Pdf juvenile angiofibroma jna is a relatively uncommon, highly vascular and benign tumor that presents most commonly in adolescent males. Review article nasopharyngeal angiofibroma ashutosh hota s. Juvenile nasopharyngeal angiofibroma jna onset most commonly is in the second decade. This specimen was a recurrent intracranial extension of angiofibroma.
Although the exact pathogenesis of the tumour is not yet known, natural history and growth patterns can be predicted. Dr kk karade ent head neck thyroid cancer center 19,005 views 3. Biomolecular analysis of juvenile nasopharyngeal angiofibroma. 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 is a rare, highly vascular. Nasopharyngeal angiofibroma is an uncommon, benign, fibrovascular tumor which exhibits a distinct predilection for adolescent males and accounts for less than 1% of all head and neck neoplasms 1, 2. Axial ct, soft tissue and bone windows, at the level of the maxillary sinus show characteristic features of juvenile nasopharyngeal angiofibroma. Staging of juvenile nasopharyngeal angiofibromas is performed with crosssectional imaging and relies on the identification of local tumor extent, and invasion of adjacent spaces. May 16, 2018 patterns of vascularization and surgical morbidity in juvenile nasopharyngeal angiofibroma.
Another case of recurring nasopharyngeal angiofibroma with lateral extension to peterygomaxillary fossa and cheek removed successfully by transpalatal and. Perioperative management of juvenile nasopharyngeal. Although it can occur in all age groups, it is most common in adolescents and young adults between 14 and 25 years of age. From a series of 5 cases studied arteriographically, 2 will be reported in some detail to illustrate these points. Juvenile nasopharyngeal angiofibroma radiology reference. Well circumscribed but unencapsulated polypoid fibrous mass, bleeds severely on manipulation and biopsy, may occlude nares. Juvenile nasopharyngeal angiofibroma, surgical approach, recurrence, residual disease introduction juvenile nasopharyngeal angiofibroma jna is a relatively rare tumor occurring mainly in adolescent boys. 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 jna is a relatively rare tumor of the nasopharynx, comprising only 0. Juvenile nasopharyngeal angiofibroma is a rare benign tumour of vascular origin found in adolescent males, originating around the sphenopalatine foramen. Shows the large size and gaping appearance of some of the blood spaces and the abundant fibrous tissue. Juvenile angiofibroma is a rare, highly vascular, histologically benign but locally invasive tumor, affecting predominantly male adolescents. Longterm results of radiation therapy for juvenile nasopharyngeal angiofibroma. Nasopharyngeal angiofibroma is usually encapsulated and composed of vascular tissue and fibrous stroma with coarse or fine collagen fibers.
As the tumor is largely spaceoccupying and highly vascular, patients typically. Though it is a benign tumor, it is locally invasive and can invade the nose, cheek, orbit. Ages of the patients ranged between 8 and 31 years. These tumors are highly aggressive and are associated with significant morbidity and mortality due to its tendency to bleed. Nasopharyngeal carcinoma biology and management ppt. The endoscopic resection is best indicated for small tumors confined to the nasopharynx, nasal cavity, ethmoid and sphenoid, and in some cases, tumors with extension into the pterygopalatine fossa 4. Pdf imaging in the diagnosis of juvenile nasopharyngeal.
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. Locally advanced staged juvenile nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibromas staging radiology. Ascending pharyngeal, ascending palatine, internal carotid, external carotid, common carotid, 7232012. Nasopharyngeal angiofibroma the journal of laryngology. Jan 14, 2017 juvenile nasopharyngeal angiofibroma for medical. Hunt, in diagnostic immunohistochemistry third edition, 2011. Seven cases of nasopharyngeal angiofibroma treated surgically have been reported.
Nasopharyngeal angiofibroma is a histologically benign but locally aggressive vascular tumor. Nasopharyngeal angiofibroma is a relatively uncommon tumor of adolescence which is usually manifested by massive nosebleeds and nasal obstruction. A juvenile nasopharyngeal angiofibroma jna is a noncancerous tumor made up of blood vessels that grow at the back of the nose. Mean age at diagnosis is 14 years may regress in late teens but may persist into adulthood rare in patients older than 25 years.
Clinical case 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. Juvenile nasopharyngeal angiofibroma nayyarent ppt. Here we present a case series of 45 patients of nasopharyngeal angiofibroma, their clinical presentation, staging. Juvenile nasopharyngeal angiofibroma jna is a histologically benign, but locally invasive neoplasm occuring almost exclusively in adolescent males. Juvenile nasopharyngeal angiofibroma linkedin slideshare.
Juvenile nasopharyngeal angiofibromas occur almost exclusively in males and usually in adolescence 15 years. Jul 18, 2019 angiofibroma nasofaringeo pdf introduction. Sep 01, 2008 juvenile nasopharyngeal angiofibroma is a rare, benign tumor that occurs most often in adolescent males. Juvenile angiofibroma is a rare benign lesion originating from the pterygopalatine fossa with distinctive epidemiologic features and growth patterns. The case of a patient with orbit involvement and intracranial extension due to intracranial extension of nasopharyngeal angiofibroma with ethmoidal origin is presented. Surgical management of juvenile nasopharyngeal angiofibroma. Adolescents and young adults between 14 and 25 years are affected, and there is a distinct male predominance. Jna progressively involves the nasopharynx, nasal cavity, paranasal. Adobe acrobat reader is required to view pdf files. Juvenile nasopharyngeal angiofibroma is a rare, highly luiz a cr, romualdo s lt, fava a s. Juvenile nasopharyngeal angiofibroma jna is a rare, benign neoplasm that accounts for less than 0. Preoperative embolization was performed in 23 patients 41%. Endoscopic removal of juvenile nasopharyngeal angiofibromas e3. Preoperative embolization of juvenile angiofibroma with.
Plump endothelial cells e and uniform fibroblasts f. Typically, the tumor has its onset in childhood, the ages of the patients ranging from seven to nineteen years, although cases in patients up to fifty years of. Endoscopic removal of juvenile nasopharyngeal angiofibromas. A juvenile nasopharyngeal angiofibroma jna is a noncancerous tumor made up of blood vessels that grow at the posterior aspect of the nose. We report the case of a 17yearold male who presented in 1995 with a mass filling the left posterior nasal cavity. Mri provides additional assessment of the tumors interface with adjacent soft tissue and is particularly valuable in evaluating intracranial and cavernous sinus extension.
Nasopharyngeal angiofibroma, an angiographic evaluation. 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 angiofibroma ja is a benign, highly vascular tumor which is. It most commonly affects adolescent males because it is a hormonesensitive tumor.
For a discussion of this entity please refer to the parent article. Common practice is to excise the tumor with open or endoscopic surgery. Juvenile nasopharyngeal angiofibroma with orbital extension. Early postoperative ct is reliable in detecting or excluding residual disease in patients with jna 2,24. Reddy ka, mendenhall wm, amdur rj, stringer sp, cassisi nj. Juvenile nasopharyngeal angiofibroma has been documented since the time of hippocrates 4 bc.
Juvenile nasopharyngeal angiofibroma jna is a rare, benign, but locally aggressive tumor occurring almost exclusively in adolescent males. Patterns of vascularization and surgical morbidity in juvenile nasopharyngeal angiofibroma. They often grow quickly during puberty, then slow down or stop growing entirely after adolescence. Although the use of nonsurgical therapies is described in the literature, surgery is currently considered the ideal treatment for. Nasopharyngeal angiofibroma in patients with familial. Nasopharyngeal angiofibroma symptoms aurora health care. In addition to its usefulness in the diagnosis of this entity, arteriography also has therapeutic implications. These tumors are benign, however they are locally invasive. Jna is classified as type i when the tumor is restricted to the nasal cavity and the nasopharynx without bone. There is an enhancing mass expanding and extending into the posterior nasal air space with erosion of the base of the right pterygoid plate and lateral extension to the infratemporal fossa.
Nasopharyngeal angiofibroma was first recognized in 1847 when chelius described a fibrous nasal polyp that commonly occur in persons about the time of puberty. In 1906 chareau revived the interest in the study of juvenile nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibromas jna are a rare benign, but locally aggressive, vascular tumor. A concise classification system and appropriate treatment options. Nasopharyngeal angiofibroma an overview sciencedirect.
The association of these two rare condi tions suggests that nasopharyngeal angiofibroma is an extracolonic manifestation of adenomatous pol yposis. May, 2010 the preoperative diagnosis of nasopharyngeal angiofibroma can be a challenging task. The diagnosis is predicated upon appropriate clinical history and the highly characteristic radiographic appearance. Juvenile nasopharyngeal angiofibroma jna is a rare vascular tumour affecting mostly adolescent males, benign by histopathology, but considered ma lignant. Juvenile nasopharyngeal angiofibroma is a rare, benign tumor that occurs most often in adolescent males. Dec 21, 2015 axial ct, soft tissue and bone windows, at the level of the maxillary sinus show characteristic features of juvenile nasopharyngeal angiofibroma. Nasopharyngeal angiofibroma naf, occurring mostly in young men, is a histologically benign tumor with aggressive clinical behavior that includes repeated epistaxis and intractable nasal obstruction.
Given the strict epidemiology, hormonal influences seem likely as investigators have revealed the presence of androgen, testosterone, and dihydrotestosterone receptors 3, 4 and. 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. Vascular supply most commonly from internal maxillary artery. The typical patient is an adolescent male with a clinical history of recurrent epistaxis and nasal obstruction. Juvenile angiofibroma jaf is a highly vascular benign tumor of head and neck. We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Jul 15, 2015 juvenile nasopharyngeal angiofibroma jna is a benign neoplasm of the nasopharynx. Juvenile nasopharyngeal angiofibroma treatment, symptoms. 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. Angiofibroma nasofaring juvenile free download as pdf file.
Nasopharyngeal angiofibroma naf is a highly vascular, histologically benign, but sometimes locally aggressive tumor of the nasopharynx that occurs almost exclusively in males, usually between 10 and 20 years of age. Infact hippocrates goes on to describe a polyp in the nose which weeped blood. Any information contained in this pdf file is automatically generated from. Juvenile nasopharyngeal angiofibroma knowledge for.
Pdf juvenile nasopharyngeal angiofibroma researchgate. Juvenile nasopharyngeal angiofibroma of jna 1,5,11 figure 2. Nasoangiofibroma youth is a highly vascularized tumor almost exclusively male adolescents presentation. Juvenile nasal angiogibroma jna is a relatively rare, benign neoplasm generally seen in prepubertal and adolescent males. Ppt juvenile nasopharyngeal angiofibroma powerpoint.
In fact, biopsy prior to definitive treatment is generally condemned as both unnecessary and hazardous. It most commonly affects adolescent males and may grow into fissures of the. Fisch classification, however, is currently accepted. Intracranial extension andor involvement of cn, hypopharynx, orbit. Juvenile nasopharyngeal angiofibroma jna is a rare benign tumor arising predominantly in the. May 16, 2018 nasopharyngeal angiofibroma is usually encapsulated and composed of vascular tissue and fibrous stroma with coarse or fine collagen fibers. These tests have led to the formulations of multiple classification methods.
Nasopharyngeal angiofibroma an overview sciencedirect topics. Angiofibroma nasofaring belia pdf juvenile nasopharyngeal angiofibroma is a pathologically benign yet locally aggressive and destructive vascular lesion of head and neck. Juvenile nasopharyngeal angiofibroma jna is a benign tumor of the nasopharynx, which accounts for 0. In the general population, jna occurs at an incidence of roughly 1.
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