe a clear and simplified classification system for juvenile nasopharyngeal angiofibroma (JNA), and to describe suitable management options. STUDY DESIGN: Retrospective medical record review. METHODS: The clinical and imaging materials of 51 cases of JNA diagnosed at our hospital between 1981 and 2011 were collected and studied. Based on our experiences, we prefer to divide JNAs into three types. Type I includes JNAs fundamentally localized to the nasal cavity, paranasal sinus, nasopharynx, or pterygopalatine fossa. Type II is a JNA extending into the infratemporal fossa, cheek region, or orbital cavity, with anterior and/or minimal middle cranial fossa extension but intact dura mater. Type III is a calabash-like massive tumor lobe in the middle cranial fossa. The management and prognosis for the three types of JNA were compared and evaluated. RESULTS: Among cases of type I JNA (n=16), the entire mass was removed by the initial operation in 15 cases and by a repeat operation in 1 case. Among cases of type II JNA (n=29), the entire mass was removed by the first operation in 24 cases and by repeat operation in 5 cases. In cases of type III JNA (n=6), the huge calabash-like lobe in the middle cranial fossa could not be completely excised; 4 cases underwent radiotherapy and 2 cases were lost to follow-up. CONCLUSIONS: 1) The transnasal cavity approach with endoscopic guidance is suitable for type I JNA resection. 2) The transantral-infratemporal fossa-nasal cavity combined approach is reliable for resection of a type II JNA, which extends into the deep anterior cranial fossa and/or minimally into the middle cranial fossa, with intact dura mater. 3) The complete removal of a type III JNA is difficult, even through a combined extracranial and intracranial approach. Radiotherapy is useful for treating the residual intracranial tumor. The successful or failed experiences of 6 typical cases prove that this revised classification system is reasonable and reliable.

Le Fort I access for juvenile nasopharyngeal angiofibroma (JNA): a prospective series of 22 cases.

INTRODUCTION: The mainstay treatment for Juvenile Nasopharyngeal Angiofibroma (JNA) is complete excision. Surgical approach should allow maximal exposure of the tumour with minimum morbidity. One such possible approach is Lefort I maxillary osteotomy. OBJECTIVE: To review our experience on feasibility of Lefort I osteotomy approach to achieve the best result and to look at the outcome in our series of patients at a tertiary centre in India. METHOD: This prospective study involves 22 patients with JNA who have been treated by Lefort I approach between August 2006 and December 2007 at the Department of Neurosurgery, National Institute of Mental Health And Neurosciences, Bangalore, India. RESULTS: All patients underwent primary surgical resection through Lefort I approach. No major intra-operative and post-operative complications were noted except in one patient where loss of vision in one eye due to prior pre-operative orbital involvement. The mean follow-up period was 2.8 years. To date, there has been no cases of residual tumour or recurrence that can be attributed to the procedure except in one case. CONCLUSION: Our experience suggests that the Lefort I osteotomy approach is a useful technique for the removal of extensive JNA which has distinct advantages over traditional approaches, providing a more direct vision, improved exposure, and cosmesis.

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.

[Solitary fibrous tumor and haemangiopericytoma: what is new?]. / Solitärer fibröser Tumor und Hämangioperizytom : Was ist neu?

Soft-tissue tumors with haemangiopericytoma (HPC)-like growth patterns can now be divided into three categories: (1) The solitary fibrous tumour (SFT) group with its variants; (2) lesions showing clear evidence of myoid/pericytic differentiation and corresponding to "true" HPCs (myopericytoma/glomangiopericytoma and a subset of sinonasal HPCs); (3) neoplasms that occasionally display HPC-like features (e.g. synovial sarcoma). In this study 268 intrathoracic and extrathoracic SFTs from the German consultation and reference center of soft tissue tumors in Jena were evaluated and analyzed immunohistochemically with antibodies CD34, Bcl-2, CD99, SMA, S100, PanCK and Ki-67. Furthermore, SFTs were categorized into the newly proposed SFT designation: Fibrous variant, cellular variant (more than 90% hypercellularity), fat-forming variant, giant cell-rich variant and malignant SFTs. This article should provide insights into the diagnosis of this entity with emphasis on the new international standard.

Seguimiento a largo plazo de angiofibromas tratados con láser de CO2 en 23 pacientes con esclerosis tuberosa / Long-term follow up of angiofibromas treated with CO2 laser in 23 patients with tuberous sclerosis

Introducción. La esclerosis tuberosa es una enfermedad autosómica dominante que determina la formación de hamartomas en múltiples órganos. Los cambios cutáneos son una de las características primarias de esta enfermedad. Dentro de éstos, los angiofibromas constituyen una forma común de presentación que causa importantes problemas cosméticos y médicos. El láser de CO2 se ha utilizado satisfactoriamente en el tratamiento de estas lesiones, pero existen escasos estudios que evalúen sus resultados a largo plazo. El objetivo de nuestro estudio es valorar la respuesta del tratamiento de los angiofibromas en el largo plazo. Métodos. Se realizó un estudio retrospectivo en 23 pacientes con angiofibromas tratados con láser de CO2. Los pacientes fueron tratados entre los años 1991 y 2000 inclusive con láser de CO2 continuo o superpulsado. Clasificamos los angiofibromas de acuerdo a su tamaño, resultados iniciales del tratamiento y edad de los pacientes (< 20 años y 20 años o más). Resultados. El rango de edad varió entre 12 y 39 años, con una media de 22,5 años. Después del tratamiento los pacientes fueron controlados durante un periodo de 6 meses a 10 años. En el análisis a largo plazo encontramos que un 30,1 % mantuvo el resultado inicial y el 60,9 % mostró diferentes grados de recidiva con un tiempo medio de recurrencia de 3 años. Cuando analizamos los resultados a largo plazo según tamaños de angiofibromas, resultado inicial y edad de los pacientes, no encontramos diferencias estadísticamente significativas entre los diferentes grupos. El análisis de supervivencia, con curvas de Kaplan-Meier, de los grupos de edad mostró que los pacientes más jóvenes (< 20 años) recayeron antes que los de mayor edad (rango logarítmico 4,01 y p < 0,05). Conclusiones. El tratamiento con láser de CO2 logra buenos resultados a corto plazo. Por otro lado, uno de los mayores problemas es la recaída en el largo plazo, ya que probablemente debido a la naturaleza de las lesiones, éstas no se pueden eliminar permanentemente. Este trabajo coincide con estudios previos que no han encontrado factores que permitan predecir la recurrencia de las lesiones. Sin embargo, podemos concluir que los pacientes de mayor edad recaen más tardíamente y tienen por tanto mejores resultados cosméticos a largo plazo

Introduction. Tuberous sclerosis is an autosomal dominant disease in which hamartomas form in multiple organs. Cutaneous changes are one of the primary characteristics of this disease. These include angiofibromas (AF), a common form of presentation that causes significant cosmetic and medical problems. The CO2 laser has been used satisfactorily in treating these lesions, but there are few studies that evaluate its long-term results. The aim of our study is to assess the long-term response of the treatment of angiofibromas. Methods. A retrospective study was carried out on 23 patients with angiofibromas treated with CO2 laser. The patients were treated between 1991 and 2000, inclusive, with continuous or superpulsed CO2 laser. We classified the angiofibromas by size, initial treatment results and patients' ages (< 20 years and 20 years or older). Results. Ages ranged from 12 to 39 years, with a median age of 22.5 years. After treatment, patients were followed up for a period of six months to 10 years. In the long-term analysis, we found that 30.1 % maintained the initial result, and 60.9 % showed different degrees of recurrence, with a mean recurrence time of 3 years. When we analyzed the long-term results by the size of the angiofibromas, initial result and patients' ages, we found no statistically significant differences among the different groups. The survival analysis of the age groups, with Kaplan-Meier curves, showed that the youngest patients (< 20 years) had earlier recurrences than the older ones (logarithmic range 4.01 and p < 0.05). Conclusions. CO2 laser treatment achieves good short-term results. On the other hand, one of the biggest problems is recurrence over the long term; this is probably due to the fact that, because of their nature, these lesions cannot be eliminated permanently. This work coincides with earlier studies which found no factors that would make it possible to predict the recurrence of the lesions. However, we can conclude that recurrence takes place at a later date in older patients, and therefore they have better cosmetic results over the long term

Angiomyxoid tumor with an intermediate feature between cellular angiofibroma and angiomyofibroblastoma in the male inguinal region.

Angiomyofibroblastoma (AMFB) and cellular angiofibroma (CA) are angiomyxoid tumors which infrequently arise in the female vulvovagina and each has been proposed to be a distinct clinicopathological entity. The former in male genitalia is exceedingly rare and has been described as its male analog or under the name of male AMFB-like tumor, while the latter in men has not been reported. We describe an angiomyxoid tumor which appeared in the inguinal region of 72-year-old man. The present case had a histopathological characteristic and immunophenotype intermediate between AMFB and CA. Male genital angiomyxoid tumors share many immunopathological features with their female counterparts, suggesting that they are male homologs rather than analogs. Immature mesenchymal cells with a potential of the multilineage differentiation might be promoted toward neoplastic myoblasts, fibroblasts and adipocytes, resulting in genital angiomyxoid tumors showing a broad spectrum in the immunopathological phenotype.

Angiofibromas juveniles nasofaríngeos / Youthful rhinofaringeal angiofibromas

Los angiofibromas juveniles nasofaríngeos son los tumores nasofaríngeos benignos más frecuentes. Se presentan predominantemente en varones púberes. Los síntomas más frecuentes son obstrucción nasal y epistaxis recurrentes. A pesar de que su comportamiento biológico es benigno, tiende a crecer destruyendo las estructuras óseas vecinas a la nasofaringe hasta extenderse a la base del cráneo. Recurren con una frecuencia que va del 35 al 57 por ciento después de la extirpación quirúrgica incompleta. El diagnóstico diferencial debe hacerse con hemangiopericitoma, tumor fibroso solitario, hemangiomas, schwannoma e histiocitoma fibroso

Nasoangiofibroma juvenil: revisión y experiencia de 21 Años en el Instituto Nacional de Cancerología

Objetivos. Describir el manejo y seguimiento de pacientes con nasoangiofibroma juevenil en el Instituto Nacional de Cancerología así como sus complicaciones; establecer criterios de evaluación y seguimiento de pacientes con nasoangiofibroma juvenil con términos similares a los establecidos en oncología y comparar las clasificaciones de Fisch y Chandler por estados. Materiales y Métodos. El manejo estadístico de la información se realizó con el programa EPI-INFO versión 6.0 de mayo de 1994. La significancia estadística se finó en un alfa de 0.05 y para la comparaciónde promedios se utilizó el test de "t". Resultados. La mediana para el tiempo de evolución de los signos y síntomas en esta revisión fue de 12 meses (rango: 2 a 96). Se realizaron 25 arteriografías diagnósticas demostrando variabilidad en el vaso nutricio tumoral y 28 embolizaciones con Gelfoan; todos los pacientes fueron divididos por estados según las clasificaciones de Fisch y Chandler; 59 pacientes fueron llevados a cirugía y uno radioterapia como tratamiento primario, después del cual 43 (71.7 por ciento) quedaron libres de enfermedad y 17 (28.3 por ciento) con persistencia tumoral. La mediana para el tiempo global libre de enfermedad fue de 13.5 meses y para el seguimiento de los pacientes fue de 32.8 meses. Conclusiones: No se presentaron diferencias significativas entre las clasificaciones de Chandler y Fisch; la de Sessions es poco práctica. La falta de definiciones claras sobre la respuesta al tratamiento nasoangiofibroma juvenil hace menos objetivos y comparables los resultados de los estudios

Recent developments in soft tissue tumors.

The field of soft tissue tumor pathology, despite the relative rarity of the lesions concerned, has grown considerably in recent years and has featured very prominently in the academic pathology literature. This stems from the facts that increasing diagnostic sophistication has allowed the continued refinement of classification schemes and the delineation of numerous previously unrecognized 'entities' and that the application of modern cytogenetic and molecular genetic techniques has revealed the remarkable importance of reproducible cytogenetic aberrations in our understanding of the pathobiology of this group of neoplasms. This presentation will aim to summarize data regarding some of the recently characterized entities as well as the major molecular genetic advances. Newly characterized 'entities' which are important because of the ease with which they are confused with lesions of very different biologic potential include chondroid lipoma, pleomorphic hyalinising angiectatic tumor, extrapleural solitary fibrous tumor, giant cell angiofibroma, cellular angiofibroma, retiform hemangioendothelioma, composite hemangioendothelioma and low grade fibromyxoid sarcoma. The 'hemangioendotheliomas' also represent an important area of conceptual evolution as we begin to more critically analyze the concept of borderline malignancy. Concerning molecular genetic advances, the most striking data to emerge are the frequency of tumour-specific reciprocal translocations, the frequency of biologically revealing cytogenetic aberrations in benign lesions and the emerging evidence of striking differences in cell cycle abnormalities in the different sarcoma types.

Nasoangiofibroma: Conceptos actuales y revisión sobre su diagnóstico y tratamiento / Juvenil angiofibroma of the nasopharinx: Current concepts and review about its diagnosis and treatment

El nasoangiofibroma conocido también como angiofibroma nasofaríngeo juvenil es el tumor benigno más frecuente de la nasofaringe. Por su histología benigna con comportamiento clínico agresivo y destructivo local amplia se le clasifica dentro de los tumores de malignidad intermedia. Debido a la localización de su sitio de implantación en la nasofaringe con extensión frecuente a estructuras de la base del cráneo y ocacionalmente intracraneal requiere de un diagnóstico clínico y de imagenología preciso. El tratamiento de elección es el quirúrgico. Por la localización de su base de implantación en el receso esfenoetmoidal, debe elegirse una vía de abordaje adecuada y segura que permite lograr una exposición amplia y resección completa