A male, 21 years of age, without relevant history, presented with left exophthalmos and diplopia in extreme gaze positions with 2 weeks evolution, without clinic of epistaxis or nasal obstruction. Patients usually present with nasal obstruction and epistaxis. Longterm treatment outcomes of juvenile nasopharyngeal angiofibroma treated with radiotherapy risultati a lungo termine del trattamento radioterapico dell angiofibroma nasofaringeo giovanile s. Juvenile nasopharyngeal angiofibroma with orbital extension angiofibroma nasofaringeo juvenil con extension orbitaria. Although the use of nonsurgical therapies is described in the literature, surgery is currently considered the ideal treatment for. Juvenile nasopharyngeal angiofibroma extending into the oral. Juvenile nasopharyngeal angiofibroma electronic journal of. These features are compatible with juvenile angiofibroma, a benign but locally invasive tumor. The management of juvenile nasopharyngeal angiofibroma jna has changed during the last decades but it still continues to be a challenge for the multidisciplinary head and neck surgical team. Juvenile nasal angiofibroma jna is a rare sinonasal tumor that is found almost exclusively in adolescent and young adult males. Nasopharyngeal angiofibroma symptoms aurora health care. Juvenile nasopharyngeal angiofibroma is a rare, benign tumor that occurs most often in adolescent males. Pdf juvenile nasopharyngeal angiofibroma jna is a rare, benign, highly.
Angiofibroma nasofaringeo juvenil angel castro urquizo. Staging systems for juvenile nasopharyngeal angiofibroma. Two types of lateral extension in juvenile nasopharyngeal angiofibroma. Epidemiology juvenile nasopharyngeal angiofibromas. This uncommon lesion of the upper air passages has been a wellestablished entity for at least 100 years but its precise nature and origin are still subject to debate. Juvenile nasopharyngeal angiofibroma jna onset most commonly is in the second decade. The juvenile nasopharyngeal angiofibroma jna is a benign tumoral disease that may have a very aggressive behaviour, spreading from the nasopharynx to structures such as the cranial cavity, with a high morbidity.
Juvenile nasopharyngeal angiofibroma is a rare benign tumour of vascular origin found in adolescent males, originating around the sphenopalatine foramen. Juvenile angiofibroma jna is a benign tumor that tends to bleed and occurs in the nasopharynx of prepubertal and adolescent males. Juvenile nasopharyngeal angiofibromas are a rare benign, but locally aggressive, vascular tumors that occur almost exclusively in young men on imaging, they present as a vividly enhancing softtissue masses centered on the sphenopalatine foramen. We report the case of a 17yearold male who presented in 1995 with a mass filling the left posterior nasal cavity. Juvenile nasopharyngeal angiofibroma is a rare, highly. Jul 29, 2019 endoscopic and endoscopicassisted surgery for juvenile angiofibroma. Juvenile nasopharyngeal angiofibroma jna is a rare and benign but locally in the nasopharynx and nasal cavity, leading to manifestations such as nasal. Juvenile nasopharyngeal angiofibroma radiology reference. Juvenile nasopharyngeal angiofibroma praneeth koduru. Vessels are thinwalled, lack elastic fibers, have absent or incomplete smooth muscle, and vary in appearance from stellate or staghorn to barely conspicuous because of stromal compression.
Aug 15, 2019 angiofibroma nasofaringeo pdf introduction. Mohanti department of radiation oncology, all india institute of medical sciences, new delhi, india summary. The jna juvenile nasopharyngeal angiofibroma is a rare neoplasm, of benign histology, that affects almost exclusively male sex adolescent individuals. From 1962 to 1984, patients with juvenile nasopharyngeal angiofibroma jna were treated with megavoltage radiation therapy. A, pretreatment image shows encroachment of both the floor and lateral wall of the left sphenoid sinus and infratemporal fossa. It corresponds to 0,05% of all head and neck tumors and is, however, the most common nasopharynx benign tumor 1,2. Jnas are also reported to arise from the posterior aspect of the middle turbinate and rarely from other sites of the nasal cavity and in paranasal sinuses. Home acta otorrinolaringologica espanola comprar tratamiento quirurgico del angiofibroma nasofaringeo juvenil en pacientes pediatricos. Juvenile nasopharyngeal angiofibroma is an uncommon benign tumour of the nasopharynx which arises in the region of the sphenopalatine foramen and it grows insidiously invading surrounding structures. Juvenile nasopharyngeal angiofibroma evaluation and workup return to. Nasopharyngeal angiofibroma is a histologically benign but locally aggressive vascular tumor of the nasopharynx that arises from the superior margin of the sphenopalatine foramen and grows in the back of the nasal cavity. Pdf imaging in the diagnosis of juvenile nasopharyngeal. Radiologists prior knowledge on the tumors characteristics aids in establishing a diagnosis.
O angiofibroma nasofaringeo juvenil anj e um raro tumor em. Typical signs are nasal and eustachian tube obstruction, adenoidal speech, and dysphagia. Primary radiation therapy for juvenile nasopharyngeal angiofibroma. Mar 07, 2020 it most commonly affects adolescent males and may grow into fissures of the.
Juvenile nasopharyngeal angiofibroma jna is a disease of adolescent males characterised by. In addition, the term nasopharyngeal is often used instead of nasal because angiofibromas that occur. The juvenile nasopharyngeal angiofibroma is a benign neoplasm of the posterior nasopharynx it occurs almost exclusively in adolescent males, with an average age at diagnosis of 15 years old. Sep 01, 2008 juvenile nasopharyngeal angiofibroma is a rare, benign tumor that occurs most often in adolescent males. Abstract juvenile nasopharyngeal angiofibroma is a benign vascularized tumor, locally invasive, originated in the sphenopalatine foramen. Frequently, the term juvenile is not included in the name of this tumor because this lesion may occur in adults occasionally, as well as in adolescents. Nasal cavity, paranasal sinuses, nasopharynx nasopharyngeal angiofibroma. Jul 15, 2015 juvenile nasopharyngeal angiofibroma jna is a benign neoplasm of the nasopharynx. The typical patient is an adolescent male with a clinical history of recurrent epistaxis and nasal obstruction.
May 16, 2018 juvenile nasopharyngeal angiofibroma jna is a benign tumor that tends to bleed and occurs in the nasopharynx of prepubertal and adolescent males. It was first described by hippocrates in the 5 th century bc. The disease free period ranged from 10 to 36 months postoperatively. Nov 04, 2014 nasopharyngeal juvenile angiofibroma resected endoscopically. Article pdf available in journal of clinical imaging science. Given its vascularity, prominent flow voids are seen on mri leading to a salt and pepper appearance. Its tumorlike behavior causing obstruction of the airway and its fibrovascular structure giving rise to. Juvenile nasopharyngeal angiofibroma jna is a rare locally invasive neoplasm composed of cavernous vascular channels set in an abundant myxoid stroma of fibroblasts and myofibroblasts. The tumor contains many blood vessels, spreads within the area in which it. A case of angiofibroma limited to the right posterior nasal cavity, nasopharynx, and pterygopalatine fossa was operated upon transnasally under endoscopic. The management of juvenile angiofibroma ja has changed during the last decades but it still continues to be a challenge.
Juvenile nasopharyngeal angiofibromas are a rare benign, but locally aggressive, vascular tumors that occur almost exclusively in young men. Juvenile nasopharyngeal angiofibroma jna is an uncommon, slowgrowing, benign but locally invasive vascular tumor arising from tissues in the sphenopalatine foramen inside the nasal cavity. May 16, 2018 despite tumor reduction with hormones, this approach is not routinely used. Juvenile nasopharyngeal angiofibroma iowa head and neck. Juvenile angiofibroma rads see also with virtual slide. Angiofibroma nasofaring juvenile free download as pdf file. Seven cases of biopsyproved juvenile nasopharyngeal angiofibroma are presented.
A metaanalysis of the different kinds of treatment used is reported. It was friedberg who first used the term angiofibroma in 1940. It most commonly affects adolescent males and may grow into fissures of the. Jnas originate from the posterior choanal tissues and rapidly extend into the surrounding regions, including the nasopharynx, the orbits, and even the intracranial cavity. Introduction uncommon, benign and extremely vascular tumour up to 0. The patient underwent preoperative embolization and an uneventful resection. It is a rare, benign, vascular neoplasm that accounts for less than 0. Nasopharyngeal juvenile angiofibroma resected endoscopically.
Their use is purely for academic and teaching purposes. Juvenile nasopharyngeal angiofibroma jna is a rare, benign, but locally aggressive tumor occurring almost exclusively in adolescent males. Intricate mixture of stellate and staghorn blood vessels with variable vessel wall thickness ranging from single layer of endothelium to variable smooth muscle coat. Head and neck longterm treatment outcomes of juvenile. Juvenile nasopharyngeal angiofibroma is a vascular tumor that. They concluded that juvenile angiofibroma jna growth and vascularization are driven by factors released from stromal fibroblasts. Juvenile nasopharyngeal angiofibroma jna is a rare and benign but locally aggressive fibrovascular tumor arising from the posterolateral wall of the. Despite tumor reduction with hormones, this approach is not routinely used. Nasopharyngeal angiofibroma is usually encapsulated and composed of vascular tissue and fibrous stroma with coarse or fine collagen fibers. The tumor contains many blood vessels, spreads within the area in which it started locally invasive, and can cause bone damage. None of these patients required postoperative nasal packing, and all were discharged the day after surgery. Juvenile nasopharyngeal angiofibroma knowledge for medical. Juvenile nasopharyngeal angiofibroma jna is a rare tumor that exhibits a predictable spreading pattern.
Longterm treatment outcomes of juvenile nasopharyngeal. Endoscopic removal of a juvenile nasal angiofibroma ucla duration. They often grow quickly during puberty, then slow down or stop growing entirely after adolescence. The pictures used in this presentation have been obtained from a number of sources. Le fibrome nasopharyngien fnp est une tumeur rare qui represente 0. Jul 16, 2019 the disease free period ranged from 10 to 36 months postoperatively. Features are characteristic of a juvenile angiofibroma.
Juvenile nasopharyngeal angiofibroma jna is a rare vascular. Surgical resection of juvenile angiofibroma vula university of. The tumor usually arises in puberty and is more common in boys than in girls. Visual acuity was 2020 in both eyes and intraocular pressure iop 24 mmhg in left eye le without limitations in ocular movement. Transnasal endoscopic surgery in juvenile nasopharyngeal. Jnas occur almost exclusively in the nasopharynx of adolescent. Nasoangiofibroma youth is a highly vascularized tumor almost exclusively male adolescents presentation. Schuon et al reported on the immunohistochemical analysis of growth mechanisms in juvenile nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibroma is a benign but locally invasive tumor accounting for about 0. Jna is an uncommon, highly vascular, locally invasive, unencapsulated wngiofibroma with a distinct predilection for an origin in the nasopharynx of adolescent males. Juvenile nasopharyngeal angiofibroma jna is a rare tumor in adolescent males.
The free informed consent form was not needed in this context. It is the most common benign tumor of the nasopharnyx but only accounts for less than 0. Juvenile nasopharyngeal angiofibroma is a rare, highly luiz a cr, romualdo s lt, fava a s. Request pdf juvenile nasopharyngeal angiofibroma juvenile. We aimed to report the characteristic magnetic resonance imaging mri findings and the spread patterns of jna. Juvenile nasopharyngeal angiofibroma jna is a rare and benign but locally in the nasopharynx and nasal. The juvenile nasopharyngeal angiofibroma is a benign neoplasm of the posterior. The patients age and gender, as well as the geographic area. Nasopharyngeal angiofibroma introduction juvenile nasopharyngeal angiofibroma jna is a benign, but locally aggressive and extremely vascular head and neck neoplasm, occurring almost exclusively in the nasopharynx of adolescent males. As the tumor is largely spaceoccupying and highly vascular, patients typically. Juvenile angiofibroma is a rare benign lesion originating from the pterygopalatine fossa with distinctive epidemiologic features and growth patterns. Common practice is to excise the tumor with open or endoscopic surgery.
Juvenile angiofibroma on coronal contrastenhanced magnetic resonance mr images obtained before and after endoscopic resection. Genetic evidence that juvenile nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibroma jna is a rare tumor that predominantly occurs in adolescent males, the average age of occurence being 15. Oct 04, 2019 epidemiology juvenile nasopharyngeal angiofibromas. Juvenile nasopharyngeal angiofibroma jna is a benign neoplasm of the nasopharynx. Juvenile nasopharyngeal angiofibroma with orbital extension. However, this case is not typical as it doesnt invade the nasal fossae. A sevenyear experience with patients with juvenile nasopharyngeal. It most commonly affects adolescent males because it is a hormonesensitive tumor.
Examination under anesthesia and tomography and angiography were very rewarding in determining the full extent of tumor. On imaging, they present as a vividly enhancing softtissue masses centered on the sphenopalatine foram. A juvenile nasopharyngeal angiofibroma jna is a noncancerous tumor made up of blood vessels that grow at the posterior aspect of the nose. It is not as invasive as to surgery that is almost free of. Nasopharyngeal angiofibroma definition of nasopharyngeal. Adolescents and young adults between 14 and 25 years are affected, and there is a distinct male predominance. Juvenile nasopharyngeal angiofibroma radiology case. Review article nasopharyngeal angiofibroma ashutosh hota s.
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