Juvenile nasopharyngeal angiofibroma radiology reference. Juvenile nasopharyngeal angiofibroma knowledge for. Jnas occur almost exclusively in the nasopharynx of adolescent. Patients usually present with nasal obstruction and epistaxis. Although the exact pathogenesis of the tumour is not yet known, natural history and growth patterns can be predicted. It was first described by hippocrates in the 5 th century bc. These features are compatible with juvenile angiofibroma, a benign but locally invasive tumor. 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.
In addition, the term nasopharyngeal is often used instead of nasal because angiofibromas that occur. Visual acuity was 2020 in both eyes and intraocular pressure iop 24 mmhg in left eye le without limitations in ocular movement. Jul 16, 2019 the disease free period ranged from 10 to 36 months postoperatively. 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 radiology case. Typical signs are nasal and eustachian tube obstruction, adenoidal speech, and dysphagia. Mohanti department of radiation oncology, all india institute of medical sciences, new delhi, india summary. We aimed to report the characteristic magnetic resonance imaging mri findings and the spread patterns of jna. Juvenile nasopharyngeal angiofibroma jna is a rare tumor that exhibits a predictable spreading pattern. A juvenile nasopharyngeal angiofibroma jna is a noncancerous tumor made up of blood vessels that grow at the posterior aspect of the nose. The tumor usually arises in puberty and is more common in boys than in girls.
Review article nasopharyngeal angiofibroma ashutosh hota s. Juvenile angiofibroma on coronal contrastenhanced magnetic resonance mr images obtained before and after endoscopic resection. The patient underwent preoperative embolization and an uneventful resection. Juvenile nasopharyngeal angiofibroma jna is a rare and benign but locally aggressive fibrovascular tumor arising from the posterolateral wall of the.
Home acta otorrinolaringologica espanola comprar tratamiento quirurgico del angiofibroma nasofaringeo juvenil en pacientes pediatricos. Head and neck longterm treatment outcomes of juvenile. Juvenile nasopharyngeal angiofibroma with orbital extension. Juvenile nasopharyngeal angiofibroma pubmed central pmc. A sevenyear experience with patients with juvenile nasopharyngeal. It is a rare, benign, vascular neoplasm that accounts for less than 0. Staging systems for juvenile nasopharyngeal angiofibroma. Angiofibroma nasofaringeo juvenil angel castro urquizo.
Seven cases of biopsyproved juvenile nasopharyngeal angiofibroma are presented. Nov 04, 2014 nasopharyngeal juvenile angiofibroma resected endoscopically. Angiofibroma nasofaring juvenile free download as pdf file. Juvenile angiofibroma is a rare benign lesion originating from the pterygopalatine fossa with distinctive epidemiologic features and growth patterns. On imaging, they present as a vividly enhancing softtissue masses centered on the sphenopalatine foram. Juvenile angiofibroma jna is a benign tumor that tends to bleed and occurs in the nasopharynx of prepubertal and adolescent males. Its tumorlike behavior causing obstruction of the airway and its fibrovascular structure giving rise to. 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. Pdf juvenile nasopharyngeal angiofibroma jna is a rare, benign, highly. It most commonly affects adolescent males because it is a hormonesensitive tumor. Intricate mixture of stellate and staghorn blood vessels with variable vessel wall thickness ranging from single layer of endothelium to variable smooth muscle coat. The free informed consent form was not needed in this context.
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. Mar 07, 2020 it most commonly affects adolescent males and may grow into fissures of the. Given its vascularity, prominent flow voids are seen on mri leading to a salt and pepper appearance. Examination under anesthesia and tomography and angiography were very rewarding in determining the full extent of tumor. However, this case is not typical as it doesnt invade the nasal fossae.
Nasal cavity, paranasal sinuses, nasopharynx nasopharyngeal angiofibroma. From 1962 to 1984, patients with juvenile nasopharyngeal angiofibroma jna were treated with megavoltage radiation therapy. Adolescents and young adults between 14 and 25 years are affected, and there is a distinct male predominance. Juvenile nasopharyngeal angiofibroma jna is a rare tumor in adolescent males. The patients age and gender, as well as the geographic area. 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 electronic journal of. Juvenile angiofibroma rads see also with virtual slide. Juvenile nasopharyngeal angiofibroma separation of v2.
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. Juvenile nasopharyngeal angiofibroma jna is a rare vascular. It corresponds to 0,05% of all head and neck tumors and is, however, the most common nasopharynx benign tumor 1,2. Nasopharyngeal angiofibroma definition of nasopharyngeal. Genetic evidence that juvenile nasopharyngeal angiofibroma. It is not as invasive as to surgery that is almost free of.
Oct 04, 2019 epidemiology juvenile nasopharyngeal angiofibromas. Longterm treatment outcomes of juvenile nasopharyngeal. Jna is an uncommon, highly vascular, locally invasive, unencapsulated wngiofibroma with a distinct predilection for an origin in the nasopharynx of adolescent males. Sep 01, 2008 juvenile nasopharyngeal angiofibroma is a rare, benign tumor that occurs most often in adolescent males. Schuon et al reported on the immunohistochemical analysis of growth mechanisms in juvenile nasopharyngeal angiofibroma. Request pdf juvenile nasopharyngeal angiofibroma juvenile. Jnas originate from the posterior choanal tissues and rapidly extend into the surrounding regions, including the nasopharynx, the orbits, and even the intracranial cavity. Juvenile nasopharyngeal angiofibroma is a rare, benign tumor that occurs most often in adolescent males. Radiologists prior knowledge on the tumors characteristics aids in establishing a diagnosis.
It was friedberg who first used the term angiofibroma in 1940. A metaanalysis of the different kinds of treatment used is reported. Jul 29, 2019 endoscopic and endoscopicassisted surgery for juvenile angiofibroma. Epidemiology juvenile nasopharyngeal angiofibromas. It is the most common benign tumor of the nasopharnyx but only accounts for less than 0. Juvenile nasopharyngeal angiofibroma iowa head and neck. None of these patients required postoperative nasal packing, and all were discharged the day after surgery. 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. As the tumor is largely spaceoccupying and highly vascular, patients typically. Nasopharyngeal angiofibroma is usually encapsulated and composed of vascular tissue and fibrous stroma with coarse or fine collagen fibers. 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. O angiofibroma nasofaringeo juvenil anj e um raro tumor em. Juvenile nasopharyngeal angiofibroma is a rare, highly luiz a cr, romualdo s lt, fava a s. They often grow quickly during puberty, then slow down or stop growing entirely after adolescence.
Jul 15, 2015 juvenile nasopharyngeal angiofibroma jna is a benign neoplasm of the nasopharynx. Their use is purely for academic and teaching purposes. Juvenile nasopharyngeal angiofibroma extending into the oral. Juvenile nasopharyngeal angiofibroma with orbital extension angiofibroma nasofaringeo juvenil con extension orbitaria. The juvenile nasopharyngeal angiofibroma is a benign neoplasm of the posterior. The pictures used in this presentation have been obtained from a number of sources.
The management of juvenile angiofibroma ja has changed during the last decades but it still continues to be a challenge. Juvenile nasopharyngeal angiofibroma is a rare benign tumour of vascular origin found in adolescent males, originating around the sphenopalatine foramen. Juvenile nasopharyngeal angiofibroma evaluation and workup return to. Nasopharyngeal angiofibroma symptoms aurora health care. Surgical resection of juvenile angiofibroma vula university of. We report the case of a 17yearold male who presented in 1995 with a mass filling the left posterior nasal cavity. Common practice is to excise the tumor with open or endoscopic surgery. The median progressionfree survival pfs was not reached. The tumor contains many blood vessels, spreads within the area in which it started locally invasive, and can cause bone damage. 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.
Le fibrome nasopharyngien fnp est une tumeur rare qui represente 0. Juvenile nasal angiofibroma jna is a rare sinonasal tumor that is found almost exclusively in adolescent and young adult males. A, pretreatment image shows encroachment of both the floor and lateral wall of the left sphenoid sinus and infratemporal fossa. Nasoangiofibroma youth is a highly vascularized tumor almost exclusively male adolescents presentation. Juvenile nasopharyngeal angiofibroma jna is a disease of adolescent males characterised by. Endoscopic removal of a juvenile nasal angiofibroma ucla duration. Although the use of nonsurgical therapies is described in the literature, surgery is currently considered the ideal treatment for. Juvenile nasopharyngeal angiofibroma jna is a rare and benign but locally in the nasopharynx and nasal. They concluded that juvenile angiofibroma jna growth and vascularization are driven by factors released from stromal fibroblasts. Juvenile nasopharyngeal angiofibroma knowledge for medical.
Pdf imaging in the diagnosis of juvenile nasopharyngeal. A case of angiofibroma limited to the right posterior nasal cavity, nasopharynx, and pterygopalatine fossa was operated upon transnasally under endoscopic. Juvenile nasopharyngeal angiofibroma is a rare, highly. Nasopharyngeal juvenile angiofibroma resected endoscopically. Aug 15, 2019 angiofibroma nasofaringeo pdf introduction. Features are characteristic of a juvenile angiofibroma. The disease free period ranged from 10 to 36 months postoperatively. 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. Juvenile nasopharyngeal angiofibroma jna onset most commonly is in the second decade.
Article pdf available in journal of clinical imaging science. The tumor contains many blood vessels, spreads within the area in which it. Juvenile nasopharyngeal angiofibroma jna is a rare tumor that predominantly occurs in adolescent males, the average age of occurence being 15. Despite tumor reduction with hormones, this approach is not routinely used. Transnasal endoscopic surgery in juvenile nasopharyngeal. Juvenile nasopharyngeal angiofibroma jna is a rare, benign, but locally aggressive tumor occurring almost exclusively in adolescent males. Primary radiation therapy for juvenile nasopharyngeal angiofibroma. 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.
Abstract juvenile nasopharyngeal angiofibroma is a benign vascularized tumor, locally invasive, originated in the sphenopalatine foramen. The typical patient is an adolescent male with a clinical history of recurrent epistaxis and nasal obstruction. May 16, 2018 despite tumor reduction with hormones, this approach is not routinely used. 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. Juvenile nasopharyngeal angiofibroma is a vascular tumor that. 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. Two types of lateral extension in juvenile nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibroma is a benign but locally invasive tumor accounting for about 0. Juvenile nasopharyngeal angiofibroma praneeth koduru. Introduction uncommon, benign and extremely vascular tumour up to 0. 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. Longterm treatment outcomes of juvenile nasopharyngeal angiofibroma treated with radiotherapy risultati a lungo termine del trattamento radioterapico dell angiofibroma nasofaringeo giovanile s.
Juvenile nasopharyngeal angiofibromas are a rare benign, but locally aggressive, vascular tumors that occur almost exclusively in young men. 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. The jna juvenile nasopharyngeal angiofibroma is a rare neoplasm, of benign histology, that affects almost exclusively male sex adolescent individuals. Juvenile nasopharyngeal angiofibroma jna is a benign neoplasm of the nasopharynx.
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