Author links open overlay panel laura garciarodriguez ba a b kelli rudman md a b christopher h. Juvenile nasopharyngeal angiofibroma our experience at a referral hospital. Intricate mixture of stellate and staghorn blood vessels with variable vessel wall thickness ranging from single layer of endothelium to variable smooth muscle coat. Juvenile nasopharyngeal angiofibroma electronic journal of.
Juvenile nasopharyngeal angiofibroma jna is a rare benign tumor arising predominantly in the nasopharynx of adolescent males. Though it is a benign tumor, it is locally invasive and can invade the nose, cheek, orbit. Backgroundjuvenile nasopharyngeal angiofibroma jna is a benign but. Angiofibroma is a descriptive term for a group of lesions with different clinical presentations but with the same histopathology. Angiofibroma is a pink, firm, 1 to 2mm domeshaped papule most commonly located over the nasal bridge and cheeks fig. 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. Stage iii tumors involved the middle fossa or cavernous sinus or both. Dermal dendritic cells usually stain for factor xiiia. Background juvenile nasopharyngeal angiofibroma jna is a benign tumor that presents in adolescent males. It was friedberg who first used the term angiofibroma in 1940.
Imaging in the diagnosis of juvenile nasopharyngeal angiofibroma. 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. It most commonly affects adolescent males because it is a hormonesensitive tumor. Pdf juvenile nasopharyngeal angiofibroma researchgate. Tuberous sclerosis affects both the nervous system and the skin neurocutaneous and may also produce other skin lesions including shagreen spots, ash. Angiofibromas definition of angiofibromas by medical. 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. Jna is classified as type i when the tumor is restricted to the nasal cavity and the nasopharynx without bone. The contents of this presentation do not have any intended commercial use. Health, general cardiovascular tumors case studies diagnosis nasopharyngeal cancer vascular tissue tumors. Juvenile nasopharyngeal angiofibroma jna onset most commonly is in the second decade. The most common presenting symptom is painless nasal obstruction or epistaxis. The management of juvenile angiofibroma ja has changed during the last decades but it still continues to be a challenge. Download fulltext pdf download fulltext pdf juvenile angiofibroma.
Juvenile nasopharyngeal angiofibroma a free powerpoint ppt presentation displayed as a flash slide show on id. Because of the significant blood flow through the tumors, their main presenting symptom is massive nosebleed, which is often recurrent. Radiologists prior knowledge on the tumors characteristics aids in establishing a diagnosis. Starting with a brief introduction to the history, etiology and relevant anatomy, the book goes on to discuss the pathology, clinical features and treatment of this disease in depth, concluding with a summary of current controversies. Staging of juvenile nasopharyngeal angiofibromas is performed with crosssectional imaging and relies on the identification of local tumor extent, and invasion of adjacent spaces.
The juvenile nasopharyngeal angiofibroma has a characteristic growth in all directions from its origin. 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 jna is a relatively rare tumor occurring mainly in adolescent boys. The first soft tissue tumour with a relatively pelvic sitespecificity was a benign stromal polyp described in the early 1960. Ppt juvenile nasopharyngeal angiofibroma powerpoint presentation free to download id. Nasal obstruction may become total, with adenoidtype speech. These red erythematous elevated skin lesions papules are tumors madeup of fibrous tissue angiofibroma and occur with tuberous sclerosis. Generally, an angiofibroma presents as 1 to 5 mm skincolored to erythematous domeshape papule on the face. The surgeon needs free space to manipulate the surgical. The epicenter of ja is located in the sphenopalatine foramen, and its patterns of extension are medial and lateral, complicated due to the deep location of the structures involved and close. These lesions include fibrous papule, facial angiofibroma, pearly penile papule, adenoma sebaceum, periungual fibroma, and koenens tumor. Juvenile nasopharyngeal angiofibroma jna is a rare, benign, highly vascular, and locally aggressive. Juvenile nasopharyngeal angiofibroma 1 juvenile nasopharyngeal angiofibroma.
Although the use of nonsurgical therapies is described in the literature, surgery is currently considered the ideal treatment for. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. Infact hippocrates goes on to describe a polyp in the nose which weeped blood. Juvenile nasopharyngeal angiofibroma radiology reference. Treatment for angiofibroma in ghaziabad, find doctors near you. The adobe flash plugin is needed to view this content. Angiofibroma definition of angiofibroma by medical.
Article information, pdf download for nasopharyngeal angiofibroma. At present, the treatment of choice for juvenile angiofibroma ja is surgery, which has evolved to endoscopic approaches, performed at referral centers. Juvenile nasopharyngeal angiofibroma knowledge for. The typical patient is an adolescent male with a clinical history of recurrent epistaxis and nasal obstruction. These features are compatible with juvenile angiofibroma, a benign but locally invasive tumor. Sclerosing naevus these usually do not exhibit vascular ectasia. Nasal cavity, paranasal sinuses, nasopharynx nasopharyngeal angiofibroma. Fisch classification, however, is currently accepted. Analysis of intraoperative bleeding and recurrence of juvenile nasopharyngeal angiofibromas. Jnas originate from the posterior choanal tissues and rapidly extend into the surrounding regions, including the nasopharynx, the orbits, and even the intracranial cavity. The present article is a retrospective study of surgically treated patients of juvenile nasopharyngeal angiofibroma over a period of 5 years. Cellular angiofibroma is a rare benign mesenchymal neoplasm most commonly occurring in the vulvovaginal region in women and the inguinoscrotal region. Juvenile nasopharyngeal angiofibroma is a pathologically benign yet locally aggressive and destructive vascular lesion of head and neck region typically affecting adolescent boys.
Juvenile nasopharyngeal angiofibroma has been documented since the time of hippocrates 4 bc. Tumors were staged according to size and extension by using the sessions classification. Juvenile nasopharyngeal angiofibroma is a benign but locally invasive tumor accounting for about 0. The pictures used in this presentation have been obtained from a number of sources. Juvenile nasopharyngeal angiofibroma our experience at a. It is not as invasive as to surgery that is almost free of acute complications in. However, the extensions of the tumor seem to be independent, each one with distinct behavior.
Nasopharyngeal angiofibroma is a histologically benign but locally aggressive vascular tumor. Juvenile angiofibroma is a rare, benign, rich vascular tumor, and approximately one new case is diagnosed in denmark each year. A juvenile nasopharyngeal angiofibroma jna is a noncancerous tumor made up of blood vessels that grow at the posterior aspect of the nose. Angiofibroma treatment, angiofibroma treatment in pune. Ppt journal juvenile nasopharynx angiofibroma free download as powerpoint presentation. Surgical treatment of juvenile nasopharyngeal angiofibroma. Ppt journal juvenile nasopharynx angiofibroma radiation. However, this case is not typical as it doesnt invade the nasal fossae. He denies further obstruction and is disease free 27 months postoperatively. S100 stain will highlight the melanocytes, and will be negative in fibrous papules. Review article nasopharyngeal angiofibroma ashutosh hota. Cellular angiofibroma ca belongs to soft tissue tumours that predominantly occur in the distal genital tract of both genders. They often grow quickly during puberty, then slow down or stop growing entirely after adolescence.
Three of them were disease free, and in two cases residues were demonstrated that. Treatment for angiofibroma in pune, find doctors near you. It was first described by hippocrates in the 5 th century bc. This is a classical presentation of juvenile nasopharyngeal angiofibroma.
Nasopharyngeal angiofibroma staging with a novel nominal basis. Histologically, angiofibromas are characterized by fibrosis of the dermis and blood vessels. Additional interphase fish using a breakapart probe for the ncoa2 gene in the six fusionnegative cases did not reveal any split signals. Plain radiographs no longer play a role in the workup of a suspected juvenile nasopharyngeal angiofibroma, however they may still be obtained in some instances during the assessment of nasal obstruction, or symptoms of sinus obstructions. Keywords juvenile nasopharyngeal angiofibroma, angiofibroma. Juvenile nasopharyngeal angiofibroma jna is a rare tumor that exhibits a predictable spreading pattern. Ppt juvenile nasopharyngeal angiofibroma powerpoint. Management of juvenile nasopharyngeal angiofibroma. There are many classification described although none is. Evolution of management article pdf available in international journal of pediatrics 201222.
It sits in the foramen sphenopalatinum and occurs in boys from 14 to. Juvenile nasopharyngeal angiofibroma is a rare tumor in adolescent males. A number of childhood conditions are characterized by angiofibromas, but the most common is tuberous sclerosis. Juvenile angiofibroma is a rare vascular mass that usually develops in the backmost part of the nasal cavity, often but not exclusively, in adolescent males. Juvenile nasopharyngeal angiofibroma 420 introduction juvenile nasopharyngeal angiofibroma jna represents 0.
It is an aggressive neoplasm and shows a propensity for destructive local spread often extending to the base of the skull and into the cranium. Juvenile nasopharyngeal angiofibroma jna is a rare, benign, but locally aggressive tumor occurring almost exclusively in adolescent males. Pdf juvenile angiofibroma jna is a relatively uncommon, highly vascular and benign tumor that. We aimed to report the characteristic magnetic resonance imaging mri findings and the spread patterns of jna. It is not as invasive as to surgery that is almost free of. Angiofibromas are small, reddish brown or even fleshcolored, smooth, shiny, 0. This is a histologically benign lesion with very aggressive local behavior. Juvenile nasopharyngeal angiofibroma radiology case. Juvenile nasopharyngeal angiofibroma is a locally aggressive benign vascular neoplasm, composed of vasogenic and myofibroblastic elements, accounts for 0. Original article, clinical report by ear, nose and throat journal. Juvenile angiofibroma is a rare benign lesion originating from the pterygopalatine fossa with distinctive epidemiologic features and growth patterns. The tumor contains many blood vessels, spreads within the area in which it.
Interphase fish on cut sections from nine additional cases of soft tissue angiofibroma using pools of bacs covering ahrr and ncoa2 yielded fusion signals in at least 10% of the nuclei in three cases. Juvenile nasopharyngeal angiofibroma jna jnas are wellvascularized benign lesions typically occurring in adolescent males. Although surgical excision is the mainstay of treatment, recurrences complicate treatment. Juvenile nasopharyngeal angiofibroma jna is a rare and benign but locally aggressive fibrovascular tumor arising from the posterolateral wall of the. For a discussion of this entity please refer to the parent article. It is almost exclusicely encountered in adolescent males 1. Their use is purely for academic and teaching purposes.
Nasal septal angiofibroma, a subclass of extranasopharyngeal angiofibroma. Nasal obstruction may become total, with adenoidtype speech, discomfort in. Pdf download for juvenile nasopharyngeal angiofibroma. As the tumor is largely spaceoccupying and highly vascular, patients typically. Nasopharyngeal angiofibroma symptoms aurora health care.
There have been no recurrences, and all the patients are symptomfree. Juvenile angiofibroma medigoo health medical tests. Juvenile nasopharyngeal angiofibroma jna is a benign tumor that tends to bleed and occurs in the nasopharynx of prepubertal and adolescent males. Given its vascularity, prominent flow voids are seen on mri leading to a salt and pepper appearance.
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